Systemic lupus erythematosus (SLE or lupus) is a chronic autoimmune disease that can be fatal, though with recent medical advances, fatalities are becoming increasingly rare. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings L00-L99 - Diseases of the Skin and Subcutaneous tissue (L00-L08 Infections of the Skin and Subcutaneous tissue M00-M99 - Diseases of the Musculoskeletal system and Connective tissue (M00-M25 Arthropathies (M00-M03 Infectious arthropathies The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Mendelian Inheritance in Man project is a Database that catalogues all the known Diseases with a genetic component, and—when possible—links them The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. MedlinePlus, with the MedlinePlus Medical Encyclopedia, is a website network containing Health information from the world's largest medical Library eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which results in an immune response against its own cells and tissues As with other autoimmune diseases, the immune system attacks the body’s cells and tissue, resulting in inflammation and tissue damage. Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which results in an immune response against its own cells and tissues An immune system is a collection of mechanisms within an Organism that protects against Disease by identifying and killing Pathogens and Tumor SLE can affect any part of the body, but most often harms the heart, joints, skin, lungs, blood vessels, liver, kidneys, and nervous system. The heart is a muscular organ in all Vertebrates responsible for pumping Blood through the Blood vessels by repeated rhythmic A joint is the location at which two or more Bones make contact The skin is the outer covering of living tissue of an animal (or plant lung is the essential Respiration organ in air-breathing Animals including most Tetrapods a few Fish and a few Snails The most primitive The blood vessels are part of the Circulatory system and function to transport Blood throughout the body The liver is a vital organ in the human body and is present in Vertebrates and some other animals The kidneys are complicated organs that have numerous biological roles The nervous system is a Network of specialized cells that communicate information about an animal's surroundings and itself The course of the disease is unpredictable, with periods of illness (called flares) alternating with remission. Lupus can occur at any age, and is most common in women, particularly of non-European descent. ^[1] Lupus is treatable symptomatically, mainly with corticosteroids and immunosuppressants, though there is currently no cure. Symptomatic treatment is any medical therapy of a Disease that only affects its Symptoms not its cause i Corticosteroids are a class of Steroid hormones that are produced in the Adrenal cortex. Immunosuppression involves an act that reduces the activation or Efficacy of the Immune system. Survival in patients with SLE in the United States, Canada, and Europe is approximately 95% at 5 years, 90% at 10 years, and 78% at 20 years. ^[2]

Classification

Lupus is a chronic autoimmune disease. Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which results in an immune response against its own cells and tissues Clinically, it can affect multiple organ systems, including the heart, skin, joints, kidneys, and nervous system. There are several types of lupus; generally, when the word "lupus" alone is used, it refers to systemic lupus erythematosus or SLE, as discussed in this article. Other types include:

• Drug-induced lupus erythematosus, a drug-induced form of SLE; this type of lupus can occur equally for either gender. Drug-induced lupus erythematosus (DIL or DILE is an Autoimmune disorder similar to Systemic lupus erythematosus (SLE which is induced by chronic use of certain
• Lupus nephritis, an inflammation of the kidneys caused by SLE. Lupus nephritis is an inflammation of the kidney caused by Systemic lupus erythematosus (SLE a disease of the Immune system. The kidneys are complicated organs that have numerous biological roles
• Discoid lupus erythematosus, a skin disorder that causes a red, raised rash on the face and scalp. Discoid lupus occasionally (1–5%) develops into SLE. ^[3]
• Subacute cutaneous lupus erythematosus, which causes nonscarring skin lesions on patches of skin exposed to sunlight. A lesion is any abnormal tissue found on or in an organism usually damaged by disease or trauma ^[4]
• Neonatal lupus, a rare disease affecting babies born to women with SLE, Sjögren's syndrome, or sometimes no autoimmune disorder. Sjögren's syndrome is an autoimmune disorder in which Immune cells attack and destroy the Exocrine glands ref name="pmid18289371"> that It is theorized that maternal antibodies attack the fetus, causing skin rash; liver problems; low blood counts, which gradually fade; and heart block, leading to bradycardia. Bradycardia, as applied to adult medicine is defined as a resting Heart rate of under 60 beats per minute though it is seldom symptomatic until the rate drops below 50 beat/min ^[4]

Signs and symptoms

SLE is one of several diseases known as "the great imitators"^[5] because its symptoms vary so widely, it often mimics or is mistaken for other illnesses and because the symptoms come and go unpredictably. Diagnosis can be elusive, with patients sometimes suffering unexplained symptoms and untreated SLE for years. Common initial and chronic complaints are fever, malaise, joint pains, myalgias, and fatigue. Fever (also known as pyrexia, from the Greek pyretos meaning fire or a febrile response, from the Latin word Febris Malaise is a feeling of general discomfort or uneasiness an "out of sorts" feeling often the first indication of an infection or other disease Arthralgia (from Greek arthro-, joint + -algos, pain literally means joint pain; it is a symptom of injury infection illnesses -- in particular Myalgia means " Muscle Pain " and is a symptom of many diseases and disorders Because they are so often seen with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms (see below), however, they are considered suggestive.

Common symptoms explained

Dermatological manifestations

As many as 30% of patients present with some dermatological symptoms (and 65% suffer such symptoms at some point), with 30% to 50% suffering from the classic malar rash (or butterfly rash) associated with the disease. In Medicine, malar rash, also called butterfly rash, is a Medical sign consisting of a characteristic form of facial rash Patients may present with discoid lupus (thick, red scaly patches on the skin). Alopecia; mouth, nasal, and vaginal ulcers; and lesions on the skin are also possible manifestations. Alopecia or hair loss is the medical description of the loss of hair from the head or body sometimes to the extent of Baldness. An oral ulcer (from Latin ulcus) is the name for the appearance of an open sore inside the mouth caused by a break in the Mucous membrane or the epithelium

Musculoskeletal manifestations

Patients most often seek medical attention for joint pain, with the small joints of the hand and wrist usually affected, although all joints are at risk. Arthralgia (from Greek arthro-, joint + -algos, pain literally means joint pain; it is a symptom of injury infection illnesses -- in particular The Lupus Foundation of America estimates that more than 90 percent will experience joint and/or muscle pain at some time during the course of their illness. ^[6] Unlike rheumatoid arthritis, lupus arthritis is less disabling and usually does not cause severe destruction of the joints. Rheumatoid arthritis ( RA) is a chronic systemic autoimmune disorder that causes the Immune system to attack the Joints, where Fewer than ten percent of people with lupus arthritis will develop deformities of the hands and feet. ^[6]

Hematological manifestations

Anemia and iron deficiency may develop in as many as half of patients. Anemia ( AmE) or anæmia/anaemia ( BrE) (from the Ancient Greek grc-Latn anaîmia, meaning “without blood” is defined as a qualitative Low platelet and white blood cell counts may be due to the disease or a side effect of pharmacological treatment. Platelets, or Thrombocytes, are small cytoplasmic bodies derived from cells They circulate in the Blood of Mammals and are involved Patients may have an association with antiphospholipid antibody syndrome (a thrombotic disorder) wherein autoantibodies to phospholipids are present in the patient's serum. Antiphospholipid syndrome (APS or APLS or antiphospholipid antibody syndrome is a disorder of Coagulation, which causes blood clots ( Thrombosis) in both Abnormalities associated with antiphospholipid antibody syndrome include a paradoxical prolonged PTT (which usually occurs in hemorrhagic disorders) and a positive test for antiphospholipid antibodies; the combination of such findings have earned the term "lupus anticoagulant positive. Lupus anticoagulant (also known as lupus antibody, LA, or lupus inhibitors) is a medical phenomenon where autoantibodies bind to Phospholipids " Another autoantibody finding in lupus is the anticardiolipin antibody, which can cause a false positive test for syphilis. Antiphospholipid syndrome (APS or APLS or antiphospholipid antibody syndrome is a disorder of Coagulation, which causes blood clots ( Thrombosis) in both Syphilis is a Sexually transmitted disease caused by the spirochetal Bacterium Treponema pallidum pallidum.

Cardiac manifestations

Patients may present with inflammation of various parts of the heart, such as pericarditis, myocarditis, and endocarditis. The heart is a muscular organ in all Vertebrates responsible for pumping Blood through the Blood vessels by repeated rhythmic Pericarditis is an Inflammation ( -itis) of the Pericardium (the fibrous sac surrounding the heart In Medicine ( Cardiology) myocarditis is Inflammation of the Myocardium, the muscular part of the Heart. Endocarditis is an Inflammation of the inner layer of the Heart, the Endocardium. The endocarditis of SLE is characteristically noninfective (Libman-Sacks endocarditis) and involves either the mitral valve or the tricuspid valve. Libman-Sacks endocarditis is a form of nonbacterial Endocarditis that is seen in systemic lupus erythematosus. The miter valve (also known as the bicuspid valve or left atrioventricular valve) is a dual flap (bi = 2 valve in the Heart that lies between The tricuspid valve (also known as the right atrioventricular valve) is on the right side of the heart between the Right atrium and the Right ventricle Atherosclerosis also tends to occur more often and advances more rapidly in SLE patients than in the general population. Atherosclerosis is a Disease affecting arterial Blood vessels It is a chronic inflammatory response in the walls of arteries in large part due to the accumulation ^[7][8][9]

Pulmonary manifestations

Lung and pleura inflammation can cause pleuritis, pleural effusion, lupus pneumonitis, chronic diffuse interstitial lung disease, pulmonary hypertension, pulmonary emboli, and pulmonary hemorrhage. Pleurisy, also known as pleuritis, is an Inflammation of the pleura the lining of the Pleural cavity surrounding the Lungs Pleurisy has a variety Pleural effusion is excess fluid that accumulates in the Pleural cavity, the fluid-filled space that surrounds the Lungs Excessive amounts of such fluid can impair In Medicine, pulmonary hypertension (PH is an increase in blood pressure in the Pulmonary artery, Pulmonary vein, or pulmonary capillaries together known Pulmonary embolism (PE is a blockage of the Pulmonary artery or one of its branches usually occurring when a venous Thrombus (blood clot from a vein Pulmonary hemorrhage (or "pulmonary haemorrhage" refers to Bleeding from the Lung.

Hepatic involvement

See autoimmune hepatitis. Anomalous presentation of Human leukocyte antigen (HLA class II on the surface of Hepatocytes possibly due to Genetic predisposition or Acute liver infection

Renal involvement

Painless hematuria or proteinuria may often be the only presenting renal symptom. In Medicine, hematuria (or "haematuria" is the presence of Red blood cells (erythrocytes in the Urine. Proteinuria (from protein and urine) means the presence of anexcess of serum Proteins in the Urine. Acute or chronic renal impairment may develop with lupus nephritis, leading to acute or end stage renal failure. Lupus nephritis is an inflammation of the kidney caused by Systemic lupus erythematosus (SLE a disease of the Immune system. Renal failure or kidney Because of early recognition and management of SLE, end stage renal failure occurs in less than 5% of patients.

Histologically, a hallmark of SLE is membranous glomerulonephritis with "wire loop" abnormalities. Glomerulonephritis, also known as glomerular nephritis, abbreviated GN, is a renal disease characterized by Inflammation of the glomeruli ^[10] This finding is due to immune complex deposition along the glomerular basement membrane, leading to a typical granular appearance in immunofluorescence testing. The glomerular basement membrane is the Basal laminal portion of the Glomerulus which performs the actual filtration though the filtration slits between the Podocytes Immunofluorescence is the labeling of antibodies or Antigens with fluorescent Dyes This technique is often used to visualize the subcellular

Neurological manifestations

About 10% of patients may present with seizures or psychosis. An epileptic seizure is caused by excessive and/or hypersynchronous electrical Neuronal activity and is usually self-limiting Psychosis (from the Greek ψυχή "psyche" for mind or soul and -οσις "-osis" for abnormal condition with adjective psychotic One-third may test positive for abnormalities in the cerebrospinal fluid. Cerebrospinal fluid ( CSF) Liquor cerebrospinalis, is a clear Bodily fluid that occupies the Subarachnoid space and the Ventricular system

T-cell abnormalities

Abnormalities in T cell-signaling are associated with SLE, including a deficiency in CD45 phosphatase and increased expression of CD40 ligand. T cells belong to a group of White blood cells known as Lymphocytes, and play a central role in Cell-mediated immunity. In Immunology, the CD45 Antigen (CD stands for Cluster of differentiation) is a Protein which was originally called leukocyte common antigen A phosphatase is an Enzyme that removes a Phosphate group from its Substrate by hydrolysing Phosphoric acid mono Esters into

Other rarer manifestations

Lupus gastroenteritis, lupus pancreatitis, lupus cystitis, autoimmune inner ear disease, parasympathetic dysfunction, retinal vasculitis, and systemic vasculitis. Gastroenteritis (also known as gastro, gastric flu, and stomach flu, although unrelated to Influenza) is Inflammation of the Pancreatitis is the Inflammation of the Pancreas. See also Acute pancreatitis and Chronic pancreatitis for more details Cystitis is Inflammation of the Urinary bladder. The condition more often affects women but can affect either sex and all age groups Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which results in an immune response against its own cells and tissues Otitis interna ( Internal Otitis) is an Inflammation of the Inner ear and is usually considered synonymous with Labyrinthitis. The parasympathetic Nervous system ( PSNS) is a division of the Autonomic nervous system (ANS along with the Sympathetic nervous system Vasculitis (plural vasculitides) a group of diseases featuring Inflammation of the wall of Blood vessels including veins ( Phlebitis) arteries Vasculitis (plural vasculitides) a group of diseases featuring Inflammation of the wall of Blood vessels including veins ( Phlebitis) arteries

Other abnormalities include:

• Increased expression of FcεRIγ, which replaces the sometimes deficient TCR ζ chain
• Increased and sustained calcium levels in T cells
• Moderate increase of inositol triphosphate
• Reduction in PKC phosphorylation
• Reduction in Ras-MAP kinase signaling
• Deficiencies in protein kinase A I activity

Causes

Transmission

In SLE, the body's immune system produces antibodies against itself, particularly against proteins in the cell nucleus. FcεRI, or Fc epsilon RI, is the high- Affinity receptor for Immunoglobulin E (IgE an Antibody Isotype involved in See also Principality of Zeta Zeta (uppercase Ζ, lowercase ζ; Ζήτα Zita is the sixth letter of the Greek alphabet. T cells belong to a group of White blood cells known as Lymphocytes, and play a central role in Cell-mediated immunity. Inositol trisphosphate or inositol 145-trisphosphate (also commonly known as triphosphoinositol; abbreviated InsP3 or IP3 Protein kinase C ('PKC') is a family of protein kinases consisting of ~10 Isozymes. Mitogen-activated protein (MAP kinases ( are Serine/threonine-specific protein kinases that respond to extracellular stimuli ( Mitogens and regulate various cellular A protein kinase is a Kinase Enzyme that modifies other Proteins by chemically adding Phosphate groups to them ( Phosphorylation) SLE is triggered by environmental factors that are unknown (but probably include viruses) in people with certain combinations of genes in their immune system.

"All the key components of the immune system are involved in the underlying mechanisms" of SLE, according to Rahman, and SLE is the prototypical autoimmune disease. The immune system must have a balance (homeostasis) between being sensitive enough to protect against infection, and being too sensitive and attacking the body's own proteins (autoimmunity). Homeostasis (from Greek: ὅμος hómos, "equal" and ιστημι istēmi, "to stand" lit From an evolutionary perspective, according to Crow, the population must have enough genetic diversity to protect itself against a wide range of possible infection; some genetic combinations result in autoimmunity. The likely environmental triggers include ultraviolet light, drugs, and viruses. These stimuli cause the destruction of cells and expose their DNA, histones, and other proteins, particularly parts of the cell nucleus. In Biology, histones are the chief Protein components of Chromatin. Because of genetic variations in different components of the immune system, in some people the immune system attacks these nuclear-related proteins and produces antibodies against them. Ultimately, these antibody complexes damage blood vessels in critical areas of the body, such as the glomeruli of the kidney; these antibody attacks are the cause of SLE. A glomerulus is a Capillary tuft surrounded by Bowman's capsule in Nephrons of the vertebrate Kidney. Researchers are now identifying the individual genes, the proteins they produce, and their role in the immune system. Each protein is a link on the autoimmune chain, and researchers are trying to find drugs to break each of those links. ^[11][12][13]

SLE is a chronic inflammatory disease believed to be a type III hypersensitivity response with potential type II involvement. Inflammation ( Latin, inflamatio, to set on fire is the complex biological response of vascular tissues to harmful stimuli such as Pathogens Hypersensitivity (also called hypersensitivity reaction refers to undesirable (damaging discomfort-producing and sometimes fatal reactions produced by the normal immune system Hypersensitivity (also called hypersensitivity reaction refers to undesirable (damaging discomfort-producing and sometimes fatal reactions produced by the normal immune system ^[14]

Genetics

The first mechanism may arise genetically. Research indicates that SLE may have a genetic link. Genetics (from Ancient Greek grc-Latn genetikos, “genitive” and that from grc-Latn genesis, “origin” a discipline of Biology, is Lupus does run in families, but no single "lupus gene" has yet been identified. Instead, multiple genes appear to influence a person's chance of developing lupus when triggered by environmental factors. The most important genes are located on chromosome 6, where mutations may occur randomly (de novo) or may be inherited. Chromosome 6 is one of the 23 pairs of Chromosomes in Humans People normally have two copies of this chromosome Additionally, people with SLE have an altered RUNX-1 binding site, which may be either cause or contributor (or both) to the condition. Altered binding sites for RUNX-1 have also been found in people with psoriasis and rheumatoid arthritis. Psoriasis (səˈraɪəsɪs ( suh-RI-uh-sus) is a non-contagious disorder which affects the Skin and Joints It commonly causes red scaly Rheumatoid arthritis ( RA) is a chronic systemic autoimmune disorder that causes the Immune system to attack the Joints, where

Environmental triggers

The second mechanism may be due to environmental factors. These factors may not only exacerbate existing lupus conditions but also trigger the initial onset. They include certain medications (such as some antidepressants and antibiotics), extreme stress, exposure to sunlight, hormones, and infections. An antidepressant is a Psychiatric medication used for alleviating major depression or Dysthymia ('milder' depression In modern usage an antibiotic is a Chemotherapeutic agent with activity against Microorganisms such as Bacteria, fungi or Protozoa Some researchers have sought to find a connection between certain infectious agents (viruses and bacteria), but no pathogen can be consistently linked to the disease. A virus (from the Latin virus meaning Toxin or Poison) is a sub-microscopic infectious agent that is unable The Bacteria ( singular: bacterium) are a large group of unicellular Microorganisms Typically a few Micrometres in length bacteria have UV radiation has been shown to trigger the photosensitive lupus rash, but some evidence also suggests that UV light is capable of altering the structure of the DNA, leading to the creation of autoantibodies. Some researchers have found that women with silicone gel-filled breast implants have produced antibodies to their own collagen, but it is not known how often these antibodies occur in the general population, and there is no data that show that these antibodies cause connective tissue diseases such as lupus. Silicones are largely inert compounds with a wide variety of forms and uses A breast implant is a Prosthesis used to enlarge the size of a woman's Breasts (known as breast augmentation, breast enlargement, mammoplasty Collagen is the main Protein of Connective tissue in Animals and the most abundant protein in Mammals making up about 50% of the whole-body protein A connective tissue disease is any disease that has the connective tissues of the body as a target of pathology

Drug reactions

Drug-induced lupus erythematosus is a reversible condition that usually occurs in patients being treated for a long-term illness. Drug-induced lupus erythematosus (DIL or DILE is an Autoimmune disorder similar to Systemic lupus erythematosus (SLE which is induced by chronic use of certain Drug-induced lupus mimics systemic lupus. However, symptoms of drug-induced lupus generally disappear once a patient is taken off the medication that triggered the episode. There are about 400 medications currently in use that can cause this condition, though the most common drugs are procainamide, hydralazine and quinidine. Procainamide (proe-KANE-a-mide ( INN, trade names Pronestyl, Procan, Procanbid) is a Pharmaceutical Antiarrhythmic agent used for Hydralazine hydrochloride (1-hydrazinophthalazine monohydrochloride trade name Apresoline) is a direct-acting Smooth muscle relaxant used to treat Hypertension Quinidine is a Pharmaceutical agent that acts as a Class I antiarrhythmic agent in the Heart.

Non-SLE forms of lupus

Discoid (cutaneous) lupus is limited to skin symptoms and is diagnosed by biopsy of skin rash on the face, neck, or scalp. Often an antinuclear antibody (ANA) test for discoid patients is negative or a low-titer positive. Anti-nuclear antibodies ( ANA s also known as anti-nuclear factor or ANF) are antibodies directed against contents of the cell nucleus About 1–5% of discoid lupus patients eventually develop SLE.

Clearance deficiency

Clearance deficiency.

The exact mechanisms for the development of systemic lupus erythematosus (SLE) are still unclear, since the pathogenesis is a multifactorial event. Beside discussed causations, impaired clearance of dying cells is a potential pathway for the development of this systemic autoimmune disease. Autoimmune diseases arise from an overactive Immune response of the body against substances and tissues normally present in the body This includes deficient phagocytic activity and scant serum components in addition to increased apoptosis.

Monocytes isolated from whole blood of SLE patients show reduced expression of CD44 surface molecules involved in the uptake of apoptotic cells. Monocyte is a type of Leukocyte, part of the Human body 's Immune system. Whole Blood is the term used in Transfusion medicine for human Blood from a standard Blood donation. Most of the monocytes and tingible body macrophages (TBM), which are found in the germinal centres of lymph nodes, even show a definitely different morphology in patients with SLE; they are smaller or scarce and die earlier. Germinal centres (GC are areas within Lymph nodes where B lymphocytes rapidly divide and are an important part of the humoral immune response. A Lymph node ( lɪmf noʊd is an organ consisting of many types of cells and is a part of the Lymphatic system. Serum components like complement factors, CRP, and some glycoproteins are furthermore decisively important for an efficiently operating phagocytosis. The complement system is a Biochemical cascade that helps clear Pathogens from an organism C-reactive protein ( CRP) is a Plasma protein, an Acute phase protein produced by the Liver and by Adipocytes. Not to be confused with Peptidoglycan. Glycoproteins are proteins that contain Oligosaccharide chains ( Glycans) covalently attached In patients, these components are often missing, diminished, or inefficient.

The clearance of early apoptotic cells is an important function in multicellular organisms. It leads to a progression of the apoptosis process and finally to secondary necrosis of the cells if this ability is disturbed. Necrosis (in Greek Νεκρός = "dead" is the name given to unnatural Death of cells and living tissue. Necrotic cells release nuclear fragments as potential autoantigens as well as internal danger signals, inducing maturation of dendritic cells (DC), since they have lost their membranes' integrity. Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which results in an immune response against its own cells and tissues Dendritic cells (DCs are Immune cells and form part of the Mammalian Immune system. Increased appearance of apoptotic cells also simulates inefficient clearance. That leads to maturation of DC and also to the presentation of intracellular antigens of late apoptotic or secondary necrotic cells, via MHC molecules. Autoimmunity possibly results by the extended exposure to nuclear and intracellular autoantigens derived from late apoptotic and secondary necrotic cells. Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which results in an immune response against its own cells and tissues B and T cell tolerance for apoptotic cells is abrogated, and the lymphocytes get activated by these autoantigens; inflammation and the production of autoantibodies by plasma cells is initiated. T cells belong to a group of White blood cells known as Lymphocytes, and play a central role in Cell-mediated immunity. A lymphocyte is a type of White blood cell in the Vertebrate Immune system. Inflammation ( Latin, inflamatio, to set on fire is the complex biological response of vascular tissues to harmful stimuli such as Pathogens Plasma cells (also called plasma B cells or plasmocytes) are cells of the Immune system that secrete large amounts of antibodies. A clearance deficiency in the skin for apoptotic cells has also been observed in patients with cutaneous lupus erythematosus (CLE).

Germinal centres.

Accumulation in germinal centres (GC)

In healthy conditions, apoptotic lymphocytes are removed in germinal centres by specialised phagocytes, the tingible body macrophages (TBM); that’s why no free apoptotic and potential autoantigenic material can be seen. In some patients with SLE, accumulation of apoptotic debris can be observed in GC because of an ineffective clearance of apoptotic cells. Most generally the accumulation of capital refers simply to the gathering or amassment of objects of value the increase in wealth or the creation of wealth Debris (ˈdeɪbriː/ /dɛˈbriː is a word used to describe the remains of something that has been otherwise destroyed In close proximity to TBM, follicular dendritic cells (FDC) are localised in GC, which attach antigen material to their surface and, in contrast to bone marrow-derived DC, neither take it up nor present it via MHC molecules. Follicular dendritic cells (FDC are cells of the Immune system found in Lymph follicles They are probably not of Hematopoietic origin but simply look Bone marrow is the flexible tissue found in the hollow interior of Bones In adults marrow in large bones produces new Blood cells It constitutes 4% of The major histocompatibility complex ( MHC) is a large genomic region or Gene family found in most Vertebrates It is the most gene-dense region Autoreactive B cells can accidentally emerge during somatic hypermutation and migrate into the GC light zone. B cells are Lymphocytes that play a large role in the humoral immune response (as opposed to the cell-mediated immune response, which is governed by Somatic hypermutation (or SHM is a mechanism inside cells that is part of the way the immune system adapts to the new foreign elements which confront it (for example Autoreactive B cells, maturated coincidentally, normally don’t receive survival signals by antigen planted on follicular dendritic cells and perish by apoptosis. In the case of clearance deficiency, apoptotic nuclear debris accumulates in the light zone of GC and gets attached to FDC. This serves as a germinal centre survival signal for autoreactive B-cells. After migration into the mantle zone, autoreactive B cells require further survival signals from autoreactive helper T cells, which promote the maturation of autoantibody-producing plasma cells and B memory cells. In the presence of autoreactive T cells, a chronic autoimmune disease may be the consequence. Autoimmune diseases arise from an overactive Immune response of the body against substances and tissues normally present in the body

Pathophysiology

One manifestation of lupus is abnormalities in apoptosis, a type of programmed cell death in which aging or damaged cells are neatly disposed of as a part of normal growth or functioning.

Abnormalities in apoptosis

• Apoptosis is increased in monocytes and keratinocytes;
• Expression of Fas by B cells and T cells is increased;
• There are correlations between the apoptotic rates of lymphocytes and disease activity. Monocyte is a type of Leukocyte, part of the Human body 's Immune system. The keratinocyte is the major cell type of the epidermis, making up about 90% of epidermal cells Gene expression is the process by which inheritable information from a Gene, such as the DNA sequence, is made into a functional Gene product, such Fas ligand ( FasL) is a type II transmembrane protein that belongs to the tumor necrosis factor (TNF family B cells are Lymphocytes that play a large role in the humoral immune response (as opposed to the cell-mediated immune response, which is governed by T cells belong to a group of White blood cells known as Lymphocytes, and play a central role in Cell-mediated immunity.

Tingible body macrophages (TBMs) are large phagocytic cells in the germinal centers of secondary lymph nodes; they express CD68 protein. Germinal centres (GC are areas within Lymph nodes where B lymphocytes rapidly divide and are an important part of the humoral immune response. A Lymph node ( lɪmf noʊd is an organ consisting of many types of cells and is a part of the Lymphatic system. These cells normally engulf B cells that have undergone apoptosis after somatic hypermutation. Somatic hypermutation (or SHM is a mechanism inside cells that is part of the way the immune system adapts to the new foreign elements which confront it (for example In some patients with SLE, significantly fewer TBMs can be found, and these cells rarely contain material from apoptotic B cells. Also, uningested apoptotic nuclei can be found outside of TBMs. This material may present a threat to the tolerization of B cells and T cells. Dendritic cells in the germinal center may endocytose such antigenic material and present it to T cells, activating them. Dendritic cells (DCs are Immune cells and form part of the Mammalian Immune system. Also, apoptotic chromatin and nuclei may attach to the surfaces of follicular dendritic cells and make this material available for activating other B cells that may have randomly acquired self-specificity through somatic hypermutation. Somatic hypermutation (or SHM is a mechanism inside cells that is part of the way the immune system adapts to the new foreign elements which confront it (for example ^[15]

Diagnosis

Microphotograph of a histological section of human skin prepared for direct immunofluorescence using an anti-IgG antibody. Histology (from the Greek = 'tissue' is the study of the microscopic anatomy of cells and tissues of Plants and Immunofluorescence is the labeling of antibodies or Antigens with fluorescent Dyes This technique is often used to visualize the subcellular The skin is from a patient with systemic lupus erythematosus and shows IgG deposits at two different places: the first is a bandlike deposit along the epidermal basement membrane ("lupus band test" is positive); the second is within the nuclei of the epidermal cells (antinuclear antibodies are present). The basement membrane is a structure that supports overlying Epithelial or Endothelial cells. Epidermis is the outermost layer of the Skin. It forms the waterproof protective wrap over the body's surface and is made up of stratified squamous Epithelium with

Some physicians make a diagnosis on the basis of the ACR classification criteria (see below). The criteria, however, were established mainly for use in scientific research (i. e. , inclusion in randomized controlled trials), and patients may have lupus but never meet the full criteria. A randomized controlled trial (RCT is a type of scientific Experiment most commonly used in testing the Efficacy or Effectiveness of Healthcare

Antinuclear antibody testing and anti-extractable nuclear antigen (anti-ENA) form the mainstay of serologic testing for lupus. Anti-nuclear antibodies ( ANA s also known as anti-nuclear factor or ANF) are antibodies directed against contents of the cell nucleus Serology is the scientific study of blood serum. In practice the term usually refers to the Diagnostic identification of antibodies in the serum Antiphospholipid antibodies occur more often in SLE and can predispose for thrombosis. Thrombosis is the formation of a blood Clot ( Thrombus) inside a Blood vessel, obstructing the flow of Blood through the Circulatory More specific are the anti-Smith and anti-dsDNA antibodies. biology LSm proteins are a family of RNA -binding Proteins found in virtually every cellular Organism. Other tests routinely performed in suspected SLE are complement system levels (low levels suggest consumption by the immune system), electrolytes and renal function (disturbed if the kidney is involved), liver enzymes, and a complete blood count. The complement system is a Biochemical cascade that helps clear Pathogens from an organism An electrolyte is any substance containing free Ions that behaves as an electrically conductive medium Renal function, in Nephrology, is an indication of the state of the Kidney and its role in Renal physiology. Liver function tests (LFTs or LFs which include liver enzymes, are groups of Clinical biochemistry laboratory blood assays designed to give information about the A complete blood count ( CBC) also known as full blood count ( FBC) or full blood exam ( FBE) or blood panel, is

Previously, the lupus erythematosus (LE) cell test was not commonly used for diagnosis because those LE cells are only found in 50–75% of SLE patients, and are also found in some patients with rheumatoid arthritis, scleroderma, and drug sensitivities. Because of this, the LE cell test is now performed only rarely and is mostly of historical significance. ^[16]

Diagnostic criteria

The American College of Rheumatology (ACR) established eleven criteria in 1982,^[17] which were revised in 1997^[18] as a classificatory instrument to operationalise the definition of SLE in clinical trials. They were not intended to be used to diagnose individual patients and do not do well in that capacity. For inclusion in clinical trials, patients must meet the following three criteria to be classified as having SLE: (i) patient must present with four of the below eleven symptoms, (ii) either simultaneously or serially, (iii) during a given period of observation.

1. Serositis: Pleuritis (inflammation of the membrane around the lungs) or pericarditis (inflammation of the membrane around the heart); sensitivity = 56%; specificity = 86% (pleural is more sensitive; cardiac is more specific). Pleurisy, also known as pleuritis, is an Inflammation of the pleura the lining of the Pleural cavity surrounding the Lungs Pleurisy has a variety Pericarditis is an Inflammation ( -itis) of the Pericardium (the fibrous sac surrounding the heart ^[19]
2. Oral ulcers (includes oral or nasopharyngeal ulcers).
3. Arthritis: nonerosive arthritis of two or more peripheral joints, with tenderness, swelling, or effusion; sensitivity = 86%; specificity = 37%. Arthritis (from Greek arthro-, joint + -itis, inflammation plural arthritides is a group of conditions involving damage to the Joints of the body ^[19]
4. Photosensitivity (exposure to ultraviolet light causes rash); sensitivity = 43%; specificity = 96%. Photodermatitis, or sometimes called by the nonscientific term sun poisoning, is a reaction of the Skin to UV rays of the Sun, or Photoallergy ^[19]
5. Blood—hematologic disorder—hemolytic anemia (low red blood cell count) or leukopenia (white blood cell count<4000/µl), lymphopenia (<1500/µl) or thrombocytopenia (<100000/µl) in the absence of offending drug; sensitivity = 59%; specificity = 89%. Hemolytic anemia is Anemia due to Hemolysis, the abnormal breakdown of Red blood cells (RBCs either in the Blood vessels (intravascular hemolysis Red blood cells are the most common type of Blood cell and the Vertebrate body's principal means of delivering Oxygen to the body tissues via the Blood Leukopenia (or leukocytopenia, or leucopenia, from Greek λευκό -white and πενία -deficiency is a decrease in the number of Lymphocytopenia, or lymphopenia, is the condition of having an abnormally low level of Lymphocytes in the blood Thrombocytopenia (or -paenia, or thrombopenia in short is the presence of relatively few Platelets in Blood. ^[19] Hypocomplementemia is also seen, due to either consumption of C3 and C4 by immune complex-induced inflammation or to congenitally complement deficiency, which may predispose to SLE.
6. Renal disorder: More than 0. 5g per day protein in urine or cellular casts seen in urine under a microscope; sensitivity = 51%; specificity = 94%. Proteinuria (from protein and urine) means the presence of anexcess of serum Proteins in the Urine. Urinary casts are cylindrical aggregations of particulate matter that form in the distal Nephron, dislodge and eventually pass into the Urine. ^[19]
7. Antinuclear antibody test positive; sensitivity = 99%; specificity = 49%. Anti-nuclear antibodies ( ANA s also known as anti-nuclear factor or ANF) are antibodies directed against contents of the cell nucleus ^[19]
8. Immunologic disorder: Positive anti-Smith, anti-ds DNA, antiphospholipid antibody, and/or false positive serological test for syphilis; sensitivity = 85%; specificity = 93%. biology LSm proteins are a family of RNA -binding Proteins found in virtually every cellular Organism. Antiphospholipid syndrome (APS or APLS or antiphospholipid antibody syndrome is a disorder of Coagulation, which causes blood clots ( Thrombosis) in both Serology is the scientific study of blood serum. In practice the term usually refers to the Diagnostic identification of antibodies in the serum Syphilis is a Sexually transmitted disease caused by the spirochetal Bacterium Treponema pallidum pallidum. ^[19] Presence of anti-ss DNA in 70% of patients (though also positive in patients with rheumatic disease and healthy persons^[20]).
9. Neurologic disorder: Seizures or psychosis; sensitivity = 20%; specificity = 98%. An epileptic seizure is caused by excessive and/or hypersynchronous electrical Neuronal activity and is usually self-limiting Psychosis (from the Greek ψυχή "psyche" for mind or soul and -οσις "-osis" for abnormal condition with adjective psychotic ^[19]
10. Malar rash (rash on cheeks); sensitivity = 57%; specificity = 96%. In Medicine, malar rash, also called butterfly rash, is a Medical sign consisting of a characteristic form of facial rash ^[19]
11. Discoid lupus (red, scaly patches on skin that cause scarring); sensitivity = 18%; specificity = 99%. ^[19]

A useful mnemonic for these 11 criteria is SOAP BRAIN MD: Serositis (8), Oral ulcers (4), Arthritis (5), Photosensitivity (3), Blood Changes (9), Renal involvement (proteinuria or casts) (6), ANA (10), Immunological changes (11), Neurological signs (seizures, frank psychosis) (7), Malar Rash (1), Discoid Rash (2).

Some patients, especially those with antiphospholipid syndrome, may have SLE without four criteria, and SLE is associated with manifestations other than those listed in the criteria. Antiphospholipid syndrome (APS or APLS or antiphospholipid antibody syndrome is a disorder of Coagulation, which causes blood clots ( Thrombosis) in both ^[21][22][23]

Alternative criteria

Recursive partitioning has been used to identify more parsimonious criteria. Recursive partitioning is a statistical method for Multivariable analysis. ^[19] This analysis presented two diagnostic classification trees:

1. Simplest classification tree: LSE is diagnosed if the patient has an immunologic disorder (anti-DNA antibody, anti-Smith antibody, false positive syphilis test, or LE cells) or malar rash. In Medicine, malar rash, also called butterfly rash, is a Medical sign consisting of a characteristic form of facial rash

• sensitivity = 92%
• specificity = 92%

2. Full classification tree: Uses 6 criteria.

• sensitivity = 97%
• specificity = 95%

Other alternative criteria have been suggested. ^[24]

Common misdiagnoses

Porphyria

Porphyrias are complex genetic disorders that share many symptoms with lupus, but impact the enzymes responsible for building heme, a component needed in heme proteins. Porphyrias are a group of inherited or acquired disorders of certain Enzymes in the Heme biosynthetic pathway (also called Porphyrin pathway A heme ( American English) or haem ( British English) is a Prosthetic group that consists of an Iron atom contained in the center of Porphyrias are ecogenic disorders requiring both environmental and genetic backgrounds to manifest with a variety of symptoms and medical complications. They are noted for photosensitivity and have been associated with transient and permanent production of autoantibodies. The five major forms of dominantly inherited porphyrias (acute intermittent porphyria, porphyria cutanea tarda, hereditary coproporphyria, variegate porphyria, and erythropoietic protoporphyria) have been detected in systemic lupus erythematosus and discoid lupus patients over the past 50 years. Hereditary coproporphyria (HCP is a form of hepatic Porphyria associated with a deficiency of the enzyme Coproporphyrinogen III oxidase. Physicians should have a high degree of suspicion of porphyrias in all lupus cases and act accordingly when patients are in a medical crisis that may be due to an underlying acute hepatic porphyria. Drug-induced lupus and photosensitivity warrant an investigation for an underlying porphyria, since multiple drug reactions are a hallmark complication of porphyrias. Patients with both lupus and porphyrias should avoid porphyrinogenic drugs and hormone preparations, but the effects of these drugs on lupus patients is undetermined.

Patients with acute hepatic porphyrias (acute intermittent porphyria, hereditary coproporphyria, variegate porphyria) have been detected in lupus patients with severe life-threatening "lupus" complications known as neurolupus. The liver is a vital organ in the human body and is present in Vertebrates and some other animals Symptoms are identical to acute hepatic porphyria attacks and include seizures, psychosis, peripheral neuropathy, and syndrome of inappropriate antidiuretic hormone (SIADH) associated with dangerously low sodium levels (hyponatremia). Psychosis (from the Greek ψυχή "psyche" for mind or soul and -οσις "-osis" for abnormal condition with adjective psychotic Peripheral neuropathy is the term for damage to Nerves of the Peripheral nervous system, which may be caused either by diseases of the Nerve or from the Porphyria attacks require intervention with intravenous glucose, heme preparations, and the discontinuation of dangerous porphyrinogenic drugs, including antiseizure drugs. Several other lupus complications have been associated with porphyrias, including pancreatitis and pericarditis. Pancreatitis is the Inflammation of the Pancreas. See also Acute pancreatitis and Chronic pancreatitis for more details Pericarditis is an Inflammation ( -itis) of the Pericardium (the fibrous sac surrounding the heart Porphyrin testing should be performed on urine, stool/bile, and blood to detect all types of porphyrias; repeat testing should be performed in suspicious cases. Appropriate enzyme tests or DNA testing should also be pursued to obtain a complete diagnosis, which could include a dual porphyria.

Common dual diagnoses

SLE is sometimes diagnosed in conjunction with other conditions, including rheumatoid arthritis, scurvy, and fibromyalgia. Rheumatoid arthritis ( RA) is a chronic systemic autoimmune disorder that causes the Immune system to attack the Joints, where Scurvy (NLat scorbutus is a disease resulting from a deficiency of Vitamin C, which is required for the synthesis of Collagen in humans Fibromyalgia (FM is a disorder classified by the presence of chronic widespread Pain and tactile Allodynia.

Treatment

As lupus erythematosus is a chronic disease with no known cure, treatment is restricted to dealing with the symptoms. Symptomatic treatment is any medical therapy of a Disease that only affects its Symptoms not its cause i Essentially, this involves preventing flares and reducing their severity and duration when they occur. There are several means of preventing and dealing with flares, including drugs, alternative medicine, and lifestyle changes.

Drug therapy

Due to the variety of symptoms and organ system involvement with lupus patients, the severity of the SLE in a particular patient must be assessed in order to successfully treat SLE. Mild or remittent disease can sometimes be safely left untreated. If required, nonsteroidal anti-inflammatory drugs and antimalarials may be used. Non-steroidal anti-inflammatory drugs, usually abbreviated to NSAID s or NAID s, are drugs with Analgesic, Antipyretic and in higher Malaria is a vector -borne Infectious disease caused by Protozoan Parasites It is widespread in tropical and subtropical regions including

Disease-modifying antirheumatic drugs (DMARDs) are used preventively to reduce the incidence of flares, the process of the disease, and lower the need for steroid use; when flares occur, they are treated with corticosteroids. Disease-modifying antirheumatic drugs ( DMARDs) is a category of drugs used in many autoimmune disorders to slow down disease progression Corticosteroids are a class of Steroid hormones that are produced in the Adrenal cortex. DMARDs commonly in use are antimalarials and immunosuppressants (e. An immunosuppressant is a substance that performs Immunosuppression of the Immune system. g. , methotrexate and azathioprine). Azathioprine is an immunosuppressant used in Organ transplantation Autoimmune disease such as Rheumatoid arthritis and Pemphigus or inflammatory Hydroxychloroquine (trade name Plaquenil) is an FDA-approved antimalarial used for constitutional, cutaneous, and articular manifestations, while cyclophosphamide (trade names Cytoxan and Neosar) is used for severe glomerulonephritis or other organ-damaging complications. Hydroxychloroquine is an Antimalarial drug sold under the trade name Plaquenil, also used to reduce inflammation in the treatment of Rheumatoid arthritis Cyclophosphamide (the generic name for Cytoxan Neosar Revimmune also known as cytophosphane is a Nitrogen mustard alkylating agent, from the oxazophorines group Glomerulonephritis, also known as glomerular nephritis, abbreviated GN, is a renal disease characterized by Inflammation of the glomeruli In 2005, mycophenolic acid (trade name CellCept) became accepted for treatment of lupus nephritis. Mycophenolic acid ( INN) (ˌmaɪkoʊˈfɛnɒlɪk or mycophenolate is an immunosuppressant drug used to prevent rejection in Organ transplantation

In more severe cases, medications that modulate the immune system (primarily corticosteroids and immunosuppressants) are used to control the disease and prevent recurrence of symptoms (known as flares). For a list of immunosuppressive drugs see the Transplant rejection page. Patients who require steroids frequently may develop obesity, diabetes mellitus, diabetes, and osteoporosis. Obesity is a condition in which excess Body fat has accumulated to such an extent that health may be negatively affected Diabetes mellitus (ˌdaɪəˈbiːtiːz or /ˌdaɪəˈbiːtəs/ /məˈlaɪtəs/ or /ˈmɛlətəs/ often referred to simply as diabetes ( Ancient Greek: grc Diabetes mellitus (ˌdaɪəˈbiːtiːz or /ˌdaɪəˈbiːtəs/ /məˈlaɪtəs/ or /ˈmɛlətəs/ often referred to simply as diabetes ( Ancient Greek: grc Osteoporosis is a Disease of Bone that leads to an increased risk of fracture. Depending on the dosage, corticosteroids can cause other side effects, such as a puffy face, an unusually large appetite, and difficulty sleeping. Those side effects can subside if and when the large initial dosage is reduced, but long-term use of even low doses can cause elevated blood pressure and cataracts. Blood pressure is also the title of a short story by Damon Runyan in Guys and Dolls and Other Stories A cataract is a clouding that develops in the crystalline lens of the Eye or in its envelope varying in degree from slight to complete opacity Due to these side effects, steroids are avoided if possible.

Since a large percentage of lupus patients suffer from varying amounts of chronic pain, stronger prescription analgesics may be used if over-the-counter drugs (mainly nonsteroidal anti-inflammatory drugs) do not provide effective relief. Chronic pain is defined as Pain that persists longer than the temporal course of natural healing associated with a particular type of injury or disease process Moderate pain in lupus patients is typically treated with mild prescription opiates such as dextropropoxyphene (trade name Darvocet) and co-codamol (trade name Tylenol #3). Dextropropoxyphene is an Analgesic in the Opioid category It is used to treat mild to moderate pain and as an Anti-tussive. Co-codamol ( BAN) is a non- Proprietary name used to denote a Compound analgesic, a combination of Codeine phosphate and Paracetamol Moderate to severe chronic pain is treated with stronger opioids, such as hydrocodone (trade names Lorcet, Lortab, Norco, Vicodin, Vicoprofen) or longer-acting continuous-release opioids, such as oxycodone (trade name OxyContin), MS Contin, or Methadone. Hydrocodone or dihydrocodeinone is a semi-synthetic Opioid derived from two of the naturally occurring Opiates Codeine and Thebaine An opioid is a chemical Substance that has a Morphine -like action in the body Oxycodone is an Opioid Analgesic Medication synthesized from Thebaine. MS Contin is a time-released formulation of Morphine, usually taken every twelve hours for chronic pain Methadone ( Dolophine Amidone Methadose Physeptone Heptadon and many others is a synthetic Opioid, used medically as an Analgesic, Antitussive The Fentanyl Duragesic Transdermal patch is also a widely used treatment option for the chronic pain of lupus complications because of its long-acting timed release and ease of use. Fentanyl is one of the most powerful Opioid Analgesics with a potency approximately 81 times that of Morphine. When opioids are used for prolonged periods, drug tolerance, chemical dependency, and (rarely) addiction may occur. Opiate addiction is not typically a concern for lupus patients, since the condition is not likely to ever completely disappear. Thus, lifelong treatment with opioids is fairly common in lupus patients who exhibit chronic pain symptoms, accompanied by periodic titration that is typical of any long-term opioid regimen. Titration is a common laboratory method of Quantitative chemical analysis that is used to determine the unknown Concentration of a known Reactant

UVA1 phototherapy

In 1987, Tina Lomardi, M. D. first reported that long-wave ultraviolet radiation (UVA1) had a favorable effect on disease activity in SLE-model mice. In 1987, McGrath, Bak, and Michalski reported in Arthritis and Rheumatism, Vol. 30, No. 5, May, that long-wave ultraviolet radiation (UVA1) had a favorable effect on disease activity in SLE-model mice ( "Ultraviolet-A Light Prolongs Survival and Improves Immune Function in Hybrid Mice"). Both McGrath and independent Dutch researchers have repeatedly reproduced these findings in SLE patients. ^[25] Devices for administering therapeutic doses of UVA1 are available in Europe but not in the U. S. The U. S. Food and Drug Administration's Office of Science and Technology conducted UVA1 phototherapy studies in an SLE-model mouse in 1997 to "prepare for future reviews of UVA-emitting tanning devices for such clinical applications. "^[26] Some patients, however, have taken matters into their own hands. Chicago-based reporter and lupus patient Anthony DeBartolo built his own 8-lamp UVA1 home tanning device, which delivers the same 6- to 8-joule therapeutic dose of McGrath's 24-lamp clinical research equipment. DeBartolo's experiences were published in the 2004 book Lupus Underground. ^[27]

Lifestyle changes

Other measures, such as avoiding sunlight or covering up with sun-protective clothing, can also be effective in preventing photosensitivity problems. Weight loss is also recommended in overweight and obese patients to alleviate some of the effects of the disease, especially where joint involvement is significant.

Treatment research

Other immunosuppressants (drugs that lower the body's normal immune response), and bone marrow transplant autologous stem cell transplants are under investigation as a possible cure. Recently, treatments that are more specific in modifying the particular subset of the immune cells (e. g. , B or T cells) or the cytokine proteins they secrete have been gaining attention. Cytokines are a category of signalling Proteins and Glycoproteins that like Hormones and Neurotransmitters, are used extensively in cellular Research into new treatments has recently been accelerated by genetic discoveries, especially mapping of the human genome. According to a June 2006 market analysis report by Datamonitor, treatment for SLE could be on the verge of a breakthrough, as there are numerous late-phase trials currently being carried out. ^[28] There have been promising advances in the area of stem cell research, implicating a treatment with adult stem cells being harvested from the patients themselves. Stem cells are cells found in most if not all multi-cellular Organisms. ^[29][30]

Prevention

Lupus is not understood well enough to be prevented, but when the disease develops, quality of life can be improved through flare prevention. The warning signs of an impending flare include increased fatigue, pain, rash, fever, abdominal discomfort, headache, and dizziness. Early recognition of warning signs and good communication with a doctor can help individuals with lupus remain active, experience less pain, and reduce medical visits. ^[4]

Prevention of complications during pregnancy

While most infants born to mothers with lupus are healthy, pregnant mothers with SLE should remain under a doctor's care until delivery. Neonatal lupus is rare, but identification of mothers at highest risk for complications allows for prompt treatment before or after birth. In addition, SLE can flare during pregnancy, and proper treatment can maintain the health of the mother longer. Women pregnant and known to have the antibodies for anti-Ro (SSA) or anti-La (SSB) should have echocardiograms during the 16th and 30th weeks of pregnancy to monitor the health of the heart and surrounding vasculature. ^[4]

Prognosis

In the 1950s, most patients diagnosed with SLE lived fewer than five years. Advances in diagnosis and treatment have improved survival to the point where over 90% of patients now survive for more than ten years, and many can live relatively asymptomatically. The most common cause of death is infection due to immunosuppression as a result of medications used to manage the disease. An infectious disease is a clinically evident Disease resulting from the presence of Pathogenic microbial agents including Pathogenic viruses Pathogenic Immunosuppression involves an act that reduces the activation or Efficacy of the Immune system. Prognosis is normally worse for men and children than for women; fortunately, if symptoms are present after age 60, the disease tends to run a more benign course. The ANA is the most sensitive screening test, while anti-Sm (anti-Smith) is the most specific. biology LSm proteins are a family of RNA -binding Proteins found in virtually every cellular Organism. The ds-DNA (double-stranded DNA) antibody is also fairly specific and often fluctuates with disease activity; the ds-DNA titer is therefore sometimes useful to diagnose or monitor acute flares or response to treatment. Deoxyribonucleic acid ( DNA) is a Nucleic acid that contains the genetic instructions used in the development and functioning of all known ^[31]

Epidemiology

Previously believed to be a rare disease, lupus has seen an increase in awareness and education since the 1960s. This has helped many more patients get an accurate diagnosis, making it possible to estimate the number of people with lupus with some certainty. In the United States alone, it is estimated that between 270,000 and 1. 5 million people have lupus, making it more common than cystic fibrosis or cerebral palsy. Cystic fibrosis (also known as CF, mucoviscoidosis, or mucoviscidosis) is a hereditary disease affecting the exocrine (mucus glands of the lungs Cerebral palsy ( CP) is an Umbrella term encompassing a group of non-progressive, non- contagious conditions that cause Physical disability The disease affects both females and males, though young women are diagnosed nine times more often than men. SLE occurs with much greater severity among African-American women, who suffer more severe symptoms as well as a higher mortality rate. ^[32] Worldwide, a conservative estimate states that over 5 million people have lupus.

Although SLE can occur in anyone, at any age, it is most common in women of childbearing age. It affects 1 in 4000 people in the United States, again with women becoming afflicted far more often than men. The disease appears to be more prevalent in women of African, Asian, Hispanic, and Native American origin, but this may be due to socioeconomic factors. People with relatives who suffer from SLE, rheumatoid arthritis, or thrombotic thrombocytopenic purpura are at a slightly higher risk than the general population. Rheumatoid arthritis ( RA) is a chronic systemic autoimmune disorder that causes the Immune system to attack the Joints, where Thrombotic thrombocytopenic purpura ( TTP or Moschcowitz disease) is a rare disorder of the blood-coagulation system causing extensive microscopic

History

Medical historians have theorized that people with porphyria (a disease that shares many symptoms with lupus) generated folklore stories of vampires and werewolves, due to the photosensitivity, scarring, hair growth, and porphyrin brownish-red stained teeth in severe recessive forms of porphyria (or combinations of the disorder, known as dual, homozygous, or compound heterozygous porphyrias). Porphyrias are a group of inherited or acquired disorders of certain Enzymes in the Heme biosynthetic pathway (also called Porphyrin pathway

The history of lupus erythematosus can be divided into three periods: classical, neoclassical, and modern. The classical period began when the disease was first recognized in the Middle Ages and saw the description of the dermatological manifestation of the disorder. The term lupus is attributed to 12th-century physician Rogerius, who used it to describe the classic malar rash. A physician, medical practitioner or medical doctor who practices Medicine, and is concerned with maintaining or restoring human Health Rogerius (before 1140-c 1195 also called Rogerius Salernitanus, Roger Frugard, Roger Frugardi, Roggerio Frugardo, Rüdiger Frutgard The neoclassical period was heralded by Móric Kaposi's recognition in 1872 of the systemic manifestations of the disease. Moritz Kaposi (Kaposi Mór (born 23 October 1837 in Kaposvár, Hungary - 6 March 1902 in Vienna, Austria Year 1872 ( MDCCCLXXII) was a Leap year starting on Monday (link will display the full calendar of the Gregorian Calendar (or a Leap year The modern period began in 1948 with the discovery of the LE cell (the lupus erythematosus cell—a misnomer, as it occurs with other diseases as well) and is characterised by advances in our knowledge of the pathophysiology and clinical-laboratory features of the disease, as well as advances in treatment. Year 1948 ( MCMXLVIII) was a Leap year starting on Thursday (link will display the 1948 calendar of the Gregorian calendar.

Useful medication for the disease was first found in 1894, when quinine was first reported as an effective therapy. Year 1894 ( MDCCCXCIV) was a Common year starting on Monday (link will display the full calendar of the Gregorian calendar (or a Common Quinine (ˈkwaɪnaɪn kwɪˈniːn ˈkwiːniːn is a natural white Crystalline Alkaloid having Antipyretic (fever-reducing antimalarial, Four years later, the use of salicylates in conjunction with quinine was noted to be of still greater benefit. Salicylic acid (from the Latin word for the willow tree Salix, from whose bark it can be obtained is a Beta hydroxy acid (BHA with the formula This was the best available treatment to patients until the middle of the twentieth century, when Hench discovered the efficacy of corticosteroids in the treatment of SLE. Corticosteroids are a class of Steroid hormones that are produced in the Adrenal cortex.

Origins of "lupus erythematosus"

There are several explanations ventured for the term lupus erythematosus. Lupus is Latin for wolf, and "erythro" is derived from ερυθρός, Greek for "red. Latin ( lingua Latīna, laˈtiːna is an Italic language, historically spoken in Latium and Ancient Rome. " All explanations originate with the reddish, butterfly-shaped malar rash that the disease classically exhibits across the nose and cheeks. In Medicine, malar rash, also called butterfly rash, is a Medical sign consisting of a characteristic form of facial rash

1. In various accounts, some doctors thought the rash resembled the pattern of fur on a wolf's face.
2. In other accounts, doctors thought that the rash, which was often more severe in earlier centuries, created lesions that resembled wolf bites or scratches.
3. Another account claims that the term "lupus" did not come from Latin at all, but from the term for a French style of mask that women reportedly wore to conceal the rash on their faces. The mask is called a "loup," French for "wolf. "
4. Another common explanation for the term is that the disease's course involves repeated attacks like those of a voracious predator, leaving behind the red blotches.

Notable patients

• Ray Walston, character actor. Ray Walston ( December 2 1914 – January 1 2001) was an American stage, Television and Feature film
• Tim Raines, former major league baseball player, primarily with the Montreal Expos and Chicago White Sox. Timothy Raines (born September 16 1959 in Sanford Florida) nicknamed "Rock"is a former Left fielder in Major League Baseball Franchise history Creation of the franchise In 1960 Montreal lost its International League team the Montreal Royals (an affiliate of The Chicago White Sox are a professional Baseball team based in Chicago, Illinois.
• Ferdinand Marcos, former Philippine president, who died from complications of lupus in 1989. Ferdinand Emmanuel Edralín Marcos ( September 11, 1917 &ndash September 28, 1989) was President of the Philippines from 1965 to 1986
• Michael Jackson, pop superstar, diagnosed with the disease in 1984. Michael Joseph Jackson (born August 29 1958 is an American musician entertainer and businessman
• Flannery O'Connor, American fiction writer who died of the disease in 1964. Mary Flannery O'Connor ( March 25 1925 &ndash August 3 1964) was an American Novelist, Short-story
• Hugh Gaitskell, British politician. Hugh Todd Naylor Gaitskell ( 9 April 1906 &ndash 18 January 1963) was a British politician leader of the Labour Party from 1955
• J Dilla (also known as Jay Dee, born James Dewitt Yancey), a hip-hop producer and beatmaker who died of the disease in 2006. James Dewitt Yancey ( February 7, 1974 – February 10, 2006) better known by his stage name J Dilla or Jay Dee, was an James Dewitt Yancey ( February 7, 1974 – February 10, 2006) better known by his stage name J Dilla or Jay Dee, was an
• Elaine Paige, British actress and singer. Elaine Paige OBE (née Bickerstaff born 5 March 1948 is an English Singer and actress, best known for her work in Musical theatre
• Seal (born Seal Henry Olusegun Olumide Adelo Samuel), British artist/singer. Sealhenry Olusegun Olumide Adelo Samuel (born February 19, 1963 in Paddington, London) is a British soul Singer and
• Charles Kuralt, former anchor of CBS Sunday Morning. Charles Kuralt ( 10 September 1934 &ndash 4 July 1997) was an award-winning American journalist CBS News Sunday Morning is an American television news magazine program created by Robert Northshield and Charles Kuralt, and appearing continuously from January
• Inday Ba (also known as N'Deaye Ba), a Swedish-born actress who died from complications of lupus at age 32. Inday Ba (also known as N'Deaye Ba) ( 10 August 1972 – 20 April 2005) was a Swedish -born film stage and television
• Caroline Dorough-Cochran, sister of Howie D. of the Backstreet Boys, who founded the Dorough Lupus Foundation in her memory. Howard Dwaine Dorough (born August 22 1973 also known as Howie D, is an American musician and member of the Boy band Backstreet Boys. Backstreet Boys are a Grammy -nominated American pop group They were the first group launched by fallen Boy band mogul Lou Pearlman.
• Stephanie Smith, artist who died of SLE complications in 1969 at the age of 22. biology LSm proteins are a family of RNA -binding Proteins found in virtually every cellular Organism. The anti-Smith (or anti-Sm) antigen was discovered in her and is the basis of a SLE diagnostic test.
• Yonna, contestant on Ben Fogle's Extreme Dreams.
• Louisa May Alcott, American author. Louisa May Alcott ( November 29, 1832 – March 6, 1888) was an American Novelist.
• Sophie Howard, British glamour model. Sophie Howard (born February 24 1983) is a Glamour model from Southport England.
• Donald Byrne, American chess player who died from complications of lupus in 1976. Donald Byrne ( June 12, 1930 – April 8, 1976) was one of the USA 's strongest Chess players during the 1950s and 1960s Chess is a recreational and competitive Game played between two players.
• Mary Elizabeth McDonough, American actress. Mary Elizabeth McDonough is an American actress (born May 4 1961 in Van Nuys California) best known for her role as "Erin Walton" Reportedly contracted it after undergoing silicone implantation in her breasts in 1984.
• Christopher Bracey, Nigerian poet.
• Mercedes Scelba-Shorte, America's Next Top Model Season Two runner-up and model. America's Next Top Model (often shortened to Top Model or abbreviated as ANTM) is a Reality television show in which a number of women compete
• Leslie Hunt, semifinalist on American Idol, 19th place. The sixth season of American Idol premiered on the Fox Broadcasting Company on January 16, 2007 and ran until May 23,

See also

• Abzyme
• Alliance for Lupus Research
• Antinuclear antibody
• Canine discoid lupus erythematosus in dogs
• Lupus Canada
• Lupus Foundation of America

References

External links

• Systemic lupus erythematosus at the Open Directory Project
• List of lupus organizations
• Alliance for Lupus Research
• The Lupus UK Site
• Resources for Lupus