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Addison's disease
Classification and external resources
ICD-10 E27.1-E27.2
ICD-9 255.4
DiseasesDB 222
MedlinePlus 000378
eMedicine med/42 
MeSH D000224

Addison's disease (also known as chronic adrenal insufficiency, hypocortisolism or hypocorticism) is a rare endocrine disorder in which the adrenal gland produces insufficient amounts of steroid hormones (glucocorticoids and often mineralocorticoids). 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 E00-E35 - Endocrine diseases (E00-E07 Thyroid gland / Thyroid hormone ( Congenital iodine-deficiency syndrome ( E00-E35 - Endocrine diseases (E00-E07 Thyroid gland / Thyroid hormone ( Congenital iodine-deficiency syndrome ( 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 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 Adrenal insufficiency is a condition in which the Adrenal glands located above the Kidneys do not produce adequate amounts of steroid hormones (chemicals produced by the Cortisol is a Corticosteroid Hormone produced by the Adrenal gland (in the Zona fasciculata of the Adrenal cortex) The endocrine system is an integrated system of small organs that involve the release of extracellular signaling molecules known as Hormones The endocrine system is instrumental In Mammals the adrenal glands (also known as suprarenal glands) are the triangle-shaped Endocrine glands that sit on top of the Kidneys their Steroid hormones are Steroids which act as Hormones Mammalian steroid hormones can be grouped into five groups by the receptors to which they bind Glucocorticoids (GC are a class of Steroid hormones characterised by an ability to bind with the glucocorticoid receptor ( GR) and trigger similar effects Mineralocorticoids are a class of Steroid hormones characterised by their similarity to Aldosterone and their influence on salt and water balance It may develop in children as well as adults, and may occur as the result of a large number of underlying causes. The condition is named after Dr Thomas Addison, the British physician who first described the condition in his 1855 On the Constitutional and Local Effects of Disease of the Suprarenal Capsules. Thomas Addison (April 1793 - June 29, 1860) was a renowned 19th-century English physician and The United Kingdom of Great Britain and Northern Ireland, commonly known as the United Kingdom, the UK or Britain,is a Sovereign state located A physician, medical practitioner or medical doctor who practices Medicine, and is concerned with maintaining or restoring human Health Year 1855 ( MDCCCLV) was a Common year starting on Monday (link will display the full calendar of the Gregorian Calendar (or a Common year [1] The adjective "Addisonian" is used for features of the condition, as well as patients with Addison's disease. [2]

The condition is generally diagnosed with blood tests, medical imaging and additional investigations. A blood test is a laboratory analysis performed on a Blood sample that is usually extracted from a Vein in the arm using a needle, or via Medical imaging refers to the techniques and processes used to create Images of the human body (or parts thereof for clinical purposes ( Medical procedures seeking to [2] Treatment is with replacement of the hormones (oral hydrocortisone and fludrocortisone). Cortisol is a Corticosteroid Hormone produced by the Adrenal gland (in the Zona fasciculata of the Adrenal cortex) Fludrocortisone is a synthetic Corticosteroid with moderate Glucocorticoid potency and much greater Mineralocorticoid potency If the disease is caused by an underlying problem, it may be possible to address. Regular follow-up and monitoring for other health problems is necessary. [2]

Contents

Signs and symptoms

Symptoms

The symptoms of Addison's disease develop insidiously, and it may take some time to be recognized. The most common symptoms are fatigue, muscle weakness, weight loss, vomiting, diarrhea, headache, sweating, changes in mood and personality and joint and muscle pains. Muscle weakness (or "lack of strength" is a direct term for the inability to exert force with one's Muscles to the degree that would be expected given the individual's Vomiting (also called throwing up, emesis) is the forceful expulsion of the contents of one's Stomach through the Mouth and sometimes the In Medicine, diarrhea, also spelled diarrhoea (see spelling differences) is frequent loose or liquid Bowel movements Acute diarrhea A headache ( cephalalgia in medical terminology is a condition of pain in the Head; sometimes Neck or upper back pain may also be interpreted 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 Some have marked cravings for salt or salty foods due to the urinary losses of sodium. [2]

Clinical signs

On examination, the following may be noticed:[2]

Addisonian crisis

An "Addisonian crisis" or "adrenal crisis" is a constellation of symptoms that indicate severe adrenal insufficiency. A goitre ( BrE) or goiter ( AmE) ( Latin struma) also called a bronchocele, is a swelling in the neck (just below the Vitiligo (ˈvɪtəˈlaɪgoʊ or leukoderma is a chronic skin disease that causes loss of pigment, resulting in irregular pale patches of Skin This may be the result of either previously undiagnosed Addison's disease, a disease process suddenly affecting adrenal function (such as adrenal hemorrhage), or an intercurrent problem (e. g. infection, trauma) in the setting of known Addison's disease. Additionally, this situation may develop in those on long-term oral glucocorticoids who have suddenly ceased taking their medication. Glucocorticoids (GC are a class of Steroid hormones characterised by an ability to bind with the glucocorticoid receptor ( GR) and trigger similar effects It is also a concern in the setting of myxedema coma; thyroxine given in that setting without glucocorticoids may precipitate a crisis. Myxedema coma is state of decompensated Hypothyroidism. The patient may have lab values identical to a "normal" hypothyroid state but a stressful event (infections Thyroxine, or 353'5'-tetra­iodothyronine (often abbreviated as T4) a form of Thyroid hormones is the major Hormone secreted by the Glucocorticoids (GC are a class of Steroid hormones characterised by an ability to bind with the glucocorticoid receptor ( GR) and trigger similar effects

Untreated, an Addisonian crisis can be fatal. It is a medical emergency, usually requiring hospitalization. A medical emergency is an Injury or Illness that is acute and poses an immediate risk to a person's life or long term health Characteristic symptoms are:[3]

Diagnosis

Suggestive features

Routine investigations may show:[2]

Testing

Cortisol
Cortisol
Aldosterone
Aldosterone

In suspected cases of Addison's disease, one needs to demonstrate that adrenal hormone levels are low even after appropriate stimulation with synthetic pituitary hormone tetracosactide. Cortisol is a Corticosteroid Hormone produced by the Adrenal gland (in the Zona fasciculata of the Adrenal cortex) Aldosterone is a hormone that causes the tubules of the kidneys to retain sodium and water Tetracosactide (marketed under the brand name Synacthen) is a synthetic analogue of the naturally-occurring Adrenocorticotrophic hormone (ACTH Two tests are performed, the short and the long test.

The short test compares blood cortisol levels before and after 250 micrograms of tetracosactide (IM/IV) is given. If, one hour later, plasma cortisol exceeds 170 nmol/L and has risen by at least 330 nmol/L to at least 690 nmol/L, adrenal failure is excluded. If the short test is abnormal, the long test is used to differentiate between primary adrenal failure and secondary adrenocortical failure.

The long test uses 1 mg tetracosactide (IM). Blood is taken 1, 4, 8, and 24 hours later. Normal plasma cortisol level should reach 1000 nmol/L by 4 hours. In primary Addison's disease, the cortisol level is reduced at all stages whereas in secondary corticoadrenal insufficiency, a delayed but normal response is seen.

Other tests that may be performed to distinguish between various causes of hypoadrenalism are renin and adrenocorticotropic hormone levels, as well as medical imaging - usually in the form of ultrasound, computed tomography or magnetic resonance imaging (MRI). Renin (pronounced "Ree-nin" or "Rē-nin" (ˈriːnɨn also known as Angiotensinogenase, is a circulating Enzyme that participates in the body's Adrenocorticotropic hormone ( ACTH or corticotropin) is a Polypeptide Hormone produced and secreted by the Pituitary gland Medical imaging refers to the techniques and processes used to create Images of the human body (or parts thereof for clinical purposes ( Medical procedures seeking to Computed tomography (CT is a Medical imaging method employing Tomography.

Causes

Causes of adrenal insufficiency can be grouped by the way in which they cause the adrenals to produce insufficient cortisol. These are adrenal dysgenesis (the gland has not formed adequately during development), impaired steroidogenesis (the gland is present but is biochemically unable to produce cortisol) or adrenal destruction (disease processes leading to the gland being damaged). [2]

Adrenal dysgenesis

All causes in this category are genetic, and generally very rare. These include mutations to the SF1 transcription factor, congenital adrenal hypoplasia (AHC) due to DAX-1 gene mutations and mutations to the ACTH receptor gene (or related genes, such as in the Triple A or Allgrove syndrome). In biology mutations are changes to the Nucleotide sequence of the Genetic material of an organism In the field of Molecular biology, a transcription factor (sometimes called a sequence-specific DNA binding factor is a Protein that binds to specific sequences X-linked adrenal hypoplasia congenita is a Genetic disorder that mainly affects males The ACTH receptor is a type of Melanocortin receptor (type 2 which is specific for ACTH. Triple-A syndrome (also known as Allgrove Syndrome is a rare Autosomal recessive disorder DAX-1 mutations may cluster in a syndrome with glycerol kinase deficiency with a number of other symptoms when DAX-1 is deleted together with a number of other genes. Glycerol kinase is a Phosphotransferase Enzyme involved in Lipolysis. [2]

Impaired steroidogenesis

To form cortisol, the adrenal gland requires cholesterol, which is then converted biochemically into steroid hormones. Cholesterol is a Lipid found in the Cell membranes and transported in the Blood plasma of all Animals It is an essential component of mammalian Interruptions in the delivery of cholesterol include Smith-Lemli-Opitz syndrome and abetalipoproteinemia. Smith-Lemli-Opitz syndrome (also SLOS, or 7-dehydrocholesterol reductase deficiency) is a metabolic and developmental disorder that affects many Abetalipoproteinemia, or Bassen-Kornzweig syndrome, is a rare Autosomal recessive disorder that interferes with the normal absorption of fat and fat-soluble vitamins Of the synthesis problems, congenital adrenal hyperplasia is the most common (in various forms: 21-hydroxylase, 17α-hydroxylase, 11β-hydroxylase and 3β-hydroxysteroid dehydrogenase), lipod CAH due to deficiency of StAR and mitochondrial DNA mutations. Congenital adrenal hyperplasia ( CAH) refers to any of several Autosomal Recessive Diseases resulting from Mutations Congenital adrenal hyperplasia due to 21-hydroxylase deficiency ( 21-OH CAH) in all its forms accounts for about 95% of diagnosed Congenital adrenal hyperplasia due to 17α-hydroxylase deficiency is an uncommon form of Congenital adrenal hyperplasia resulting from a defect in the Gene for the 11β-Hydroxylase deficient congenital adrenal hyperplasia ( 11β-OH CAH) is an uncommon form of Congenital adrenal hyperplasia resulting from a defect in the Gene 3β-Hydroxysteroid dehydrogenase II deficient congenital adrenal hyperplasia (3βHSD CAH is an uncommon form of CAH resulting from a defective Gene for one of the Lipoid congenital adrenal hyperplasia is an uncommon form of CAH resulting from defects in the earliest stages of adrenal Cortisol synthesis the transport The steroidogenic acute regulatory protein, commonly referred to as StAR ( STARD1) is a Transport protein that regulates Cholesterol transfer Mitochondrial DNA ( mtDNA) is the DNA located in Organelles called mitochondria. [2]

Adrenal destruction

Autoimmune destruction of the adrenal cortex (often due to antibodies against the enzyme 21-Hydroxylase) is a common cause of Addison's in teenagers and adults. 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 Steroid 21- Hydroxylase (or steroid 21-monooxygenase or 21α-Hydroxylase;) is a Cytochrome P450 Enzyme which is involved with the This may be isolated or in the context of autoimmune polyendocrine syndrome (APS type 1 or 2). In Medicine, autoimmune polyendocrine syndromes are a heterogeneous group of Rare diseases characterised by Autoimmune activity against more than one Adrenal destruction is also a feature of adrenoleukodystrophy (ALD), and when the adrenal glands are involved in metastasis (seeding of cancer cells from elsewhere in the body), hemorrhage (e. Adrenoleukodystrophy (ALD (also known as Addison-Schilder Disease or Siemerling-Creutzfeldt Disease) is a rare (1 in 20000 boys inherited disorder that leads to Metastasis ( Greek: displacement μετά=next + στάσις=placement, plural metastases) sometimes abbreviated mets, Cancer (medical term Malignant Neoplasm) is a class of Diseases in which a group of cells display uncontrolled Bleeding, technically known as hemorrhaging / haemorrhaging (see American and British spelling differences) is the loss of Blood from g. in Waterhouse-Friderichsen syndrome or antiphospholipid syndrome), particular infections (tuberculosis, histoplasmosis, coccidioidomycosis), deposition of abnormal protein in amyloidosis. Waterhouse-Friderichsen syndrome ( WFS) is a disease of the Adrenal glands mostly commonly caused by the bacterium Neisseria meningitidis. Antiphospholipid syndrome (APS or APLS or antiphospholipid antibody syndrome is a disorder of Coagulation, which causes blood clots ( Thrombosis) in both An infection is the detrimental Colonization of a host Organism by a foreign Species. Tuberculosis (abbreviated as TB for tubercle bacillus or T u' b' erculosis Bacillus --> is a common Histoplasmosis, also known as Darling's disease, is a Disease caused by the Fungus Histoplasma capsulatum which was discovered in Coccidioidomycosis (also known as Valley fever, San Joaquin Valley Fever, California valley fever, desert fever and (incorrectly In Medicine, amyloidosis refers to a variety of conditions in which Amyloid Proteins are abnormally deposited in organs and/or tissues Some medications interfere with steroid synthesis enzymes (e. g. ketoconazole), while others accelerate the normal breakdown of hormones by the liver (e. Ketoconazole is a synthetic Antifungal drug used to prevent and treat skin and fungal infections especially in immunocompromised patients such as those The liver is a vital organ in the human body and is present in Vertebrates and some other animals g. rifampicin, phenytoin). Rifampicin ( INN) (rɪˈfæmpəsɪn or rifampin ( USAN) is a Bactericidal Antibiotic drug of the Rifamycin group Phenytoin sodium is a commonly used Antiepileptic. Phenytoin acts to dampen the unwanted runaway brain activity seen in seizure by reducing electrical conductance among brain [2]

Treatment

Maintenance treatment

Treatment for Addison's disease involves replacing the missing cortisol, usually in the form of hydrocortisone tablets, in a dosing regimen that mimics the physiological concentrations of cortisol. Cortisol is a Corticosteroid Hormone produced by the Adrenal gland (in the Zona fasciculata of the Adrenal cortex) Alternatively one quarter as much prednisolone may be used for equal glucocorticoid effect as hydrocortisone. Prednisolone is the active metabolite of Prednisone. Uses It is a Corticosteroid drug with predominantly Glucocorticoid and low Mineralocorticoid Glucocorticoids (GC are a class of Steroid hormones characterised by an ability to bind with the glucocorticoid receptor ( GR) and trigger similar effects Treatment must usually be continued for life. In addition, many patients require fludrocortisone as replacement for the missing aldosterone. Fludrocortisone is a synthetic Corticosteroid with moderate Glucocorticoid potency and much greater Mineralocorticoid potency Caution must be exercised when the person with Addison's disease becomes unwell, has surgery or becomes pregnant. Surgery (from the χειρουργική cheirourgikē, via chirurgiae meaning "hand work" is a medical specialty that uses operative manual and instrumental Pregnancy ( Latin graviditas) is the carrying of one or more offspring known as a Fetus or Embryo, inside the Uterus of a Female Medication may need to be increased during times of stress, infection, or injury.

Addisonian crisis

Treatment for an acute attack, an Addisonian crisis, usually involves intravenous (into blood veins) injections of:

Surgery

Surgeries may require significant adjustments to medication regimens prior to, during, and following any surgical procedure. Cortisol is a Corticosteroid Hormone produced by the Adrenal gland (in the Zona fasciculata of the Adrenal cortex) Intravenous therapy or IV therapy is the giving of Liquid substances directly into a Vein. Glucose (Glc a Monosaccharide (or simple Sugar) also known as grape sugar, is an important Carbohydrate in Biology. The best preparation for any surgery, regardless of how minor or routine it may normally be, is to speak to one's primary physician about the procedure and medication implications well in advance of the surgery.

Pregnancy

Many women with Addison's have given birth successfully and without complication, both through natural labor and through cesarean delivery. Both of these methods will require different preventative measures relating to Addison's medications and dosages. As is always the case, thorough communication with one's primary physician is the best course of action. Occasionally, oral intake of medications will cause debilitating nausea and vomiting, and thus the woman may be switched to injected medications until delivery. [4] Addison's treatment courses by the mother are generally considered safe for baby during pregnancy.

Epidemiology

The frequency rate of Addison's disease in the human population is sometimes estimated at roughly 1 in 100,000. [5] Some research and information sites put the number closer to 40-60 cases per 1 million population. (1/25,000-1/16,600)[6] (Determining accurate numbers for Addison's is problematic at best and some incidence figures are thought to be underestimates. [7]) Addison's can afflict persons of any age, gender, or ethnicity, but it typically presents in adults between 30 and 50 years of age. Young women are most affected, outnumbering men by a factor of four. [8] Research has shown no significant predispositions based on ethnicity. [6]

Prognosis

While treatment solutions for Addison's disease are far from precise, overall long-term prognosis is typically good. Because of individual physiological differences, each person with Addison's must work closely with their physician to adjust their medication dosage and schedule to find the most effective routine. Once this is accomplished (and occasional adjustments must be made from time to time, especially during periods of travel, stress, or other medical conditions), symptoms are usually greatly reduced or occasionally eliminated so long as the person continues their dosage schedule.

Canine hypoadrenocorticism

The condition is relatively rare, but has been diagnosed in all breeds of dogs. In general, it is underdiagnosed, and one has to have a clinical suspicion of it as an underlying disorder for many presenting complaints. Females are overrepresented, and the disease often appears in middle age (4-7 years), although any age or gender may be affected.

Hypoadrenocorticism is treated with prednisolone and/or fludrocortisone (Florinef (r)) or a monthly injection called Percorten V (desoxycorticosterone pivlate (DOCP)). Routine blood work is necessary periodically to assess therapy.

Most of the medications used in the therapy of hypoadrenocorticism cause excessive thirst and urination. It is absolutely vital to provide fresh drinking water for the canine sufferer.

If the owner knows about an upcoming stressful situation (shows, traveling etc. ), patients generally need an increased dose of prednisone to help deal with the added stress. Avoidance of stress is important for dogs with hypoadrenocorticism.

Famous Addisonians

See also

References

  1. ^ Thomas Addison [1855]. The ACTH stimulation test (also called the Cosyntropin test or Tetracosactide test) is a Medical test usually ordered and interpreted Thomas Addison (April 1793 - June 29, 1860) was a renowned 19th-century English physician and On The Constitutional And Local Effects Of Disease Of The Supra-Renal Capsules (HTML reprint), London: Samuel Highley.  
  2. ^ a b c d e f g h i j Ten S, New M, Maclaren N (2001). "Clinical review 130: Addison's disease 2001". J. Clin. Endocrinol. Metab. 86 (7): 2909-22. doi:10.1210/jc.86.7.2909. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 11443143.  
  3. ^ Addison's Disease National Endocrine and Metabolic Diseases Information Service. Retrieved on 26 October 2007. Events 740 - An Earthquake strikes Constantinople, causing much damage and death Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century.
  4. ^ addison's disease. Retrieved on 2007-07-25. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 285 - Diocletian appoints Maximian as Caesar, co-ruler
  5. ^ Addison Disease � Health information regarding this hormonal (endocrine) disorder on MedicineNet.com. Retrieved on 2007-07-25. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 285 - Diocletian appoints Maximian as Caesar, co-ruler
  6. ^ a b eMedicine - Addison Disease : Article by Sylvester Odeke. Retrieved on 2007-07-25. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 285 - Diocletian appoints Maximian as Caesar, co-ruler
  7. ^ medhelp. Retrieved on 2007-07-25. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 285 - Diocletian appoints Maximian as Caesar, co-ruler
  8. ^ Volpé, Robert (1990). Autoimmune Diseases of the Endocrine System. CRC Press, 299. ISBN 0849368499.  
  9. ^ Nicholas JA, Burstein CL, Umberger CJ, Wilson PD (November 1955). "Management of adrenocortical insufficiency during surgery". AMA Arch Surg 71 (5): 737–42. PMID 13268224.  
  10. ^ Lord Owen (May 2003). "Diseased, demented, depressed: serious illness in Heads of State". QJM 96 (5): 325–36. PMID 12702781.  
  11. ^ The Australian Addison's Disease Association. Retrieved on 2007-07-25. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 285 - Diocletian appoints Maximian as Caesar, co-ruler
  12. ^ Eugene Shoemaker (1928-1997). Retrieved on 2007-07-25. Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century. Events 285 - Diocletian appoints Maximian as Caesar, co-ruler
  13. ^ Terry Jones. Patron Saints Index: Blessed Elizabeth of the Trinity. Retrieved on 2008-05-04. 2008 ( MMVIII) is the current year in accordance with the Gregorian calendar, a Leap year that started on Tuesday of the Common Events 1256 - The Augustinian monastic order is constituted at the Lecceto Monastery when Pope Alexander IV
  14. ^ Upfal A (2005). "Jane Austen’s lifelong health problems and final illness: New evidence points to a fatal Hodgkin’s disease and excludes the widely accepted Addison's". J Med Ethics Med Humanities 31: 3-11.  
  15. ^ L. Williams et al. (1991). "The Nineteenth Century: Victorian Period". The Year's Work in English Studies 72 (1): 314–360. Oxford University Press. doi:10.1093/ywes/72.1.314. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document.  

External links

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Dictionary

Addison's disease

-noun

  1. (pathology) A morbid condition causing a peculiar brownish discoloration of the skin, and thought, at one time, to be due to disease of the suprarenal capsules (two flat triangular bodies covering the upper part of the kidneys), but now known not to be dependent upon this causes exclusively. It is usually fatal.
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