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Autoimmune Adrenal Insufficiency

Autoimmune Primary Adrenal Insufficiency

Autoimmune Adrenal Insufficiency - Symptom Checker

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Presentation

  • Case Presentation: A 42 year-old woman with a history of hypothyroidism, remote type II diabetes mellitus, and premature ovarian failure (POF) presented to the Emergency Department of an academic community hospital with the one day of diffuse myalgias[shmabstracts.com]
  • As clinical features were not thoroughly explored in the patients from these reports, clinical presentation of the present patients cannot be compared with those of these patients.[karger.com]
  • The constitutive class II expression on antigen-presenting cells is under the control of the MHC class II transactivator, encoded by the MHC2TA gene, which is mapped to chromosome 16p13.[ncbi.nlm.nih.gov]
  • Our presentation aimed at comparing two groups of patients: 1) 238 patients with AD (183 women, F/M 4.3), aged 8–61 years at time of diagnosis and 2) 301 patients with SAI (269 women, F/M 8.4), aged 17–87 years at time of diagnosis.[endocrine-abstracts.org]
  • A contemporary overview of these conditions with special emphasis on diagnosis is presented.[books.google.com]
Constitutional Symptom
  • Seven patients, although asymptomatic in their daily lives without abnormalities in basal clinical laboratory tests, presented with a variety of nonspecific gastrointestinal or constitutional symptoms under stress-associated circumstances.[karger.com]
Euthyroid
  • All of them were maintained in a euthyroid state.[karger.com]
Swelling of the Finger
  • The pain and swelling of the fingers remitted following GC supplementation (patient 2).[karger.com]
Muscle Weakness
  • Symptoms include Weight loss Muscle weakness Fatigue that gets worse over time Low blood pressure Patchy or dark skin Lab tests can confirm that you have Addison disease. If you don't treat it, it can be fatal.[medlineplus.gov]
  • Symptoms & Causes The most common symptoms are fatigue, muscle weakness, loss of appetite, weight loss, and abdominal pain.[niddk.nih.gov]
  • Clinical symptoms include ANOREXIA; NAUSEA; WEIGHT LOSS; MUSCLE WEAKNESS; and HYPERPIGMENTATION of the SKIN due to increase in circulating levels of ACTH precursor hormone which stimulates MELANOCYTES.[fpnotebook.com]
  • Other common symptoms of adrenal hypofunction include fatigue that keeps getting worse, weight loss, muscle weakness, loss of appetite, headache and irregular or no periods in women. Causes Adrenal hypofunction has more than one cause.[livestrong.com]
  • Clinical symptoms include anorexia; nausea; weight loss; muscle weakness; and hyperpigmentation of the skin due to increase in circulating levels of acth precursor hormone which stimulates melanocytes.[icd10data.com]

Workup

  • A diagnostic workup for adrenal insufficiency should be performed in patients who do not respond to hypothyroidism treatment.[najms.org]
  • Addison Disease workup at eMedicine Further reading [ edit ] Bornstein, SR; Allolio, B; Arlt, W; Barthel, A; Don-Wauchope, A; Hammer, GD; Husebye, ES; Merke, DP; Murad, MH; Stratakis, CA; Torpy, DJ (February 2016).[en.wikipedia.org]
  • Pediatric Adrenal Insufficiency (Addison Disease) Workup. Medscape Reference. Available online at . Accessed February 2017. Johannsson G, et al. Adrenal Insufficiency: Review of Clinical Outcomes With Current Glucocorticoid Replacement Therapy.[labtestsonline.org]
  • Thomas Addison first described the clinical presentation of primary adrenocortical insufficiency in 1855 in his classic paper, On the Constitutional and Local Effects of Disease of the Supra-Renal Capsules. [1] Workup The diagnosis of adrenocortical insufficiency[emedicine.medscape.com]
Hyponatremia
  • Initial laboratory values were notable for hyperkalemia of 9.3 mmol/L, hypoglycemia, hyponatremia, non-anion gap metabolic acidosis, mild peripheral eosinophilia, and random cortisol level of 0.2 micrograms/dL.[shmabstracts.com]
  • Biochemical analyses revealed that hyperkalemia was typical for AD, while hyponatremia was more characteristic for SAI. Additional autoimmune disorders were present in 79% of AD and in 67% in SAI.[endocrine-abstracts.org]
  • Patients with long-standing adrenal insufficiency may present with postural hypotension, nausea, vomiting, weight loss, anorexia, lethargy, depression, and/or chronic hyponatremia.[amboss.com]
  • She was mildly hypotensive and had a “tanned skin” appearance while initial routine laboratory features revealed hyponatremia and hyperkalemia. Subsequent laboratory investigations (including an ACTH stimulation test, rather than endoscopy!)[pubs.sciepub.com]
  • Other testsCBC: anemia, moderate neutropenia, eosinophilia, relative lymphocytosisBMP: hyponatremia (90%), hyperkalemia(65%), hypoglycemia TSH, CXR plasma very long chain fatty acids S/ DHEA levels Plasma renin activity 24.[slideshare.net]

Treatment

  • Make the best clinical decisions with an enhanced emphasis on evidence-based practice and expert opinions on treatment strategies. Zero in on the most relevant and useful references with the aid of a more focused, concise bibliography.[books.google.com]
  • Treatment modalities that improve HRQoL are needed.[portal.research.lu.se]

Prognosis

  • […] adrenalitis): enlargement of both adrenal glands, with necrotic centers and peripheral enhancing rims chronic : both adrenal glands appear small and atrophic, associated with calcifications ( adrenal calcification ) in granulomatous adrenalitis Treatment and prognosis[radiopaedia.org]
  • Clinics in Dermatology 2006; 24(4): 276-280 Links: aetiology associations clinical features investigation management and prognosis self care advice for patients with Addison's disease[gpnotebook.co.uk]
  • The prognosis for any patient with adrenal insufficiency will depend on the underlying cause. In those patients in whom the prognosis is not affected by the underlying pathology, replacement therapy should result in a return to health.[patient.info]
  • However, the prognosis for an untreated patient with adrenal insufficiency (Addison disease) is poor.[emedicine.medscape.com]
  • Outlook and Prognosis for Individuals With Adrenal Insufficiency If treated properly, individuals with adrenal insufficiency can live long, healthy, and active lives.[medstarwashington.org]

Etiology

  • Criteria for defining an autoimmune disease and the main features of autoimmune AD (history, prevalence, etiology, histopathology, clinical and laboratory findings, cell-mediated andhumoral immunity, autoantigens and their autoepitopes, genetics, animal[ncbi.nlm.nih.gov]
  • Etiology of sarcoidosis: does infection play a role? Yale J Biol Med. 2012; 85 (1): 133-41[ PubMed ] 9. Papadopoulos KI, Hornblad Y, Liljebladh H, Hallengren B. High frequency of endocrine autoimmunity in patients with sarcoidosis.[endometabol.com]
  • Furthermore, aldosterone deficiency leads to low levels of sodium and high levels of potassium in the blood. 21-Hydroxylase Antibody Testing A positive 21-hydroxylase antibody test result confirms an autoimmune etiology of PAI, in which the body’s own[arupconsult.com]
  • Still another type of adrenal hypoplasia congenita, an autosomal recessive form of uncertain etiology, has also been described (OMIM 240200 ).[emedicine.medscape.com]
  • Secondary adrenal insufficiency due to lack of stimulation of the gland is a more common etiology overall.[radiopaedia.org]

Epidemiology

  • Assessing the worldwide epidemiology of sarcoidosis: challenges and future directions. Eur Respir J. 2016; 48 (6): 1545-8[ DOI ][ PubMed ] 6. Valeyre D, Prasse A, Nunes H, Uzunhan Y, Brillet PY, Muller-Quernheim J. Sarcoidosis.[endometabol.com]
  • [Polyglandular autoimmune syndrome type II: epidemiology and forms of manifestation]. Dtsch Med Wochenschr. 1999;124(49):1476-81. [PMID:10629665] Comment: Reviews epidemiology of PAS II. Eisenbarth G, Wilson P, Ward F, et al.[hopkinsguides.com]
  • However, only few epidemiological studies are available dealing with endocrine complications in different types of primary immunodeficiency diseases.[ncbi.nlm.nih.gov]
  • 140 per million people and an annual incidence of 4.7-6.2 per million people in Western populations (1). in UK Addison’s disease affects around 1 in 10,000 individuals with an estimate of around 8400 currently diagnosed cases (2) according to a recent epidemiological[gpnotebook.co.uk]
  • Introduction Clinical definition disorder characterized by the loss of adrenal gland function leading to the deficiency of aldosterone (mineralcorticoids), catecholamines, and cortisol can be either acute or chronic depending of the etiology Epidemiology[medbullets.com]
Sex distribution
Age distribution

Pathophysiology

  • References: [1] [2] [3] Pathophysiology Clinical features Most cases of adrenal insufficiency are subclinical and only become apparent during periods of stress (e.g., surgery, trauma, infections), when the cortisol requirement is higher![amboss.com]
  • Pathophysiology and natural course of autoimmune adrenal failure in Europe Project details EU contribution: EUR 2 999 931 Funding scheme: CP-FP - Small or medium-scale focused research project Objective Autoimmune Addison’s disease (AAD) is an endocrine[cordis.europa.eu]
  • [PMID:19411300] Comment: Provides review of the pathophysiology and role of genetic testing in PAS. Eisenbarth GS, Gottlieb PA. Autoimmune polyendocrine syndromes. N Engl J Med. 2004;350(20):2068-79. [PMID:15141045] Dittmar M, Kahaly GJ.[hopkinsguides.com]
  • ., Holländer, G.A. (2009) Clearing the AIRE: On the pathophysiological basis of the autoimmune poyendocrinopathy syndrome type 1.[scindeks.ceon.rs]
  • Predominant sex: females males (slight) No racial predilection Incidence 0.6:100,000 Prevalence 10 to 14: 100,000 Etiology and Pathophysiology Autoimmune adrenal insufficiency ( 80% of cases in the United States) Infectious causes: TB (most common infectious[unboundmedicine.com]

Prevention

  • This product is not intended to diagnose, treat, cure or prevent any disease.[adrenalfatigue.org]
  • Explain preventative measures. Tell the patient that to prevent getting ill, she or he should avoid being in groups with people who are ill and environments where temperatures change from very hot to very cold.[medical-dictionary.thefreedictionary.com]
  • Avoid fruit and sweets before lunch to prevent blood sugar swings early in the day; it helps prevent that afternoon “crash”. Eat every 2-3 hours to keep blood sugar stable.[autoimmunewellness.com]
  • Chronic steroid use, then experiencing severe infection, trauma, or surgical procedures General Prevention No preventive measures known for Addison disease; focus on prevention of complications Anticipate adrenal crisis and treat before symptoms begin[unboundmedicine.com]
  • Prevention Addison's disease can't be prevented, but there are steps you can take to avoid an addisonian crisis: Talk to your doctor if you always feel tired, weak, or are losing weight. Ask about having an adrenal shortage.[mayoclinic.org]

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