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| __NOTOC__ | | __NOTOC__ |
| {{Adrenal insufficiency}} | | {{Adrenal insufficiency}} |
| {{CMG}}; {{AE}} {{Ayeesha}} | | {{CMG}}; {{AE}} {{Ayeesha}} |
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| | '''Synonyms and keywords:''' Addison's disease |
| ==[[Adrenal insufficiency overview|Overview]]== | | ==[[Adrenal insufficiency overview|Overview]]== |
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| ==[[Adrenal insufficiency historical perspective|Historical Perspective]]== | | ==[[Adrenal insufficiency historical perspective|Historical Perspective]]== |
| ==Classification==
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| '''[[Adrenal insufficiency]]''' is classified based on the location of the [[pathology]] into: <ref>{{cite web |url=https://www.ncbi.nlm.nih.gov/books/NBK279083/ |title=Adrenal Insufficiency - Endotext - NCBI Bookshelf |format= |work= |accessdate=}}</ref>
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| *'''[[Primary adrenal insufficiency]]'''
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| *'''[[Secondary adrenal insufficiency]]'''
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| *'''Tertiary [[adrenal insufficiency]]'''
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| In [[Primary adrenal insufficiency]], the [[pathology]] lies in the [[adrenal glands]] leading to decreased production of [[cortisol]] and [[aldosterone]].The most common cause of [[primary adrenal insufficiency]] is [[autoimmune adrenalitis]]. In [[secondary adrenal insufficiency]], the [[pathology]] lies in the [[pituitary gland]] leading to reduced [[ACTH]] production, whereas in tertiary [[adrenal insufficiency]] the [[pathology]] lies in the [[hypothalamus]] leading to reduced [[CRH production]]. The most common cause of [[tertiary adrenal insufficiency]] is chronic [[glucocorticoid]] therapy. Secondary and tertiary [[adrenal insufficiency]] together are categorised into central [[adrenal insufficiency]]. The following table summaries the causes of adrenal insufficiency.
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| {| class="wikitable"
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| ! colspan="3" |<font size="+2">Causes of [[adrenal insufficiency]]<font>
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| |'''<font size="+1">[[Primary adrenal insufficiency]]'''<br>'''([[Addison's disease]])'''<font>
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| |'''<font size="+1>[[Secondary adrenal insufficiency]]'''<font>
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| |'''<font size="+1>[[Tertiary adrenal insufficiency]]'''<font>
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| *'''[[Autoimmune]]:'''
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| **Sporadic
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| **[[Autoimmune polyendocrine syndrome type 1]]<br>(APS type 1)
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| **[[Autoimmune polyendocrine syndrome type 2]]<br>(Schmidt's syndrome)
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| *'''[[Infections]]:'''
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| **[[Tuberculosis]],
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| **[[Fungal infections]],
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| **[[Cytomegalovirus]],
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| **[[HIV]]
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| *'''[[Adrenal]] [[Metastasis]]'''
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| *'''[[Adrenal]] infiltration'''
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| *'''[[Adrenal hemorrhage]]:'''
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| **[[Waterhouse-Friderichsen syndrome]],
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| **Drugs - Anticoagulants- [[Heparin]], [[Warfarin]])
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| *'''[[Adrenoleukodystrophy]]'''
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| *'''Congenital adrenal hypoplasia'''
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| *'''Bilateral adrenelectomy'''
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| *'''[[Pituitary]] [[tumors]]'''
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| *'''[[Iatrogeneic]]:'''
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| **[[Pituitary]] [[surgery]],
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| **[[Pituitary]] [[radiation]]
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| *'''[[Granulomatous diseases]]:'''
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| **[[Tuberculosis]]
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| **[[Sarcoidosis]]
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| **[[Histiocytosis]]
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| **[[Wegener's granulomatosis]]
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| *'''[[Metastasis]]'''
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| *'''[[Pituitary apoplexy]]'''
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| *'''[[Sheehan's syndrome]]'''<br>(Peripartum pituitary necrosis)
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| *'''[[Genetic]]:'''
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| **Isolated [[ACTH]] deficiency,
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| **Multiple pituitary hormone deficiencies
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| *'''Trauma'''
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| *'''[[Glucocorticoid]] therapy'''
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| *'''[[Cushing's syndrome]]'''<br>(Excess endogenous [[glucocorticoid]] production)
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| *'''Drugs:'''
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| **[[Mifepristone]]
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| **[[Chlorpromazine]]
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| **[[Imipramine]]
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| *'''[[Hypothalamic]] tumors'''<br>(Primary and metastatic)
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| *'''[[Iatrogenic]]:'''
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| **[[Hypothalamic]] [[surgery]]
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| **[[Hypothalamic]] [[radiation]]
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| *'''[[Granulomatous diseases]]:'''
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| **[[Tuberculosis]]
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| **[[Sarcoidosis]]
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| **[[Histiocytosis]]
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| **[[Wegener's granulomatosis]]
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| *'''Trauma'''
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| |} | | ==[[Adrenal insufficiency classification|Classification]]== |
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| ==[[Adrenal insufficiency pathophysiology|Pathophysiology]]== | | ==[[Adrenal insufficiency pathophysiology|Pathophysiology]]== |
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| The [[pathogenesis]] of [[adrenal insufficiency]] varies based on the [[etiology]] as follows: <ref> {{cite web |url=https://www.ncbi.nlm.nih.gov/books/NBK441832/ |title=Adrenal Insufficiency - StatPearls - NCBI Bookshelf |format= |work= |accessdate=}} </ref>
| | ==[[Adrenal insufficiency differential diagnosis|Differential diagnosis]]== |
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| <font size="+1">'''[[Autoimmune adrenalitis]]'''</font>: Humoral as well as cell mediated immune mechanisms attack various enzymes involved in the synthesis of adrenal cortical enzymes. Strong genetic association has bene seen with '''HLA DR3/DQ2''' and '''DR4/DQ8'''. On [[gross anatomy]] the [[adrenal gland]] is atrophied with preservation of [[adrenal medulla]]. [[Histopathology]] shows lymphocytic infiltration with fibrosis of the parenchyma. The patients are asymptomatic until up to 90% of the cortex is destroyed. Autoantibodies against '''21-hydroxylase''', an essential enzyme required in the biosynthesis of steroid hormones of the adrenal cortex are seen.
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| *'''Isolated autoimmune adrenalitis''' accounts for 30-40% cases.
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| *'''Autoimmune Polyglandular Syndrome (APS)''' account for 60-70%. Which is further subclassified as follows:
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| '''Autoimmune Polyglandular Syndrome Type 1 (APS type 1)''': Exhibits [[autosomal recessive]] mode of inheritance. It is also known as Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED). Caused due to mutations in the autoimmune regulator gene (AIRE). Apart from [[adrenal insufficiency]] it presents with chronic [[mucocutaneous candidiasis]], [[hypoparathyroidism]], total [[alopecia]].
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| '''Autoimmune Polyglandular Syndrome Type 2 (APS type 2)''': It is more common than APS type 1 and has polygenic inheritance. Strong association has been shown with '''HLA DR3''' of '''MHC'''. Apart from [[adrenal insufficiency]] it presents with [[autoimmune thyroiditis]], [[vitiligo]], premature ovarian failure, [[type 1 diabetes mellitus]], [[pernicious anemia]].
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| <font size="+1">'''[[X linked Adrenoleukodystrophy(X-ALD)]]'''</font>:
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| X-ALD occurs due to [[mutations]] in the [[peroxisomal]] ATP-binding cassette (ABC) transporter encoded by the ABCD1 gene. Disruption of this transport protein leads to the accumulation of [[Very Long Chain Fatty Acids]] (VLCFA). Male patients usually present in childhood or adolescence, whereas heterozygous females present between 40-50 years. The phenotypic expression is variable and can present as pre-symptomatic, cerebral inflammatory demyelination, myelopathy, adrenal insufficiency. The lifetime prevalence of adrenal insufficiency is 80% in males, with the highest risk being in the first decade. Adrenal insufficiency is extremely rare in females. <ref name="pmid24316281">{{cite journal| author=Berger J, Forss-Petter S, Eichler FS| title=Pathophysiology of X-linked adrenoleukodystrophy. | journal=Biochimie | year= 2014 | volume= 98 | issue= | pages= 135-42 | pmid=24316281 | doi=10.1016/j.biochi.2013.11.023 | pmc=3988840 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24316281 }} </ref>
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| <font size="+1"> '''Chronic glucocorticoid use'''</font>:
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| [[Tertiary adrenal insufficiency]] induced by chronic oral and inhaled glucocorticoid use is the most common cause of [[adrenal insufficiency]]. Exogenous glucocorticoid use leads to the feedback inhibition of the '''[[HPA]]''' axis leading to reduced synthesis of '''[[CRH]]''' and '''[[ACTH]]''' by [[hypothalamus]] and [[pituitary]]. As a consequence of reduced '''[[ACTH]]''', the [[adrenal cortex]] slowly loses the ability to synthesize [[cortisol]]. The [[mineralocorticoid]] synthetic function of the adrenal cortex is retained as it depends on '''[[RAAS]]'''. '''[[HPA]]''' axis function recovers quickly if [[glucocorticoids]] were used for less than 10-14 days. If [[glucocorticoids]] were used for >2weeks, weaning and assessment of '''[[HPA]]''' integrity are recommended. In some cases the '''[[HPA]]''' axis may remain suppressed for as long as 6-12 months after [[glucocorticoid]] withdrawal. <ref name="pmid29184808">{{cite journal| author=Younes AK, Younes NK| title=Recovery of steroid induced adrenal insufficiency. | journal=Transl Pediatr | year= 2017 | volume= 6 | issue= 4 | pages= 269-273 | pmid=29184808 | doi=10.21037/tp.2017.10.01 | pmc=5682381 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29184808 }} </ref>. <ref> {{cite web |url=https://www.ncbi.nlm.nih.gov/books/NBK279156/ |title=Glucocorticoid Therapy and Adrenal Suppression - Endotext - NCBI Bookshelf |format= |work= |accessdate=}} </ref>
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| The other causes of adrenal insufficiency are due to the destruction of the [[adrenal]], [[pituitary]] or [[hypothalamus]] due to various causes as mentioned in the table above.
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| ==[[Adrenal insufficiency causes|Causes]]==
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| ==[[Adrenal insufficiency differential diagnosis|Differentiating Xyz from other Diseases]]== | |
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| ==[[Adrenal insufficiency epidemiology and demographics|Epidemiology and Demographics]]==
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| ==[[Adrenal insufficiency risk factors|Risk Factors]]==
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| ==[[Adrenal insufficiency screening|Screening]]==
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| ==[[Adrenal insufficiency natural history, complications and prognosis|Natural History, Complications and Prognosis]]== | | ==[[Adrenal insufficiency epidemiology and demographics |Epidemiology and Demographics]]== |
| | ==[[Adrenal insufficiency risk factors | Risk factors]]== |
| | ==[[Adrenal insufficiency screening | Screening]]== |
| | ==[[Adrenal insufficiency natural history, complications and prognosis | Natural History, Complications and Prognosis]]== |
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| ==Diagnosis== | | ==Diagnosis== |
| [[Adrenal insufficiency diagnostic study of choice|Diagnostic study of choice]] | [[Adrenal insufficiency history and symptoms|History and Symptoms]] | [[Adrenal insufficiency physical examination|Physical Examination]] | [[Adrenal insufficiency laboratory findings|Laboratory Findings]] | [[Adrenal insufficiency electrocardiogram|Electrocardiogram]] | [[Adrenal insufficiency x ray|X-Ray Findings]] | [[Adrenal insufficiency echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Adrenal insufficiency CT scan|CT-Scan Findings]] | [[Adrenal insufficiency MRI|MRI Findings]] | [[Adrenal insufficiency other imaging findings|Other Imaging Findings]] | [[Adrenal insufficiency other diagnostic studies|Other Diagnostic Studies]] | | [[Adrenal insufficiency diagnostic study of choice|Diagnostic study of choice]] | [[Adrenal insufficiency history and symptoms|History and Symptoms]] | [[Adrenal insufficiency physical examination|Physical Examination]] | [[Adrenal insufficiency laboratory findings|Laboratory Findings]] | [[Adrenal insufficiency electrocardiogram|Electrocardiogram]] | [[Adrenal insufficiency x ray|X-Ray Findings]] | [[Adrenal insufficiency echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Adrenal insufficiency CT scan|CT Scan Findings]] | [[Adrenal insufficiency MRI|MRI Findings]] | [[Adrenal insufficiency other imaging findings|Other Imaging Findings]] | [[Adrenal insufficiency other diagnostic studies|Other Diagnostic Studies]] |
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| ==Treatment== | | ==Treatment== |
| [[Adrenal insufficiency medical therapy|Medical Therapy]] | [[Adrenal insufficiency interventions|Interventions]] | [[Adrenal insufficiency surgery|Surgery]] | [[Adrenal insufficiency primary prevention|Primary Prevention]] | [[Adrenal insufficiency secondary prevention|Secondary Prevention]] | [[Adrenal insufficiency cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Adrenal insufficiency future or investigational therapies|Future or Investigational Therapies]]
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| ==Case Studies==
| | [[Adrenal insufficiency medical therapy|Medical Therapy]] | [[Adrenal insufficiency surgery|Surgery]] | [[Adrenal insufficiency primary prevention|Primary Prevention]] | [[Adrenal insufficiency secondary prevention|Secondary Prevention]] | [[Adrenal insufficiency cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Adrenal insufficiency future or investigational therapies|Future or Investigational Therapies]] |
| [[Adrenal insufficiency case study one|Case #1]] | |
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