{"id":4422407,"date":"2025-01-11T17:41:59","date_gmt":"2025-01-11T23:41:59","guid":{"rendered":"https:\/\/myendoconsult.com\/learn\/topics\/addisons-disease\/"},"modified":"2025-01-12T16:26:57","modified_gmt":"2025-01-12T22:26:57","slug":"addisons-disease","status":"publish","type":"oen_topic","link":"https:\/\/myendoconsult.com\/learn\/topics\/addisons-disease\/","title":{"rendered":"Addison&#8217;s Disease"},"content":{"rendered":"\n<h2 class=\"wp-block-heading\">ACUTE ADRENAL FAILURE\u2014ADRENAL CRISIS<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Definition and Importance<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Acute adrenal failure (adrenal crisis)<\/strong> is an <strong>endocrine emergency<\/strong>, potentially fatal if untreated.<\/li>\n\n\n\n<li><strong>Dominant features<\/strong>: Dehydration and <strong>cardiovascular collapse<\/strong> (shock).<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Clinical Settings for Adrenal Crisis<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Known <a href=\"https:\/\/myendoconsult.com\/learn\/primary-adrenal-insufficiency-sick-day-rules\/\" data-wpil-monitor-id=\"189\">Primary Adrenal Insufficiency<\/a><\/strong>\n<ul class=\"wp-block-list\">\n<li>Omission of glucocorticoid therapy.<\/li>\n\n\n\n<li>Inadequate dose increase during physical stress (e.g., infection, surgery).<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Undiagnosed <a href=\"https:\/\/myendoconsult.com\/learn\/courses\/diagnosis-and-treatment-of-primary-adrenal-insufficiency\/\" data-wpil-monitor-id=\"190\">Primary Adrenal Insufficiency<\/a><\/strong>\n<ul class=\"wp-block-list\">\n<li>Undergoing major stress without prior recognition (acute infection, trauma, etc.).<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Necrosis of Adrenals<\/strong>\n<ul class=\"wp-block-list\">\n<li>Intra-adrenal hemorrhage\/infarction.<\/li>\n\n\n\n<li>May be caused by infection, trauma, anticoagulant therapy, or coagulopathy.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Secondary Adrenal Insufficiency<\/strong> (less common cause)\n<ul class=\"wp-block-list\">\n<li>Intact mineralocorticoid secretion usually prevents crisis, but severe stress or abrupt withdrawal of high-dose exogenous glucocorticoids can precipitate.<\/li>\n<\/ul>\n<\/li>\n<\/ol>\n\n\n\n<h3 class=\"wp-block-heading\">Adrenal Hemorrhage<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Consider<\/strong> in shock with infection, trauma, anticoagulation (heparin\/warfarin), or coagulopathy (e.g., antiphospholipid syndrome).<\/li>\n\n\n\n<li><strong>Possible back\/flank\/abdominal pain<\/strong> from intra-adrenal bleeding.<\/li>\n\n\n\n<li><strong>Fulminant Meningococcemia<\/strong>: Hemorrhagic destruction of both adrenals \u2192 <strong>Waterhouse-Friderichsen syndrome<\/strong> (especially children\/young adults).\n<ul class=\"wp-block-list\">\n<li>Presents with purpura, meningitis, shock, DIC, purpura fulminans.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Clinical Presentation<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Shock<\/strong>: Dehydration, hypotension (circulatory collapse).<\/li>\n\n\n\n<li><strong>Additional Symptoms<\/strong>: Anorexia, nausea, vomiting, abdominal pain, fever, confusion, lethargy.<\/li>\n\n\n\n<li><strong>Possible Preexisting Adrenal Failure<\/strong> signs: Hyperpigmentation, weight loss, hyponatremia, hyperkalemia.<\/li>\n\n\n\n<li><strong>Misdiagnosis Risk<\/strong>: Fever + abdominal pain can mimic an acute surgical abdomen \u2192 potentially disastrous unnecessary surgery.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Empiric Treatment<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Consider<\/strong> in severely ill patients with shock refractory to fluids and pressors.<\/li>\n\n\n\n<li><strong>Do not delay<\/strong> therapy for diagnostic test results if <a href=\"https:\/\/myendoconsult.com\/learn\/adrenal-crisis-in-addisons-disease\/\" data-wpil-monitor-id=\"191\">adrenal crisis<\/a> is suspected.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Treatment for Adrenal Crisis<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Glucocorticoid Replacement<\/strong>\n<ul class=\"wp-block-list\">\n<li>Hydrocortisone sodium succinate: 100 mg IV bolus initially.<\/li>\n\n\n\n<li>Continue 100 mg IV q6\u20138h until resolution of precipitating illness, then taper.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Volume Repletion<\/strong>\n<ul class=\"wp-block-list\">\n<li>Dextrose in isotonic saline (2\u20134 L over 4 hours), adjusted for comorbidities and response.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Identify Underlying Cause<\/strong>\n<ul class=\"wp-block-list\">\n<li>Search for infection, hemorrhage, infarction, or other triggers.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Monitoring<\/strong>\n<ul class=\"wp-block-list\">\n<li>Frequent checks of serum electrolytes, acid\u2013base status, glucose, renal function.<\/li>\n<\/ul>\n<\/li>\n<\/ol>\n\n\n\n<figure class=\"wp-block-image size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"3284\" height=\"2402\" src=\"https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/addisons-emergency.png\" alt=\"\" class=\"wp-image-4422797\" srcset=\"https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/addisons-emergency.png 3284w, https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/addisons-emergency-300x219.png 300w, https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/addisons-emergency-768x562.png 768w, https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/addisons-emergency-1536x1123.png 1536w, https:\/\/myendoconsult.com\/learn\/wp-content\/uploads\/addisons-emergency-2048x1498.png 2048w\" sizes=\"auto, (max-width: 3284px) 100vw, 3284px\" \/><figcaption class=\"wp-element-caption\">Addison&#8217;s disease<\/figcaption><\/figure>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">CHRONIC ADRENAL INSUFFICIENCY<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Rate and Extent of Adrenal Destruction<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>&gt;90% cortical destruction<\/strong> needed for overt clinical insufficiency.<\/li>\n\n\n\n<li><strong>Slow Progression<\/strong>: May remain subclinical until precipitated by stress (infection, trauma, surgery).<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Signs and Symptoms<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Both Glucocorticoid &amp; Mineralocorticoid Deficiency<\/strong> (Primary Failure):\n<ul class=\"wp-block-list\">\n<li>Fatigue, generalized weakness, diffuse myalgias, arthralgias.<\/li>\n\n\n\n<li>Anorexia, weight loss, nausea, vomiting, abdominal pain.<\/li>\n\n\n\n<li>Psychiatric changes (irritability, depression, apathy).<\/li>\n\n\n\n<li><strong>Postural hypotension<\/strong>, hyperpigmentation, salt craving.<\/li>\n\n\n\n<li><strong>Hyponatremia<\/strong>, <strong>hyperkalemia<\/strong>, mild normocytic anemia.<\/li>\n\n\n\n<li><strong>Hypoglycemia<\/strong> (more common in children).<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Hyperpigmentation<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Increased ACTH \u2192 MSH effect.<\/li>\n\n\n\n<li>Darkening of friction areas (knees, knuckles, elbows), belt line, scars, inner lip surfaces, palmar creases, areola, freckles.<\/li>\n\n\n\n<li>Vitiligo (~20% of autoimmune Addison\u2019s) from autoimmune destruction of melanocytes.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Gender-Specific<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Women<\/strong>: Loss of axillary &amp; pubic hair, \u2193libido due to low adrenal androgens.<\/li>\n\n\n\n<li><strong>Men<\/strong>: Testicular androgens \u2192 minimal androgen deficiency signs.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Etiologies of Primary Adrenal Failure<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Autoimmune Adrenalitis<\/strong> (~80% in developed countries).<\/li>\n\n\n\n<li><strong>Infections<\/strong> (tuberculosis historically; fungal, HIV).<\/li>\n\n\n\n<li><strong>Metastatic Cancer<\/strong> (e.g., lung, breast, melanoma).<\/li>\n\n\n\n<li><strong>Adrenal Hemorrhage<\/strong> (trauma, antiphospholipid syndrome).<\/li>\n\n\n\n<li><strong>Adrenoleukodystrophy<\/strong>.<\/li>\n\n\n\n<li><strong>Bilateral Adrenalectomy<\/strong>.<\/li>\n\n\n\n<li><strong>Drug-Induced<\/strong> (mitotane, ketoconazole).<\/li>\n\n\n\n<li><strong>Congenital<\/strong> (adrenal hypoplasia e.g., DAX1 or SF-1 mutations).<\/li>\n<\/ol>\n\n\n\n<h3 class=\"wp-block-heading\">Autoimmune Associations<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>21-Hydroxylase Antibodies<\/strong>: Nearly universal in autoimmune Addison\u2019s.<\/li>\n\n\n\n<li><strong>Polyglandular Autoimmune Syndrome Type II (APS2)<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Addison\u2019s + autoimmune thyroid disease + type 1 <a href=\"https:\/\/myendoconsult.com\/learn\/diabetes-mellitus\/\" data-wpil-monitor-id=\"187\">diabetes mellitus<\/a>.<\/li>\n\n\n\n<li>More common in women, onset 20\u201340 years old.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Polyglandular Autoimmune Syndrome Type I (APS1)<\/strong>:\n<ul class=\"wp-block-list\">\n<li>Rare, autosomal recessive (AIRE gene mutations).<\/li>\n\n\n\n<li>Triad: Hypoparathyroidism, chronic mucocutaneous candidiasis, Addison\u2019s.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">LABORATORY FINDINGS AND TREATMENT OF PRIMARY ADRENAL INSUFFICIENCY<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Lab Findings<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Low Cortisol<\/strong>, <strong>High ACTH<\/strong>.<\/li>\n\n\n\n<li><strong>Hyponatremia<\/strong>, <strong>Hyperkalemia<\/strong> (loss of <a href=\"https:\/\/myendoconsult.com\/learn\/topics\/aldosteronism\/\" data-wpil-monitor-id=\"188\">aldosterone<\/a>).<\/li>\n\n\n\n<li>Mild metabolic acidosis, azotemia from volume depletion.<\/li>\n\n\n\n<li>Low DHEA-S, androstenedione \u2192 female loss of body hair.<\/li>\n\n\n\n<li><strong>Cosyntropin Stimulation Test<\/strong>: No significant rise in cortisol.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Treatment Principles<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Glucocorticoid Replacement<\/strong>\n<ul class=\"wp-block-list\">\n<li>Hydrocortisone, cortisone acetate, or prednisone.<\/li>\n\n\n\n<li><em>Stress Dosing<\/em> for major infections, surgery, trauma (2\u20133\u00d7 usual dose).<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Mineralocorticoid Replacement<\/strong>\n<ul class=\"wp-block-list\">\n<li><strong>Fludrocortisone<\/strong> (50\u2013200 \u00b5g daily) to normalize serum K\u207a, Na\u207a, BP.<\/li>\n\n\n\n<li>Adjust dose by checking for hypokalemia, edema, or persistent hyperkalemia\/hyponatremia.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Patient Education<\/strong>\n<ul class=\"wp-block-list\">\n<li>Dose adjustments during illness, use of IM injection if vomiting.<\/li>\n\n\n\n<li>Medical identification (bracelet\/card \u201cadrenal insufficiency\u2014give cortisone\u201d).<\/li>\n\n\n\n<li>Keep emergency dexamethasone syringes for self-injection.<\/li>\n<\/ul>\n<\/li>\n<\/ol>\n\n\n\n<h3 class=\"wp-block-heading\">Perioperative Management<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Surgery with General Anesthesia<\/strong>: Stress dose coverage (e.g., methylprednisolone 20\u201340 mg IM pre-op, taper over 2\u20133 days).<\/li>\n\n\n\n<li><strong>No Additional Mineralocorticoid<\/strong> typically needed until oral intake resumes.<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">LABORATORY FINDINGS AND TREATMENT OF SECONDARY ADRENAL INSUFFICIENCY<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Key Distinctions from Primary<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>No Hyperpigmentation<\/strong>: ACTH is low\/normal (no MSH effect).<\/li>\n\n\n\n<li><strong>Aldosterone Levels<\/strong> usually <em>normal<\/em> (RAAS intact) \u2192 minimal hyperkalemia or salt craving.<\/li>\n\n\n\n<li><strong>Possible Hyponatremia<\/strong>: due to impaired free-water excretion, elevated vasopressin.<\/li>\n\n\n\n<li><strong>May Present with<\/strong> pituitary\/hypothalamic mass symptoms or other pituitary hormone deficits (e.g., hypothyroidism, hypogonadism).<\/li>\n<\/ol>\n\n\n\n<h3 class=\"wp-block-heading\">Lab Findings<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Low Cortisol<\/strong>, <strong>Low\/Undetectable ACTH<\/strong> \u2192 diagnosing certain if cortisol &lt;5 \u00b5g\/dL at 8 am with severely low ACTH.<\/li>\n\n\n\n<li><strong>Cosyntropin Stimulation<\/strong> may show a <em>delayed rise<\/em> but peak &lt;18 \u00b5g\/dL.<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Treatment<\/h3>\n\n\n\n<ol class=\"wp-block-list\">\n<li><strong>Glucocorticoid Replacement<\/strong>\n<ul class=\"wp-block-list\">\n<li>No mineralocorticoid needed (aldosterone normal).<\/li>\n\n\n\n<li>Hydrocortisone or equivalent, often 2\/3 AM, 1\/3 PM dosing.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Avoid<\/strong> Over- or Undertreatment\n<ul class=\"wp-block-list\">\n<li>Monitor clinical signs.<\/li>\n<\/ul>\n<\/li>\n\n\n\n<li><strong>Stress Dosing<\/strong> for severe illness or surgery.<\/li>\n\n\n\n<li><strong>If Additional Pituitary Deficits<\/strong>: Manage hypothyroidism, hypogonadism, etc. <em>after<\/em> ensuring adequate cortisol replacement.<\/li>\n<\/ol>\n","protected":false},"excerpt":{"rendered":"<p>ACUTE ADRENAL FAILURE\u2014ADRENAL CRISIS Definition and Importance Clinical Settings for Adrenal Crisis Adrenal Hemorrhage Clinical Presentation Empiric Treatment Treatment for Adrenal Crisis CHRONIC ADRENAL&hellip;<\/p>\n","protected":false},"featured_media":0,"template":"","oen_topic_chapter":[684],"class_list":["post-4422407","oen_topic","type-oen_topic","status-publish","hentry","oen_topic_chapter-adrenal-gland"],"_links":{"self":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422407","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic"}],"about":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/types\/oen_topic"}],"version-history":[{"count":4,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422407\/revisions"}],"predecessor-version":[{"id":4422799,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic\/4422407\/revisions\/4422799"}],"wp:attachment":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/media?parent=4422407"}],"wp:term":[{"taxonomy":"oen_topic_chapter","embeddable":true,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/oen_topic_chapter?post=4422407"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}