{"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 INSUFFICIENCY Rate and Extent of Adrenal Destruction Signs and Symptoms Gender-Specific Etiologies of Primary Adrenal Failure Autoimmune Associations LABORATORY FINDINGS AND TREATMENT OF PRIMARY ADRENAL INSUFFICIENCY Lab Findings Treatment Principles Perioperative [&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","post-wrapper","thrv_wrapper"],"_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}]}}