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Addison’s Disease

Addison’s Disease . NP Rounds May 11, 2010. Case 1. 61 year old man with adrenal insufficiency diagnosed March 2009 PMH: HSV infection of eye: 2005, 2006, 2008 presented to us as new patient with herpetic lesion Raynaud’s syndrome

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Addison’s Disease

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  1. Addison’s Disease NP Rounds May 11, 2010

  2. Case 1 • 61 year old man with adrenal insufficiency diagnosed March 2009 • PMH: HSV infection of eye: • 2005, 2006, 2008 presented to us as new patient with herpetic lesion • Raynaud’s syndrome • FMH: no hx adrenal insufficiency, thyroid dysfunction, brother type 2 DM died of complications age 52 • Medications: prednisone 7.5 mg daily, florinfef 0.05 mg daily, vitamins

  3. HPI continued • Social hx: married, adopted daughter, self-employed builder, non-smoker, 4 drinks/week • Presentation of Addison’s disease • Fatigue, dizziness, lightheadedness • Weight loss, no nausea, vomiting or abd pain • Hypotension • Increased skin pigmentation • No headaches, no change in visual fields, no temperature intolerance • Initial lab work Na 131, low cortisol confirmed by ACTH stimulation test, other labs unremarkable

  4. HPI continued • Labs/Referral for work up for adrenal insufficiency: • TSH, T3/4, fasting glucose, ACTH, testosterone, electrolytes, creatinine, DHEA(S) • Referral to endocrinology to confirm diagnosis and recommendations for management • Confirmed diagnosis based on: TSH 3.88, free T4 9, free T3 4.8, FBG 4.4 ACTH 225, testosterone 23.2, ACTH stimulation 64 to 68 to 71, electrolytes normal • Endo impression: Addison’s, his glucocorticoid replacement is adequate/slightly underreplaced, DHEA is redundant

  5. Plan for ongoing management • Continue with intitalprednison of 5 mg daily • Recheck ACTH level which will never be normalized ideal goal between 15-20 • Glucocorticoid replacement is followed with clinical status, including orthostatics (BP), creatinine and electrolytes • Treated with fludrocotrisone (florinef) 0.05 mg daily to improve BP to 110 systolic – this med is a mineralocorticoid replacement • If ACTH remains on high side increase prednisone to 6 mg daily • 2x2 rule – double his dose of prednisone for at least 2 days if unwell – critical element of management to above adrenal crisis, if unable to tolerate fluids/solids by mouth go immediately to hospital

  6. Addison’s disease • Is uncommon disorder caused by the destruction/dysfunction of the adrenal cortices • It is characterized by chronic deficiency of cortisol, aldosterone and adrenal androgens • It causes skin pigmentation that is subtle or stikingly dark • Volume and sodium depletion with potassium excess eventually occur in primary adrenal failure

  7. Addison’s disease • Etiology: autoimmune destruction is most common cause, most other causes are rare • Clinical signs and symptoms: weakness, fatigue, myalgias, arthralgias, fever, anorexia, nausea, vomiting, anxiety, mental irritability • Lab findings: moderate neutropenia, lymphocytosis, and total eosinophil count over 300, low Na in 90% cases, low plasma cortisol am, ACTH level above 200 confirmed with stimulation test, low DHEA levels with antiadrenal antibodies in 50% cases

  8. Addison’s disease • Hydrocortisone is drug of choice 15-25 mg with some patients responding better to prednisone, with adjustments in dose made according to clinical response – a prper dose usually results in nroma differential white count • Fludrocortisone acetate is often required as many patients don’t obtain sufficient salt-retaining effect – used in doses 0.05-0.3 mg with dose lowered for postural hypotension, hypoatremia, or hyperkalemia • All infections should be treated immediately and vigorously

  9. Case 2 • 77 year-old woman who joined our practice in Dec 2008 with a diagnosis of Addison’s • 2 episodes of adrenal crises in 2002 and 2007 – 1st episode flu-like symptoms precipitated coma x 5 days, 2nd episode post opt TKR missed medication, coma resulting in increase predisone – 2x2 rule • Initial presentations to clinic for GERD, hypertension and MSK issues • Asked her to return for CPX, referred back to endocrinologist managing care in Victoria

  10. Recommendations for management • My consult letter • Endocrinologist’s response • Increased history: Cushing syndrome 1960 ? Diagnosis correct, sounded like bilateral hyperplasia/adenomas of pituitary, surgically resected • Adrenal crises 2001 flu-like event, 2005 first TKR not give post-op steroids • Other history – cataracts, HTN, back surgery • Current meds: cortisone acetate 25 mg daily, Vit D, Ca, other vitamins • Allergic to penicillin • Ex-smoker, occasional drink • FMH: son has hypothyroidism, daughter thyroidism

  11. Management • Adrenal insufficiency – clinically well, electrolytes normal • TSH normal • Bone density change secondary to more than 6 months of additional prednisone post TKR – he felt would remain same or increase with cortisone • Vit D checked was low so increased dose and PTH normal • HTN situational

  12. Follow up Feb 2010 • Patient has been well with no adrenal problems • BP remains high • TSH 5.90 Na 132-134 • Impressions: adrenal insufficienty secondary to bilateral adrenaletomy in 60s – now believes she does not have Addison’s and feels she is adequately replaced – empty sella so ACTH levels of little benefit – question of mineralocorticoid deficiency raised by BP actually on high side • Recommended rechecking DEXA March 2011 – if continues to deteriorate consider bone preserving agent • Trial of coversyl 2 mg daily if remains hypertensive and recheck creat and lytes

  13. Update • BP improved – no need to add anti-hypertensive • Normal Na and K+ • No adrenal crises since moving to Cortes • Regular standing orders for labs and follow up

  14. Questions? • Web resources • http://www.addisonsociety.ca/b resources • http://www.topalbertadoctors.org/informed_practice/cpgs/addisons_disease.html • http://www.healthlinkbc.ca/kb/content/mini/hw65865.html

  15. Update • By Nov prednisone increased to 7.5 mg daily due to ACTH • Ongoing regular labs to monitor CBC, TSH, ACTH, Cortisol am, DHEA-S, FBG, creat, lytes • Oct 09 ACTH 13 Mar 24 135 with TSH 3.88 to 4.6, normal Na – advised to see endo – his recommendations: • Addison’s – glucocorticoid axis adequately replaced clinically although ACTH little on high side, slightly underreplaed on mineralcorticoid axis so increase florinef to 0.075 mg daily to improve salt craving and BP, recheck lytes 1/12 • Rising TSH – known autoimmunity, will check auto antibodies

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