Addison’s Disease

NP Rounds May 11, 2010

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

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

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

Continue with intital prednison 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

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

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

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

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

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

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

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

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

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

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