Transcript Addison`s Disease

NP Rounds May 11, 2010
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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
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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
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Social hx: married, adopted daughter, selfemployed 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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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BP improved – no need to add antihypertensive
Normal Na and K+
No adrenal crises since moving to Cortes
Regular standing orders for labs and follow
up
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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
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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