Internal Medicine

0521779407-19 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:21

1330 Scleroderma

specific therapy

■No specific treatment available. Treat the complications.

■Hypertension: invariably high renin; treat w/ACE inhibitors or

angiotensin receptor blockers

■Pulmonary hypertension: oxygen therapy as indicated; endothelin

receptor antagonist bosentan

■Alveolitis: life-threatening & potentially controllable w/oral cyclo-

phosphamide & low-dose daily corticosteroids. Adverse reactions

include serious bacterial, viral & opportunistic infections, hemor-

rhagic cystitis, transitional cell malignancies, lymphoproliferative

disorders, suppression of gonadal function/infertility

■Esophageal dysfunction: proton pump inhibitor, elevate head of bed

on masonry blocks, esophageal dilatation for strictures

■GI dysmotility: frequent small meals, metoclopramide, antibiotics

for bacterial overgrowth

■Arthritis: analgesics & NSAIDs (selective COX-2 inhibitors for pts

w/esophageal disease)

■Raynaud’s: Avoid cold exposure. Stop smoking. Avoid vasoconstric-

tive agents (decongestants, caffeine, beta blockers, ergot alkaloids,

amphetamines). Calcium channel blockers & sildenafil are both

effective. For severe Raynaud’s, sympathectomy.

■Digital ulcerations: topical or oral antibiotics as necessary for infec-

tions

■Sjogren’s: methylcellulose eye drops, frequent dental prophylaxis,

ophthalmic lubricants, lacrimal duct plugging–consult ophthalmol-

ogist

follow-up

During Treatment

■Attain BP control <130/80, evaluate end-organ function through

blood & urine tests

■PFTs w/lung volumes & diffusing capacity for carbon monoxide,

resting & exercise ABGs

■Some recommend semiannual or annual 2-D echocardiogram to

detect pulmonary hypertension

Routine

■Monitor BP daily if hypertensive, periodically if normal

complications & prognosis

■Renal crisis, now uncommon, includes malignant hypertension,

microangiopathic hemolytic anemia, oliguric renal failure. Consult