Internal Medicine

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

Scleroderma 1329

■Bloating & emesis due to intestinal paresis, wide-mouthed colonic

diverticula, malabsorption due to intestinal bacterial overgrowth

■Dyspnea from isolated pulmonary hypertension (CREST syndrome),

interstitial pulmonary fibrosis, alveolitis, heart failure due to myocar-

dial fibrosis

■Chest pain & angina (myocardial infarctions w/o coronary artery

disease), pericarditis w/arrhythmias & effusions

■Hypertension, mild proteinuria, scleroderma renal crisis

■Numbness due to neuropathies, facial pain & numbness due to tri-

geminal neuralgia

tests

Basic Tests

■Assess end-organ involvement through CBC, serum creatinine, uri-

nalysis

Specific Diagnostic Tests

■Antinuclear antibody (ANA) positive in >85%, typically nucleolar pat-

tern. Specificities included antibodies against topoisomerase Scl-70,

centromere, RNA polymerases I & III, fibrillarin, nucleolus organizer

region protein. Sensitivity approx. 20–40%, specificity 95+%.

■Anticentromere ANAs w/CREST syndrome

■Anti-ribonucleoprotein w/diffuse & limited scleroderma

Other Tests as Appropriate

■Pulmonary function tests (PFTs) including lung volumes & diffusing

capacity, possibly high-resolution CT scan to detect inflammatory

alveolitis (ground-glass appearance)

differential diagnosis

■Raynaud’s phenomenon alone, due to exposures listed above, or

w/SLE or other inflammatory rheumatic disease

■Finger contractures w/longstanding poorly controlled diabetes

(cheiropathy)

management

What to Do First

■Check blood pressure & obtain blood tests for end-organ involve-

ment

■Teach pt about potentially serious but treatable hypertension, lung

& GI problems

■Consult rheumatologist