Scleroderma: Clinical Features

" SCLERODERMA "

•   Sclerodactyly
•   CREST syndrome, Capillaroscopy as diagnostic tool
•   Lung involvement: pulmonary fibrosis, pulmonary HT^N, aspiration pneumonia, recurrent chest infection, bronchoalveolar ca, pneumothorax
•   Liver disease and primary biliary cirrhosis
•   Exposure to environmental toxins like organic solvents, hydrocarbons and silica as aetiology
•   Renal crisis, HT^N, Renal failure
•   Oral vasodilators as treatment of choice for Raynaud's disease
•   Digital ischaemia leading to autoamputation, Dry eyes, D-pencillamine as Rx
•   Doppler Echo as diagnostic tool for pul.HT^N
•   Endoscopy to assess  GI involvement
•   Resorption of phalanges due to bone ischaemia
•   Microstomia, Malabsorpion syndromes, Myocarditis, Myocardial fibrosis
•   Anti scl 70, Anti-centromere antibodies; ACE inhibitors as useful therapy

Did you know?

ACE inhibitors may dramatically improve scleroderma renal crisis

All patients should be followed carefully for the change in P2 as an evidence of decompensation with pulmonary hypertension

CREST/ CRST syndrome: Calcinosis, Raynaud's (WBC: White-Blue-Crimson color changes), Esophageal dysmotility, Sclerodactyly & Telangiectasia

GERD may predispose to Barrett's esophagus which is precancerous