Evaluation - Systemic Sclerosis (Scleroderma, SSC)

History and physical examination with particular attention to blood pressure (heralding feature of renal disease).
Laboratories: ESR, ANA (anticentromere pattern associated with limited SSc), specific antibodies may include antitopoisomerase I (Scl-70), (UA). An increased range of autoantibodies correlating with specific clinical features have become recognized (Table 382-3, HPIM-19, p. 2157).
Radiographs: CXR, barium swallow if indicated, hand x-rays may show distal tuft resorption and calcinosis.
Additional studies: ECG, echo, PFT, consider skin biopsy.

Treatment: Systemic Sclerosis

Education regarding warm clothing, smoking cessation, antireflux measures.
Calcium channel blockers (e.g., nifedipine) useful for Raynaud's phenomenon. Other agents with potential benefit include sildenafil, losartan, nitroglycerin paste, fluoxetine, bosentan, digital sympathectomy.
ACE inhibitors: particularly important for controlling hypertension and limiting progression of renal disease.
Antacids, H₂ antagonists, omeprazole, and metoclopramide may be useful for esophageal reflux.
D-Penicillamine: controversial benefit to reduce skin thickening and prevent organ involvement; no advantages to using doses >125 mg every other day.
Glucocorticoids: no efficacy in slowing progression of SSc; indicated for inflammatory myositis or pericarditis; high doses may be associated with development of renal crisis.
Cyclophosphamide: improves lung function and survival in pts with alveolitis.
Epoprostenol or treprostinil (prostacyclin), bosentan (endothelin-1 receptor antagonist), sildenafil (phosphodiesterase type 5 inhibitor): may improve cardiopulmonary hemodynamics in pts with pulmonary hypertension.