Clinical meaning
Systemic sclerosis involves three pathologic processes: vasculopathy (endothelial damage causing Raynaud's and digital ischemia), immune activation (autoantibodies and T-cell infiltration), and fibrosis (excessive collagen deposition in skin, lungs, heart, and GI tract). The fibrotic process replaces normal tissue architecture with dense collagen, impairing organ function.
Diagnosis & workup
Diagnostics & workup: - Order ANA and scleroderma-specific antibodies (anti-Scl-70, anti-centromere) - Order pulmonary function tests (DLCO for early ILD detection) - Order echocardiogram for pulmonary hypertension screening - Order high-resolution CT chest for interstitial lung disease - Order right heart catheterization if PH suspected - Order nailfold capillaroscopy - Order esophageal motility studies if dysphagia
Risk factors: - Female sex (4:1) - Age 30-50 years - African American descent (more severe disease) - Silica or organic solvent exposure - Anti-Scl-70 antibody (diffuse disease) - Anti-centromere antibody (limited/CREST)