4 Interesting Facts of Systemic Sclerosis
1. It is important to distinguish localized scleroderma (e.g., morphea) from the systemic form (systemic sclerosis [SSc]) because localized disease does not have internal or systemic manifestations.
2. Distinguishing the limited versus diffuse form of SSc and identifying disease-specific autoantibodies is important because these factors often impact the pace of disease and pattern of internal organ involvement.
3. Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the chief causes of mortality; therefore, vigilant screening assessments for their presence are indicated in all patients with SSc as early detection and intervention strategies may favorably impact the natural history of the disease.
4. Scleroderma renal crisis (SRC), often heralded by an acute rise in blood pressure, is associated with early diffuse cutaneous SSc (dcSSc) and RNA polymerase III antibody. It requires rapid recognition and initiation of angiotensin-converting enzyme (ACE)-I therapy.
Systemic Sclerosis commonly referred to as scleroderma, is a rare and potentially devastating systemic autoimmune disease characterized by vasculopathy and organ fibrosis.
Almost all patients with scleroderma have skin thickening (“sclero” = thick, “derma” = skin), Raynaud’s phenomenon, and esophageal reflux or dysmotility. ILD and pulmonary arterial hypertension are the leading causes of scleroderma-related death.