ABSTRACT
Diagnosis of systemic sclerosis (SSc) is mostly clinical. The European league against rheumatism/American college of rheumatology classification criteria is the most recent method for diagnosis of SSc. Skin biopsy and histopathological examination is not an absolute requirement in the diagnostic algorithm of SSc. However, histopathological features of SSc are characteristic and helpful in differentiating from other scleroderma-like conditions. Patients with SSc require repeated pulmonary screening for interstitial lung disease and pulmonary arterial hypertension. Any patient with SSc must be thoroughly screened for gastro-intestinal involvement, even in absence of overt symptoms. This is because such symptoms may be subjective and may not be true indicator of the underlying involvement. Assessment of the disease severity helps in making therapeutic decision and intervention; as early inflammatory stage of SSc is more amenable to therapeutic measures than the late fibrotic phase.
