Pulmonary manifestations of systemic sclerosis (SSc, scleroderma) are manifold (Table 1) and often have a profound influence on the course of the disease. A discussion of all the pulmonary complications of SSc is beyond the scope of this article but is presented in a recent review.<sup>10 Bolster M., Silver R.M. Systemic sclerosis Lung Disease in Rheumatic Disorders. London: Baillie`re-Tindall (1993).  79-88

Cliccare qui per andare alla sezione Riferimenti</sup> The two major pulmonary conditions occurring among SSc patients, pulmonary hypertension and interstitial fibrosis (Table 2), will be discussed in detail. Isolated pulmonary hypertension occurs primarily in patients with limited cutaneous SSc (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia [CREST] variant) in the absence of significant pulmonary fibrosis, whereas interstitial fibrosis occurs in either limited or diffuse cutaneous SSc.