[Pulmonary involvement in systemic scleroderma]

Pulmonary involvement in progressive systemic sclerosis can affect either the parenchyma or the vasculature. Both forms are fatal within a few months or years, due to respiratory and/or cardiac failure. Patients must be screened for pulmonary hypertension by ultrasonography, to be confirmed by right heart catheterization if necessary, as early diagnosis and immediate treatment improves the prognosis. The interstitial pulmonary disease progresses slowly, leading to respiratory failure, secondary pulmonary hypertension, and heart failure. Immunosuppressive treatments are disappointing but if prescribed early may prevent or delay the otherwise ineluctable aggravation. Pulmonary involvement threatens the vital and functional prognosis of patients with scleroderma, who may also develop several other complica- tions (especially infections and adverse effects of drugs) that are not dealt with here. This paper focuses on the most significant disease manifestations and current therapeutic options.