Lung involvement in systemic sclerosis

Scleroderma is a multisystem disease characterized by a severe inflammatory process and exuberant fibrosis. Lung involvement is a frequent complication and a leading cause of morbidity and mortality in this syndrome. Two major pulmonary syndromes are associated with scleroderma; a pulmonary vascular disorder evolving over time into relatively isolated pulmonary arterial hypertension (PAH), and interstitial lung disease (ILD). Each syndrome, when present, is a cause of morbidity and significantly reduces survival of scleroderma patients when compared to patients free of lung complication. When pulmonary hypertension and ILD are combined, survival is further reduced. Current therapy appears to have no meaningful effect on either condition and, thus, there is a need for better understanding of underlying pathogenic mechanisms. This review focuses on clinical, diagnostic, and therapeutic features of PAH and ILD as well as other frequent but less debilitating lung complications of scleroderma .

In this issue

Pulmonary Involvement in systemic diseases

Humbert M. (Clamart, France)

Lung involvement in systemic sclerosis

Hassoun P. M. (Baltimore, USA)

Pleural and pulmonary involvement in systemic lupus erythematosus

Torre O, et al. (Milan, Italy)

The lung in rheumatoid arthritis

Amital A., et al. (Petach Tikva, Israel)

Pulmonary manifestations of Sjögren’s syndrome

Hatron P.-Y., et al. (Lille, France)

Pulmonary veno-occlusive disease: the bête noire of pulmonary hypertension in connective tissue diseases?

O’Callaghan D. S., et al. (Clamart, France)