Pulmonary manifestations of SSc are leading causes of disease-related morbidity and mortality. Key clinical issues relate to the early detection of fibrotic lung disease, the interpretation of its significance, and decisions on when to start therapy. Respiratory symptoms are common in patients with SSc, but physical examination often fails to establish if the underlying cause is interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), impaired locomotion due to systemic disease or loss of fitness. Impaired lung function is usually evident on pulmonary function testing, with the pattern of functional impairment often discriminating usefully between ILD and PAH. The presence of PAH can be indicated by echocardiography and must be confirmed by right heart catheterization. Whereas chest radiographs detect established ILD, high-resolution CT can identify earlier or very mild inflammatory changes. Treatment options for ILD are limited to immunosuppressive agents, notably cyclophosphamide, often given in combination with low-dose prednisolone. Recent studies have shown that cyclophosphamide is effective in stabilizing pulmonary function--especially in patients with severe fibrotic disease--and in improving health-related quality of life. Progression of pulmonary manifestations and responses to treatment are best monitored using pulmonary function testing. For patients with severe end-stage pulmonary fibrosis, lung transplantation may offer a viable alternative therapeutic option.
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