Pulmonary Complications of Collagen Vascular Disease

Chronic interstitial lung disease complicates collagen vascular disorders in more than 5% of cases and is an important cause of morbidity and mortality in this patient population. An alveolitis characterizes the early phases of the lung lesion; perpetuation of this inflammatory process may result in irreversible pulmonary fibrosis and respiratory failure. In this chapter, we review the role of conventional studies (such as chest radiographs and pulmonary function tests) and newer diagnostic modalities (such as bronchoalveolar lavage, gallium scanning, and thin-section computed tomographic scanning) to identify patients with active alveolitis who are at risk for functional deterioration. While optimal treatment is controversial, the role of corticosteroids, immunosuppressive/cytotoxic, and antifibrotic agents is discussed.