Respiratory system

Abstract

Pulmonary involvement is common in the connective tissue diseases (CTDs) and is associated with significant morbidity and mortality. Improved management of systemic disease has led to increasing numbers of surviving patients with clinically significant pulmonary disease. Screening for pulmonary complications highlights the frequency of subclinical involvement. In this chapter, the pulmonary manifestations of the more common CTDs are detailed, including rheumatoid arthritis (RA), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), polymyositis/dermatomyositis (PM/DM), Sjögren's syndrome (SS), and, more briefly, ankylosing spondylitis (AS). A broad spectrum of pulmonary disorders are seen in association with the CTDs or the drugs used to treat the underlying disorder, including interstitial lung disease, pulmonary infections, airways disease, pulmonary nodules, pleural disease, chest wall pathology and pulmonary vascular disease; the discussion is stratified by pulmonary complication. In many cases, two or more pulmonary manifestations of CTD coexist or there are other concurrent diseases such as asthma and lung cancer, resulting in potentially confusing mixed imaging and pulmonary function abnormalities. This chapter presents a comprehensive approach to the investigation, screening, prognostic evaluation, and treatment decisions in pulmonary disease associated with the CTDs.