Connective Tissue Disease

It has been recognized for over a century that patients with “rheumatism” may have respiratory abnormalities as well. However, only in the last 20 years or so, as tests of pulmonary function have become increasingly sophisticated and more widely available, has it become clear that pleuropulmonary manifestations in the various connective tissue disorders are in fact quite common.1,2 Involvement of the lungs has been described in nearly every variety of connective tissue disease (CTD), from the hereditary syndromes of Marfan and Ehlers-Danlos3,4,5 through the immunologically mediated collagen vascular diseases (CVD) to the related systematic vasculitis, granulomatosis, and lymphoproliferative disorders.