Connective Tissue Diseases and the Heart

CONNECTIVE tissue disease is a term used to describe any one of a group of rheumatoid disorders of uncertain cause, which have overlapping clinical manifestations and may share common mechanisms of pathogenesis. The diseases included in the discussion are rheumatoid arthritis, systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS) (scleroderma), classic polyarteritis nodosa (PAN), polymyositis (dermatomyositis), ankylosing spondylitis, and Reiter syndrome. Acute rheumatic fever and chronic rheumatic heart disease are not discussed. This selection is necessarily arbitrary and is defended only by general familiarity with these diagnostic categories and their recognized potential for producing cardiac and pericardial lesions (Table). Diagnostic criteria and extracardiac manifestations of these diseases are summarized comprehensively elsewhere. 1 The most apparent external clinical manifestations of these disorders typically involve joints and skin. However, cardiac disease occurs as well and plays an important role in determining whether a patient with one of these diseases has a chronic