Pulmonary involvement by a connective tissue disease can result in cli
nically important complications. Pathogenic mechanisms vary from granu
lomatous reaction and interstitial inflammation to primary vasculitis
and immune complex-mediated disease. Understanding the pulmonary compl
ications of connective tissue diseases is challenging in that several
distinct patterns of involvement are associated with the same disease
but the same lung abnormalities are found with several different disea
ses. Early recognition and treatment of pulmonary involvement may offe
r the patient a better chance of recovery from serious conditions that
often carry a grim prognosis if undetected.