Autoimmune disorders can manifest in the lung with a protean variability of presentations. The disease may affect the airways, interstitium and/or vascular compartments that may be involved by inflammation and/or fibrosis. Pulmonary involvement in these patients imparts significant morbidity, and in some entities, constitutes the leading cause of death. Interstitial lung disease could also be the presenting manifestation of an undiagnosed connective tissue disorder. We will review the clinical and pathologic characteristics of lung involvement associated with autoimmune disorders such as but not limited to scleroderma, systemic lupus erythematosus, polymyositis/dermatomyositis, rheumatoid arthritis and interstitial pneumonia with autoimmune features.