Collagen based vascular diseases with pulmonary involvement comprise rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, dermatomyositis and polymyositis, ankylosing spondylitis, Sjögren's syndrome, and mixed connective tissue diseases. The different characteristics of pulmonary involvement are described. In such circumstances, early recognition of lung involvement is of considerable significance and the relationship to the corresponding disease has to be made. Frequently unrecognised pulmonary involvement ends up as fibrosis with irreversible deficits in respiratory function.