ABSTRACT

Connective tissue diseases are a group of idiopathic systemic inflammatory disease. Lung is one of the most frequently involved organs; and one of the most serious involvement is interstitial lung disease. Prognosis is closely related to histopathological patterns. In connective tissue diseases except rheumatoid arthritis, the most common histopathological pattern is nonspecific interstitial pneumonitis; and it has better prognosis than other paterns. In rheumatoid arthritis, usual interstitial pneumonia with poor prognosis is common. Early diagnosis and treatment is important because interstitial lung disease is a major cause of mortality and morbidity. Pulmonary function tests with diffusing capacity of carbon monoxide and imaging are particularly important for diagnosis. There are very few randomized controlled trials about treatment. It is recommended that to be follow-up in asymptomatic and mild patients and to be treated with immunosuppressive agents in patients with severe disease activity or progressive diseases. In this review, we describe the current diagnostic and treatment approaches.