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Interstitial diseases represent one of the most frequent type of lung involvement associated with connective tissue diseases and contribute to increased morbidity and mortality. The main connective tissue diseases associated with interstitial lung disease are rheumatoid arthritis (RA), systemic sclerosis (SSc) and idiopathic inflammatory myositis (IIM), including dermatomyositis (DM), polymyositis (PM) and antisynthetase syndrome (ASS). However, the autoimmune diseases that cause interstitial lung disease also include Sjögren’s syndrome (SS), systemic lupus erythematosus (SLE) and mixed connective disease (MCTD). High-resolution computed tomography (HRCT) is the cornerstone examination for the identification of interstitial lung disease and for the definition of specific radiological patterns. In this regard, interstitial lung diseases associated with connective tissue disease can present as: usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), lymphocytic interstitial pneumonia (LIP), desquamative interstitial pneumonia (DIP), diffuse alveolar damage (DAD), bronchiolitis. In this review we describe the interstitial lung involvement of the main connective tissue diseases, focusing on their prevalence, the clinical and radiological presentation and their prognosis. [ABSTRACT FROM AUTHOR]