A variety of interstitial lung diseases (ILDs) have been reported in association with connective tissue diseases (CTDs). ILD is commonly associated with multiple CTDs and accounts for significant morbidity and mortality in these conditions. In rheumatoid arthritis and systemic sclerosis, ILD commonly occurs in the course of these disorders (incidence: 20–44%). The pathological findings of ILDs are similar to those of idiopathic interstitial pneumonia. A wide variety of histopathologic features, such as various types of interstitial pneumonia and airway involvement, have been observed that are specific for ILDs in rheumatoid arthritis, and this high variety makes its pathology complicated. The diagnosis of ILD is generally based on clinical presentation, bronchioalveolar lavage fluid and high-resolution computed tomography, among others. The most important differential diagnosis is infection, especially pneumocystis pneumonia, and treatment-related toxic damage. The immunosuppressive agents most widely used for the treatment of ILDs are cyclophosphamide, azathioprine, mycophenolate mofetil and calcineurin inhibitors. Other therapeutic strategies are currently being extensively studied, such as antifibrotic agents, endothelin-1 receptor antagonists, tyrosine kinase inhibitors and newer biological agents. In this article, we describe novel therapies for ILDs associated with CTDs.