Connective tissue diseases and vasculitis are a wide group of autoimmune pathologies that can affect multiple organs. One of the most frequently affected systems is the respiratory system, with compromise of the airway, lung parenchyma, pleura and mediastinum. These pathologies constitute an important cause of morbimortality, making it essencial to maintain a high clinical suspicion. Furthermore, it is of vital relevance to rule out other causes of affectation like adverse drug reactions and infections due to the immunosuppressed state of patients with rheumatic diseases. Both the classification and its management must be done by a multidisciplinary team, based on the clinical history, serology, lung function tests, high-resolution computed tomography, bronchoalveolar lavage and/or biopsy, depending on each case. This review article aims to highlight the importance of a multidisciplinary approach in the management of connective tissue diseases and vasculitis; complex pathologies that can affect the respiratory system, constituting an important cause of morbidity and mortality. The classification and management of these conditions should be carried out by a multidisciplinary team, based on clinical status, laboratory tests, pulmonary function tests, imaging studies, and biopsies, according to each case.