SAD are a heterogeneous group of immune-mediated inflammatory disorders in which the body's function to distinguish its own from other people's antigens has failed. The body, consequently reacts to its own antigens and causes multisystem inflammation of the organism. The most common SADs in which pulmonary complications occur are: systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, Sjögren's syndrome, polymyositis /dermatomyositis and mixed connective tissue disease. It is hard to determine the exact prevalence of pulmonary manifestations in SAD because they are often asymptomatic and the proof of their existence is found on autopsy or accidentally during performing regular diagnostic procedures. The most common symptoms are cough, dyspnea on exertion, chest pain and fever, but they can vary and depend on which is the predominant pathophysiological mechanism and which part of the respiratory system is predominantly affected. Although pulmonary complications occur predominantly in developed stages of SAD, sometimes the first manifestation can be from the respiratory tract. Clinical manifestations of SADs include: interstitial lung disease, pleuritis, pleural effusion, pulmonary hypertension, bronchiolitis and pneumonia. At the appearance of the first symptoms or signs of the disease, routine laboratory findings and a chest X-ray are taken. Although most of the changes can already be seen on X-ray, high-resolution computed tomography has been proven superior then the X-ray for further classification of changes of the lungs. Pulmonary function tests should be performed to demonstrate functional lung changes. If that is not enough for establishing the diagnosis, bronchoscopy with bronchoalveolar lavage is indicated as the next step, and the lung biopsy is the final choice. General treatment measures include smoking cessation, administration of flu and pneumococcal vaccinations, maintenance of bronchopulmonary hygiene and pulmonary rehabilitation. In case of reduced saturation, the use of oxygen is indicated. Drug treatment includes anti-inflammatory and immunomodulatory drugs such as nonsteroidal antirheumatic drugs, corticosteroids, drugs that modify the course of the disease and immunosuppressants. Recently, biological therapy has become more represented. It is very important to diagnose the disease in its early onset in order to start adequate therapy in time and prevent irreversible changes of the respiratory system.