Pulmonary hypertension (PH) is an important cause of morbidity and mortality in connective tissue diseases (CTD). CTD may cause PH due to several mechanisms; pulmonary arterial hypertension (PAH), associated interstitial lung disease, neuromuscular disease, and/or sleep disordered breathing leading to hypoxia, associated thromboembolic PH, and pulmonary venous hypertension due to left ventricular dysfunction. Recent studies have clarified that pulmonary hypertension associated with connective tissue diseases (CTD-PH) has some distinctive clinical aspects from other PH, such as high prevalence, venous and cardiac involvement, less favourable outcomes, helpfulness of detection algorithm DETECT, response to immunosuppression, existence of combine form. Pressure in pulmonary artery can be measured on echocardiography, but the gold standard for diagnosis is right heart catheterization. PАH-specific therapy in addition to immunosuppression is the most common treatment used for CTD-PH patients, there are currently
many controversial thoughts about the beginning and duration of these drug prescribing. In this narrative review, we discuss the epidemiologic burden, pathogenesis, clinical presentation, with physicians’ emphasis on diagnostic criteria and algorithms of early disease detection and management of CTD- PH disease.