Pneumon 2018, 31(4): 223-230, Review
Systemic Sclerosis - Associated Pulmonary Hypertension
Authors Information

2nd Department of Critical Care Medicine and Pulmonary Hypertension Clinic, ATTIKON University Hospital, National and Kapodistrian University of Athens, Chaidari, Greece


Lung disease is a major complication of systemic sclerosis associated with high morbidity and mortality rates. Pulmonary hypertension and lung fibrosis are the prevalent manifestations of lung disease, usually with overlapping characteristics. A major challenge in the management of the disease, is to identify the phenotypes of patients who might benefit from specific therapeutic modalities. The aim of the present review is to describe the clinical and epidemiological characteristics, classification, risk assessment and management of systemic sclerosis associated pulmonary hypertension.