2020 Chinese expert-based consensus statement regarding the diagnosis and treatment of connective tissue disease associated pulmonary arterial hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue disease(CTD),being one of the leading causes of morbidity and mortality for patients with this condition. To establish the expert-based consensus on diagnosis and treatment of CTD-associated PAH, a multidisciplinary consensus development panel was established. The panel of consensus was composed of 45 experts in rheumatology, cardiology, pulmonology, radiology, most of whom were members of Group of Pulmonary Vascular and Interstitial Diseases Associated with Rheumatic Diseases. The consensus development panel developed 9 recommendations for the diagnosis and treatment of CTD-associated PAH. The consensus covers the early screening, diagnosis, disease evaluation and risk assessment, immunosuppressive and PAH-specific therapy with a treat-to-target approach. This consensus is intended to facilitate the decision-making and standardize the care of CTD-associated PAH in China.