Pulmonary Hypertension in Autoimmune Diseases – A Review of New Concepts in Screening, Diagnosis and Therapeutic Management
Authors:   Department of Internal Medicine, Dr. Carol Davila University Central Military Emergency Hospital, Bucharest, Romania, Anghel Daniela ,   daniela.anghel@prof.utm.ro (DA),   iulianadine.nicolau@rez.umfcd.ro (INN),   dr.lianamihalca@gmail.com (LIM) Department of Medico-Surgical an, Nicolau Iulia N. ,   Department of Internal Medicine, Dr. Carol Davila University Central Military Emergency Hospital, Bucharest, Romania; daniela.anghel@prof.utm.ro (DA); iulianadine.nicolau@rez.umfcd.ro (INN); dr.lianamihalca@gmail.com (LIM), Mihalca Liana I. ,   Department of Internal Medicine, Dr. Carol Davila University Central Military Emergency Hospital, Bucharest, Romania; daniela.anghel@prof.utm.ro (DA); iulianadine.nicolau@rez.umfcd.ro (INN); dr.lianamihalca@gmail.com (LIM), Petrache Oana G. ,   Department of Internal Medicine, Dr. Carol Davila University Central Military Emergency Hospital, Bucharest, Romania; daniela.anghel@prof.utm.ro (DA); iulianadine.nicolau@rez.umfcd.ro (INN); dr.lianamihalca@gmail.com (LIM) Carol Davila University of Medic, Costache Raluca S. ,   Carol Davila University of Medicine and Pharmacy, Bucharest, Romania; oana-georgiana.petrache@drd.umfcd.ro Department of Gastroenterology, Dr. Carol Davila Central Military Emergency University Hospital, Bucharest, Romania; florentina.ionita-radu@umfcd.ro, Opriș-Belinski Daniela ,   Carol Davila University of Medicine and Pharmacy, Bucharest, Romania; oana-georgiana.petrache@drd.umfcd.ro Department of Rheumatology, ‘Sf. Maria’ Clinical Hospital, Bucharest, Romania; daniela.opris@umfcd.ro, Bojincă Violeta C. ,   Carol Davila University of Medicine and Pharmacy, Bucharest, Romania; oana-georgiana.petrache@drd.umfcd.ro Department of Internal Medicine, ‘Sf. Maria’ Clinical Hospital, Bucharest, Romania; violeta.bojinca@umfcd.ro, Ioniță Radu Florentina ,   Carol Davila University of Medicine and Pharmacy, Bucharest, Romania; oana-georgiana.petrache@drd.umfcd.ro Department of Gastroenterology, Dr. Carol Davila Central Military Emergency University Hospital, Bucharest, Romania; florentina.ionita-radu@umfcd.ro
Published: August 2023

Pulmonary hypertension (PH) has gained more and more attention in the last years given the increased mortality and morbidity rate and in particular pulmonary arterial hypertension associated with autoimmune diseases (a specific subgroup of PH), entities with an increasing incidence and prevalence globally. Among these, systemic sclerosis is of particular interest, being the most common among connective tissue diseases (CTDs) with PH as a major complication. Other CTDs such as mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), primary Sjogren's syndrome, and dermatomyositis may also develop PH as a complication of these severe and complex conditions, which can have a major impact on prognosis and quality of life. PH has also been reported in rheumatoid arthritis and ankylosing spondylitis but further studies are necessary. Several studies have shown that the 3-year survival rate for patients with systemic sclerosis-associated with pulmonary hypertension (SSc-PH) is between 31% and 52%, while the 5-year survival rate is below <50%. Therefore, an early and accurate diagnosis as well as subsequent management of PH in these conditions is mandatory, since survival remains suboptimal despite treatment advances. The aim of this article is to provide an overview of different autoimmune diseases associated with PH and to provide an easy reference source on current best practices.