Idiopathic and connective tissue disease-associated pulmonary arterial hypertension (PAH): Similarities, differences and the role of autoimmunity

Elvira Favoino; Marcella Prete; Vasiliki Liakouli; Patrizia Leone; Adriana Sisto; Luca Navarini; Marta Vomero; Francesco Ciccia; Piero Ruscitti; Vito Racanelli; Roberto Giacomelli; Federico Perosa

doi:10.1016/j.autrev.2024.103514

Idiopathic and connective tissue disease-associated pulmonary arterial hypertension (PAH): Similarities, differences and the role of autoimmunity

Autoimmun Rev. 2024 Jan 3;23(4):103514. doi: 10.1016/j.autrev.2024.103514. Online ahead of print.

Authors

Affiliations

¹ Laboratory of Cellular and Molecular Immunology, Department of Interdisciplinary Medicine, University of Bari Medical School, Bari, Italy. Electronic address: elvira.favoino@uniba.it.
² Internal Medicine Unit, Department of Interdisciplinary Medicine, University of Bari Medical School, Bari, Italy.
³ Rheumatology Section, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", Naples, Italy.
⁴ Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine, University of Bari Medical School, Bari, Italy.
⁵ Clinical and research section of Rheumatology and Clinical Immunology, Fondazione Policlinico Campus Bio-Medico, Via Álvaro del Portillo 200, 00128, Rome, Italy; Rheumatology and Clinical Immunology, Department of Medicine, University of Rome "Campus Biomedico", School of Medicine, Rome, Italy.
⁶ Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
⁷ Centre for Medical Sciences, University of Trento and Internal Medicine Division, Santa Chiara Hospital, Provincial Health Care Agency (APSS), Trento, Italy.
⁸ Rheumatic and Systemic Autoimmune Diseases Unit, Department of Interdisciplinary Medicine, University of Bari Medical School, Bari, Italy. Electronic address: federico.perosa@uniba.it.

PMID: 38181859
DOI: 10.1016/j.autrev.2024.103514

Abstract

Pre-capillary pulmonary arterial hypertension (PAH) is hemodynamically characterized by a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg, pulmonary capillary wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) > 2. PAH is classified in six clinical subgroups, including idiopathic PAH (IPAH) and PAH associated to connective tissue diseases (CTD-PAH), that will be the main object of this review. The aim is to compare these two PAH subgroups in terms of epidemiology, histological and pathogenic findings in an attempt to define disease-specific features, including autoimmunity, that may explain the heterogeneity of response to therapy between IPAH and CTD-PAH.

Keywords: Autoimmunity; Connective tissue diseases; Epidemiology; Histology; Idiopathic pulmonary arterial hypertension; Pathogenesis; Pulmonary arterial hypertension.

Publication types

Review