• María Lázaro Salvador, Luis Rodríguez-Padial, Clara Soto Abánades, Alejandro Cruz Utrilla, Joan Albert Barberá Mir, Manuel López-Meseguer, Javier Segovia Cubero, SamperGustavo JuanGJDepartment of Pneumology, Hospital General Universitario de Valencia, Valencia, Spain., Isabel Blanco Vich, Pilar Escribano-Subías, REHAP, and investigators.
• Department of Cardiology, Hospital Universitario de Toledo, Toledo, Spain.
• J. Intern. Med. 2022 Jul 1; 292 (1): 116-126.

BackgroundPulmonary arterial hypertension (PAH) is an independent predictor of death in patients with human immunodeficiency virus (HIV) infection. HIV is the leading cause of PAH (HIV-PAH) worldwide.AimsWe described the characteristics, treatment patterns, and prognosis of a cohort of HIV-PAH patients and compared them with those of an equivalent cohort of patients with idiopathic/familial PAH (IPAH/FPAH).MethodsWe retrospectively analysed and compared the demographic, clinical, and treatment data from patients with HIV-PAH and those with IPAH/FPAH in the Spanish PAH registry (REHAP) from 1998 to 2018. The HIV-PAH overall survival (OS) rate up to 5 years was compared to the age- and sex-matched IPAH/FPAH population. Changes in treatment patterns in patients with HIV-PAH after 2010 and their effects on OS were also analysed.ResultsCompared to those with IPAH/FPAH (n = 739), patients with HIV-PAH (n = 132) were younger, mainly men, and had a better functional status. The clinical presentation, haemodynamics, and respiratory function were similar between the groups. Parenteral drug use was the most common mode of HIV transmission. Approximately 11% of patients with HIV-PAH did not receive PAH-targeted therapy. The age- and sex-adjusted 5-year OS rate from diagnosis was 74.0% for patients with HIV-PAH and 68.7% for those with IPAH (p < 0.159). During/after 2010, 23% of patients with IPAH/FPAH received upfront dual oral combination, while oral monotherapy remained the main first-line treatment in patients with HIV-PAH. The overall OS rate remained stable.ConclusionsPatients with HIV-PAH were predominantly young men. The short-term prognosis is similar to that of age- and sex-matched patients with IPAH/FPAH, despite a better functional status. Oral monotherapy remains the preferred first-line treatment in the current cohorts.© 2022 The Association for the Publication of the Journal of Internal Medicine.

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