Association between Rheumatoid Arthritis and Pulmonary Hypertension: Data from the French Pulmonary Hypertension Registry

Abstract

Background: Precapillary pulmonary hypertension (PH), and particularly pulmonary arterial hypertension (PAH), is a life-threatening complication of connective tissue diseases (systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease). The relationship between PH and rheumatoid arthritis (RA) has not been clearly established. Objectives: The aim of the study was to evaluate the relationship between precapillary PH and RA. Methods: We identified patients with PH and suspected RA included in the French PH Registry between 1 May 2004 and 31 December 2012 and evaluated the prevalence of confirmed RA in this population. RA phenotypes, clinical, functional, and hemodynamic data, and patient outcomes were recorded. Results: RA was confirmed in 20 patients (70% female; mean age 52 years) with precapillary PH, including 10 patients with PAH, 6 with severe PH due to lung disease, and 4 with chronic thromboembolic PH. The prevalence of RA was 0.35% (95% CI: 0.23–0.54) in the French PH Registry and 0.58% (95% CI: 0.30–1.11) in idiopathic PAH, comparable to that in the general population. The RA phenotype was characterized by the presence of specific RA autoantibodies and joint erosions in 75% of the patients. The outcomes of PH in the RA patients were unremarkable compared to those in other patients from the registry, and RA therapies had no major impact on the cardiopulmonary parameters. Conclusion: When precapillary PH occurs in RA patients, all PH subsets may be identified. The RA prevalence in the French PH Registry is similar to that in the general population, which does not support a specific association or an indication for PH screening in RA patients.

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

“…Pulmonary arterial hypertension is a well-known pulmonary vascular complication of SSc [173,[502][503][504], systemic lupus erythematosus (SLE) [505][506][507], mixed CTD [506], and, rarely, dermatomyositis [508] and Sjögren's syndrome [509]. Conversely, the relationship between rheumatoid arthritis and PAH is not established [510]. After IPAH, PAH-CTD is the second most prevalent type of PAH in western countries [511].…”

Pulmonary Complications of Rheumatoid Arthritis

“…A retrospective study of the French Pulmonary Hypertension Registry found the prevalence of RA to be 0.35% in the cohort overall, and 0.58% in the group classified as idiopathic pulmonary hypertension. 137 This prevalence is similar to the prevalence of RA in the general population, which does not support a specific association between the two conditions. 137 The REVEAL registry, involving 54 centers in the United States, found an overall prevalence of RA of 3.5% among 2,438 consecutive patients identified as having group 1 pulmonary arterial hypertension.…”

“…137 This prevalence is similar to the prevalence of RA in the general population, which does not support a specific association between the two conditions. 137 The REVEAL registry, involving 54 centers in the United States, found an overall prevalence of RA of 3.5% among 2,438 consecutive patients identified as having group 1 pulmonary arterial hypertension. 138 A study of 321 Korean patients with connective tissue disease meeting a modified definition of WHO group I pulmonary arterial hypertension reported that 7.8% of patients had RA.…”

Diversity of hemodynamic types in connective tissue disease associated pulmonary hypertension: more than a subgroup of pulmonary arterial hypertension

“…SLE, SS and SSc took up most of the CTD-PH patients in our cohort, prevalence of which are 49.8%, 16.4% and 13.7%. PH develops much more uncommonly with RA, vasculitis, PM or DM [ 25 , 26 ], and is rarely seen in patients with AOSD, only a few cases have been reported [ 27 ]. However, because of lacking large cohorts, prevalence of PH in patients with CTDs other than SSc remains to be further determined.…”