Pulmonary arterial hypertension in adults with Still’s disease: another pulmonary manifestation associated with HLA-DRB1*15

• DOI: 10.1016/j.ard.2025.04.016
• Publication Date: 2025-05-15T11:17:47Z
• Authors: Athénaïs Boucly; Stéphane Mitrovic; Maryvonnick Carma...; Laurent Savale; Xavier Jaïs; Jean-Luc Taupin; Estibaliz Lazaro; Emilie Berthoux; Nicolas Schleinitz; Maria-Rosa Ghigna; Joanna Kedra; Xavier Mariette; Céline Roussin; Pierre-Antoine Juge; Marc Humbert; Olivier Sitbon; David Montani; Bruno Fautrel

Inflammatory lung disease in Still's (SD) has recently been described. Among its manifestations, pulmonary arterial hypertension (PAH) is a rare and life-threatening event, with only few case reports published. The objective was to report the largest adult cohort of PAH occurring context SD. We identified 16 SD patients (PAH+) by call for observations from CRI-IMIDIATE (Club Rhumatismes & Inflammation - Immune-Mediated Disease Alliance Translational Clinical Research) network (https://cri-net.com/recherche/reseau-CRI-Imidiate/) search French registry. Patient characteristics evolution were retrospectively compared those 111 controls without (PAH-) followed reference centre. profile PAH+ PAH- groups differed: 100% versus 69.4% female (P = .006), 75% 17.1% Black < .0001), more active both at diagnosis throughout course, likely present macrophage activation syndrome (62.5% vs 14.4%, P .0001) exhibit eosinophilia during course (68.7% 7.2%, .0001). For 84 out 127 genetic typing, HLA-DRB1*15 allele prevalent than (8/11 [72.7%] 22/73 [30.1%], .014). frequently received canakinumab immunosuppressants did had higher frequency drug reactions interleukin 1 (IL-1) and/or IL-6 inhibitors (37.5% .002). Mortality 0.9%, all deaths related flare. These results reinforce association between severe forms raise question therapeutic optimisation such patients.