OP0094\u2005PULMONARY ARTERIAL HYPERTENSION IN ADULT-ONSET STILL’S DISEASE: A CASE SERIES OF 13 PATIENTS

Abstract

Pulmonary Arterial Hypertension (PAH) is a rare but potentially fatal complication of Adult-Onset Still’s Disease (AOSD) (1). To date, only isolated observations have been published.To establish the largest case series of AOSD patients with PAH, and to describe their clinical profile, evolution and response to treatments.Cases were retrospectively identified from the French PAH network database and from an online call of the “Club Rhumatismes et Inflammation” (http://www.cri-net.com). To be included, all patients had to fulfil the Yamaguchi or Fautrel’s criteria for AOSD and PAH had to be confirmed by right heart catheterization. The data were collected using a standardized questionnaire.Thirteen patients were identified. All were female, the mean age at PAH diagnosis was 32± 12 years, 2 (15%) patients were Caucasian, 6 (46%) from Sub-Saharan Africa, 1 (8%) from Asia and 4 (31%) from West Indies. Only 2 (15%) patients were smokers. All patients had a systemic onset of AOSD, 12 had a polycyclic and 1 a chronic articular evolution, and the mean delay between AOSD and PAH diagnosis was 2.9 (range 1.7 -5.4) years. At PAH diagnosis, patients were receiving the following treatments: 13 (100%) corticosteroids (median dose 12 mg [interquartile range (IQR) 9-18]), 3 (23%) methotrexate, 8 (61%) interleukin (IL)-1 inhibitors (exposure median duration 6.7 months [IQR 3.6-8.5]), none IL-6 inhibitors, 2 (15%) TNF inhibitors. Six (46%) patients developed PAH during an AOSD flare. PAH was severe at diagnosis: 2 (15%), 7 (54%) and 4 (31%) patients were in NYHA functional class II, III and IV, respectively, with a median 6-minute walk distance of 289 m [IQR 0-448], a mean pulmonary arterial pressure of 41 ± 12 mmHg, a mean pulmonary arterial occlusion pressure of 6 ± 3 mmHg, a mean cardiac output of 3.9 ± 1.2 L/min, a mean cardiac index of 2.5 ± 0.9 L/min/m2 and a median pulmonary vascular resistance of 7 Wood Units [IQR 6-11]. The treatment prescribed after PAH diagnosis is detailed in the table. The median follow-up was 34 months [IQR 7-42]. Five patients (38.5 %) died. Figure 1 shows the overall survival. The haemodynamic response to PAH treatment seemed to be dissociated from the prognosis since several patients have died while their haemodynamic had improved or almost normalised.PAH is a rare but potentially severe complication of AOSD, leading to death in 38.5% of our cases series. AOSD remission should be physicians’ objective, since PAH seems to occur when the underlying disease is not controlled.[1]Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still’s disease. Nat Rev Rheumatol. 2018;1:603-618.Table 1.Therapeutic managementTreatmentn (%)Inotropic therapy5 (38%)HTAP treatment10 (77%)•0Monotherapy3•oInitial oral dual combination therapy3•nDual combination therapy including intravenous (IV) prostacyclin1•uUpfront triple combination therapy including IV prostacyclin3High-dose corticosteroids9 (69%)Interleukin 1 inhibitors initiation2 (15%)Interleukin 6 inhibitors initiation5 (38%)The authors want to thank the Club Rhumatismes et Inflammation for the diffusion of the online call.None declared