Cardiac involvement in adult-onset Still's disease: Manifestations, treatments and outcomes in a retrospective study of 28 patients

Quentin Bodard (1) , Vincent Langlois (2) , Philippe Guilpain (3, 4) , Alain Le Quellec (3, 4) , Olivier Vittecoq (5) , David Noel (6) , Vincent Eble (7) , Séverine Josse (8) , Jean Schmidt (9) , Achille Aouba (10) , Hervé Levesque (11, 12) , Maelle Le Besnerais (11, 12) , Ygal Benhamou (11, 12)

1 CH Angoulême - Centre Hospitalier d'Angoulême
2 Groupe Hospitalier du Havre
3 Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB)
4 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
5 Service de Rhumatologie [CHU Rouen]
6 Centre Hospitalier Intercommunal Elbeuf - Louviers - Val de Reuil
7 Centre Hospitalier Eure-Seine - Hôpital d'Evreux - Vernon (Evreux)
8 Centre hospitalier de Dieppe
9 CHU Amiens-Picardie
10 Service de médecine interne [CHU Caen]
11 Service de Médecine Interne [CHU Rouen]
12 Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes

Objective Adult-onset Still's disease (AOSD) is a rare inflammatory disease that may be life-threatening if complicated by cardiac problems. We performed a retrospective multicenter study to describe the manifestations, treatments and outcomes of cardiac involvement in AOSD. Methods We reviewed the medical databases of eight centers. All AOSD patients identified as fulfilling Yamagushi's or Fautrel's criteria were included in the study. Cardiac involvement, clinical manifestations, laboratory features, the course of the disease and treatments were evaluated. Results We included 96 AOSD patients in this study: 28 (29%) had documented cardiac involvement (AOSD + C group) and 68 (71%) had no cardiac involvement (control group). Cardiac complications were observed at diagnosis in 89% of cases. It were pericarditis (n = 17), tamponade (n = 5), myocarditis (n = 5) and non-infectious endocarditis (n = 1). Levels of leukocytes, neutrophils and C-reactive protein were significantly higher (p = 0.02, p = 0.02 and p = 0.002, respectively in the AOSD + C group than in the control group. Admission to intensive care, and the use of biotherapy were more frequent during follow-up in the AOSD + C group than the control group (p = 0.0001 and p = 0.03 respectively). Cardiac involvement was associated with refractory form in multivariate analyzed (p = 0.01). Corticosteroids were effective with or without methotrexate in 71% of patients but not in severe involvement as myocarditis or tamponade. Conclusion Cardiac complications are frequent, inaugural, can be life-threatening and predictive of a refractory course in patients with AOSD. Systematic cardiac screening should be proposed at diagnosis and biotherapy early use should be considered especially in myocarditis.

Domaines: Sciences du Vivant [q-bio]

Quentin Bodard, Vincent Langlois, Philippe Guilpain, Alain Le Quellec, Olivier Vittecoq, et al.. Cardiac involvement in adult-onset Still's disease: Manifestations, treatments and outcomes in a retrospective study of 28 patients. Journal of Autoimmunity, 2021, 116, pp.102541 -. ⟨10.1016/j.jaut.2020.102541⟩. ⟨hal-03493825⟩