Cardiac involvement in adult-onset Still's disease: Manifestations, treatments and outcomes in a retrospective study of 28 patients. Issue 116 (January 2021)

• Record Type: Journal Article
• Authors: Bodard, Quentin; Langlois, Vincent; Guilpain, Philippe; Le Quellec, Alain; Vittecoq, Olivier; Noel, David; Eble, Vincent; Josse, Séverine; Schmidt, Jean; Aouba, Achille; Levesque, Hervé; Le Besnerais, Maelle; Benhamou, Ygal
• Is Part Of: Journal of autoimmunity. Issue 116(2020)
• Publication Date: 2021-01
• Subjects: Adult-onset Still's disease -- Cardiac involvement -- Pericarditis -- Tamponade -- Myocarditis -- Biotherapy; AOSD Adult-onset Still's disease -- RHL Reactive hemophagocytic lymphohistiocytosis -- DMARDs Disease-modifying antirheumatic drugs -- MTX Methotrexate -- bDMARDs Biological disease-modifying antirheumatic drugs -- AOSD + C Adult-onset Still's disease with cardiac involvement -- TTU Transthoracic ultrasonography -- ECG Electrocardiogram -- PAH pulmonary arterial hypertension -- PMNs Polymorphonuclear cells -- CRP C-reactive protein -- SF Serum ferritin -- MRI Magnetic resonance imaging -- NSAIDs Non-steroidal anti-inflammatory drugs -- WBC White blood cell -- TNFα Tumor necrosis factor-alpha -- sICAM-1 soluble intercellular adhesion molecule-1 -- ANA Antinuclear antibodies -- RF Rheumatoid factor; Autoimmunity -- Periodicals; Autoimmune diseases -- Periodicals; Autoantibodies -- Periodicals; Autoimmune Diseases -- Periodicals; Auto-immunité -- Périodiques; Maladies auto-immunes -- Périodiques; Electronic journals; 616.978005
• DOI: 10.1016/j.jaut.2020.102541
• Languages: English
• ISSNs: 0896-8411

Abstract

Abstract: 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. Highlights: About 30% of Adult onset Still's disease developed cardiac involvement. AAdmission to intensive care and use of biotherapy were more frequent during follow-up in AOSD + C group than control group. Cardiac involvement was associated with refractory form in multivariate analyzed.