Adult-onset Still's disease revealed by perimyocarditis and a concomitant reactivation of an EBV infection

Abstract

SummaryWe describe a 17-year-old patient presenting perimyocarditis as the initial manifestation of the adult-onset Still's disease. Corticotherapy was rapidly successful but induced major acute hepatitis in relation with Epstein-Barr virus reactivation. After 1 year, even if the global outcome is favourable, a slightly lowered ejection fraction still persists. Former case reports and differential diagnosis with reactive haemophagocytic syndrome would be discussed. BACKGROUND

Constrictive pericarditis following atrial fibrillation ablation – A rare complication

“…None of the patients underwent pericardiectomy and almost all had resolution of symptoms 11 . Conversely, prolonged treatment with a corticosteroid can cause several adverse effects, 12 particularly owing to their immunosuppressive effect, with renal toxicity, possible reactivation of viral infections, 13 and exacerbation of bacterial infections, as was the case of our patient, which unfortunately culminated in a fatal outcome.…”

Genetic Basis of Myocarditis: Myth or Reality?

“…The clinical phenotypes associated with HA20 are heterogeneous, including autoimmune diseases (Behçet's disease, hereditary fever-like condition, juvenile idiopathic arthritis, systemic lupus erythematosus, and rheumatoid arthritis) and, recently, AOSD: a splice site mutation on the TNFAIP3 gene has been identified in a patient previously diagnosed with adult-onset Stills' disease (AOSD) [264]; once correctly diagnosed, the patient was successfully treated with anti-IL6 receptor biologic tocilizumab [265]. Myocarditis has been repeatedly reported in AOSD, presenting as myocarditispericarditis [265][266][267][268][269][270][271][272][273][274], with pericarditis occurring in more than 50% of cases [265]. Coronary arteritis has also been described [272].…”