Article

[Adult-onset Still's disease revealed by a pericardial tamponade: report of two cases].

Service de Médecine Interne, Hôpital La-Rabta, 1007 Tunis, Tunisie.
La Revue de Médecine Interne (Impact Factor: 1.32). 08/2006; 27(7):546-9.
Source: PubMed

ABSTRACT Adult onset Still's disease is a systemic inflammatory disorder of unknown etiology characterized by the association of a high spiking fever, an evanescent skin rash, arthritis, and hyperleukocytosis. Pericarditis is amongst the most common systemic manifestations of adult onset Still's disease.
We report on two patients with a pericardial tamponade revealing an adult onset Still's disease in a 52-year-old female and a 31-year-old male. Pericardial fluid was bloody in the two cases, and histopathology only disclosed non specific inflammatory changes. Both patients received corticosteroids and outcome was uneventful with a follow-up of 8 years and 12 months, respectively.
Pericardial tamponade is an uncommon clinical feature of adult-onset Still's disease and usually occurs at disease onset. It makes the diagnosis of adult-onset Still's disease difficult as the other disease manifestations are commonly neglected. Adult onset Still's disease should be added to the differential of acute pericarditis and tamponade.

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Available from: Imed Ben Ghorbel, Jul 25, 2015
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    ABSTRACT: Introduction Adult Still's disease may present with multiple non-specific clinical manifestations leading to diagnostic difficulties. Presentation as initial myopericarditis is rare but must be known. Case report We report a 49-year-old man who presented with the sudden onset of a high fever, arthralgia and myalgia, skin rash, dyspnea and chest pain related to a myopericarditis. Infectious, neoplastic and autoimmune diagnostic work-up was non-contributive. Adult Still's disease was discussed and confirmed by the decrease in the glycosylated fraction of ferritin. Corticosteroids followed by interleukin-1 receptor antagonist (anakinra) therapy allowed a rapid improvement in clinical and biological manifestations. Conclusion Adult Still's disease should be considered in any systemic inflammatory disease without a diagnosis, even with atypical manifestations, especially since it can be life-threatening in the absence of treatment.
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