In this Grand Round we present a 32-yr-old African man who became severely ill after a 5-month history of weight loss, pyrexia, arthralgia, sweats and rash. He went on to develop pericarditis, pericardial effusion with tamponade, hepatomegaly with abnormal liver function tests, lymphadenopathy, massive proteinuria and required ventilatory, circulatory and renal support. The differential diagnosis was adult onset Still's disease, systemic lupus erythematosus (SLE), infection and lymphoma. Primary infection and lymphoma were excluded and he was treated, with dramatic success, with intravenous immunoglobulins (i.v.IG). Subsequent renal biopsy excluded SLE but confirmed collapsing glomerulopathy. The proteinuria improved dramatically following treatment with mycophenolate mofetil. We discuss some of the difficult diagnostic and management issues raised by this patient and the different uses and mechanisms of action of i.v.IG.
[Show abstract][Hide abstract] 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.
La Revue de Médecine Interne 08/2006; 27(7):546-9. · 1.07 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: IntroductionAdult 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.
La Revue de Médecine Interne 07/2006; 27(7):546-549. DOI:10.1016/j.revmed.2006.03.027 · 1.07 Impact Factor
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