Macrophage activation syndrome in a patient with juvenile systemic lupus erythematosus

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Macrophage activation syndrome in a patient with juvenile systemic lupus erythematosus

Introduction Macrophage activation syndrome (MAS) is a rare but life threatening complication of infectious, neoplastic and rheumatic diseases. Clinically, patients usually have high fever, hepatosplenomegaly, lymphadenopathy and neurologic symptoms. In laboratory results pathognomonic are pancytopenia, decline in the value of sedimentation rate (ESR), increased levels of ferritin and coagulati...

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Association of Macrophage Activating Syndrome with Castleman’s Syndrome in Systemic Lupus Erythematosus

Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still's disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Ira...

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Angioedema secondary to C1 inhibitor deficiency has been rarely reported to be associated with systemic lupus erythematosus. A genetic defect of C1 inhibitor produces hereditary angioedema, which is usually presented with cutaneous painless edema, but edema of the genital area, gastrointestinal and laryngeal tracts have also been reported.In lupus patients, angioedema may be the result of an ac...

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Macrophage activation syndrome: an under-recognised complication in juvenile systemic lupus erythematosus

Macrophage activation syndrome (MAS) is a life-threatening complication in juvenile systemic lupus erythematosus(J SLE). We report three cases of MAS in Juvenile SLE which occurred over the last 6 months. Interestingly, in all the 3 cases, MAS occurred acutely at the time of first presentation of lupus. All of them had fever at presentation. Two of them had lymphadenopathy and splenomegaly and ...

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Macrophage activation syndrome (MAS) in juvenile systemic lupus erythematosus (JSLE): an underrecognized complication?

Open Access Poster presentation Macrophage activation syndrome (MAS) in juvenile systemic lupus erythematosus (JSLE): an underrecognized complication? A Parodi*1, S Davì1, AB Pringe2, S Magni-Manzoni3, P Miettunen4, B BaderMeunier5, G Espada6, S Ozen7, D Wright8, C Magalhaes9, P Woo10, R Kubchandani11, A Grom12, H Michels13, C Wouters14, CE Toro Gutierrez16, G Sterba15, K Hayward17, D Guseinova...

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ژورنال

عنوان ژورنال: Pediatric Rheumatology

سال: 2014

ISSN: 1546-0096

DOI: 10.1186/1546-0096-12-s1-p221