Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders |
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Authors: | Sawhney S Woo P Murray K J |
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Institution: | Department of Rheumatology, Great Ormond Street Hospital, London WC1N 3JH, UK. |
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Abstract: | AIMS—To review the precipitating
events, clinical features, treatment, and outcome of macrophage
activation syndrome (MAS). METHODS—Retrospective review of
cases of MAS from a prospectively collected database of children with
rheumatic diseases from 1980to 2000. RESULTS—Nine patients (eight girls)
were considered to have evidence of MAS. The primary diagnosis was
systemic onset juvenile idiopathic arthritis in seven, enthesitis
related arthritis in one, and chronic infantile neurological cutaneous
articular syndrome in one. Mean age of onset was 5.7 years, and
duration prior to MAS, 4.2 years. No medication was identified as a
trigger. Eight had infections prior to MAS; specific infectious agents
were identified in four. High grade fever, new onset
hepatosplenomegaly, and lymphadenopathy were common clinical features.
Platelet counts fell dramatically, from an average of 346 to 99 × 109/l. Mean erythrocyte sedimentation rate (in three
patients) fell from 115 to 28 mm/h. Eight had abnormal liver function
during the disease course, and six had coagulopathy. Bone marrow
examination supported the diagnosis with definite haemophagocytosis in
four of seven. All received high dose steroids (eight intravenous, one
oral), five cyclosporin, two cyclophosphamide, and one antithymocyte globulin. Two of three patients with significant renal impairment died. CONCLUSION—MAS is a rare and
potentially fatal complication of childhood rheumatic disorders. Most
of our patients were female, and most cases were preceded by infection.
Bone marrow studies support the diagnosis. Deranged renal function may
be a poor prognostic sign. Aggressive early therapy is essential.
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