https://scholars.lib.ntu.edu.tw/handle/123456789/525010
標題: | Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases | 作者: | Lin C.I. HSIN-HUI YU JYH-HONG LEE LI-CHIEH WANG YU-TSAN LIN YAO-HSU YANG BOR-LUEN CHIANG |
公開日期: | 2012 | 卷: | 31 | 期: | 8 | 起(迄)頁: | 1223-1230 | 來源出版物: | Clinical Rheumatology | 摘要: | Macrophage activation syndrome (MAS) belongs to secondary hemophagocytic lymphohistiocytosis (HLH) syndrome. It is usually associated with rheumatic diseases. We retrospectively reviewed our hospital's medical records of 102 HLH/MAS patients from the past 20 years. Demographics, clinical data, treatment, and outcomes were analyzed. Among 102 patients, eight patients with underlying juvenile systemic lupus erythematous (two patients), mixed connective tissue disease (one patient), primary antiphospholipid syndrome (one patient), and systemic type juvenile rheumatoid arthritis (sJRA; four patients) with 13 episodes of MAS were studied. Clinical manifestations of MAS included fever (100 %), hepatosplenomegaly (77 %), lymphadenopathy (38 %), skin rash (62 %), and neurological involvement (31 %). Laboratory features included leukopenia (54 %), anemia (46 %), thrombocytopenia (77 %), jaundice (27 %), hypofibrinogenemia (40 %), decreased erythrocyte sedimentation rate (67 %), and elevated liver enzymes (77 %), lactate dehydrogenase (100 %), ferritin (88 %), triglycerides (91 %), C-reactive protein (85 %), plasma D-dimer (50 %), and hemophagocytosis in bone marrow (83 %). The Epstein-Barr virus and adenovirus infection triggered MAS in two patients with sJRA. Methylprednisolone pulse therapy was effective in two out of three patients, and high-dose intravenous immunoglobulin (IVIG) was effective in two out of six patients. Patients with sJRA responded well to corticosteroids and cyclosporine. Complications included opportunistic infection with Pneumocystis jiroveci, multiple organ failure, and intensive care unit myopathy. The mortality rate was one out of eight (12.5 %). Our results showed that MAS could be fatal and complicate various pediatric autoimmune diseases. It generally has a good response to corticosteroids and IVIG. Prompt recognition and timely treatment can result in good outcomes. ? Clinical Rheumatology 2012. |
URI: | https://www.scopus.com/inward/record.uri?eid=2-s2.0-84865986803&doi=10.1007%2fs10067-012-1998-0&partnerID=40&md5=9071a7451f7ec554637f322a89e22b87 https://scholars.lib.ntu.edu.tw/handle/123456789/525010 |
ISSN: | 0770-3198 | DOI: | 10.1007/s10067-012-1998-0 | SDG/關鍵字: | alanine aminotransferase; aspartate aminotransferase; bilirubin; C reactive protein; corticosteroid; cyclosporin A; D dimer; etanercept; ferritin; fibrinogen; immunoglobulin; lactate dehydrogenase; lamotrigine; methylprednisolone; nonsteroid antiinflammatory agent; phenytoin; triacylglycerol; adenovirus infection; adolescent; adult; anemia; antiphospholipid syndrome; article; autoimmune disease; bone marrow; central nervous system disease; child; clinical feature; consciousness disorder; corticosteroid therapy; drug dose reduction; drug efficacy; drug induced disease; drug megadose; drug pulse therapy; drug treatment failure; Epstein Barr virus infection; erythrocyte sedimentation rate; erythrophagocytosis; fever; groups by age; hepatosplenomegaly; human; hypofibrinogenemia; immune deficiency; immunotherapy; inflammation; intensive care; jaundice; laboratory test; leukopenia; lymphadenopathy; macrophage activation syndrome; major clinical study; medical record review; mixed connective tissue disease; mortality; multiple organ failure; myopathy; opportunistic infection; Pneumocystis jiroveci infection; pneumocystosis; priority journal; rash; retrospective study; school child; seizure; systemic lupus erythematosus; thrombocyte count; thrombocytopenia; treatment outcome; Adolescent; Antiphospholipid Syndrome; Arthritis, Juvenile Rheumatoid; Autoimmune Diseases; Child; Cyclosporine; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Macrophage Activation Syndrome; Male; Methylprednisolone; Retrospective Studies; Treatment Outcome |
顯示於: | 醫學系 |
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