Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases
Journal
Clinical Rheumatology
Journal Volume
31
Journal Issue
8
Pages
1223-1230
Date Issued
2012
Author(s)
Abstract
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.
SDGs
Other Subjects
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
Type
journal article