Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: clinical experience from northwest India |
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Authors: | Surjit Singh Shanmuganathan Chandrakasan Jasmina Ahluwalia Deepti Suri Amit Rawat Nishath Ahmed Reena Das M U S Sachdeva Neelam Varma |
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Institution: | (1) Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India;(2) Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India |
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Abstract: | The objective of this study is to describe the clinical and laboratory features of macrophage activation syndrome (MAS) in
systemic onset juvenile idiopathic arthritis (SOJIA) at a tertiary care center in northwest India. Review of medical records
of all children with SOJIA admitted during the period January 1995–December 2008 in Pediatric Allergy and Immunology Unit,
Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, was done. Six patients (5
boys and 1 girl) with SOJIA and MAS were identified. Mean age at time of disease onset was 6.5 years. MAS was the presenting
manifestation of SOJIA in 4 patients. Clinical manifestations included fever (6/6), clinical shock (6/6), encephalopathy (5/6),
generalized lymphadenopathy (4/6), hepatosplenomegaly (3/6), jaundice and abdominal tenderness (3/6), cardiac involvement
(3/6), and meningeal irritation (2/6). Laboratory findings at onset of MAS included decreasing total leukocyte and platelet
counts, coagulopathy, elevated transaminases, hyponatremia, and lipid abnormalities. Hemophagocytosis was demonstrable in
the bone marrow in 4 patients and in the lymph node in 1. For treatment, we used intravenous methylprednisolone (4/6), oral
prednisolone (2/6), and intravenous immunoglobulin (2/6). Outcome was favorable in all patients except one who died of rapidly
progressive disease. This paper describes the experience of JIA-related macrophage activation syndrome in a tertiary Indian
center. We have shown that MAS can be the early presenting manifestation of evolving SOJIA. Early diagnosis and aggressive
management can have a significant impact on the mortality associated with this syndrome. We stress on the role of glucocorticoids
in the management of this condition and believe that glucocorticoids have a far more important role in the management of this
condition than what has been previously reported. |
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