title: Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: clinical experience from northwest India.
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.
authors.affiliation: Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India. surjitsinghpgi@rediffmail.com
authors.forename: Surjit
authors.initials: S
authors.lastname: Singh
authors.forename: Shanmuganathan
authors.initials: S
authors.lastname: Chandrakasan
authors.forename: Jasmina
authors.initials: J
authors.lastname: Ahluwalia
authors.forename: Deepti
authors.initials: D
authors.lastname: Suri
authors.forename: Amit
authors.initials: A
authors.lastname: Rawat
authors.forename: Nishath
authors.initials: N
authors.lastname: Ahmed
authors.forename: Reena
authors.initials: R
authors.lastname: Das
authors.forename: M U S
authors.initials: MU
authors.lastname: Sachdeva
authors.forename: Neelam
authors.initials: N
authors.lastname: Varma
mesh_terms: D001171:Arthritis, Juvenile / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007194:India; D055501:Macrophage Activation Syndrome / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use
chemical_list: D005938:Glucocorticoids; D008775:Methylprednisolone