Macrophage activation syndrome in @entity1 with systemic onset XXXX : clinical experience from northwest India.

@entity1 @entity3329 @entity1667 @entity1087 @entity75 @entity14822 @entity15835 @entity1003 @entity4932 @entity828 @entity221 @entity2753 @entity617
The objective of this study is to describe the clinical and laboratory features of @entity15835 ( @entity15835 ) in systemic onset @entity4932 (SOJIA) at a tertiary care center in northwest India. Review of medical records of all @entity1 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 @entity1 (5 @entity1 and 1 @entity1 ) with SOJIA and @entity15835 were identified. Mean age at time of disease onset was 6.5 years. @entity15835 was the presenting manifestation of SOJIA in 4 @entity1 . Clinical manifestations included @entity221 (6/6), clinical shock (6/6), @entity75 (5/6), generalized @entity3329 (4/6), @entity828 (3/6), @entity1087 and abdominal tenderness (3/6), cardiac involvement (3/6), and meningeal irritation (2/6). Laboratory findings at onset of @entity15835 included decreasing total leukocyte and platelet counts, @entity1003 , elevated transaminases, @entity2753 , and @entity14822 . Hemophagocytosis was demonstrable in the bone marrow in 4 @entity1 and in the lymph node in 1. For treatment, we used intravenous @entity1667 (4/6), oral @entity617 (2/6), and intravenous immunoglobulin (2/6). Outcome was favorable in all @entity1 except one who died of rapidly progressive disease. This paper describes the experience of JIA-related @entity15835 in a tertiary Indian center. We have shown that @entity15835 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.
