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.

Macrophage activation syndrome: early diagnosis is key

“…They also showed a certain amount of irritability, with lethargy, comas, and headaches 10. These CNS involvements, as found in MAS, were more prominent than in active SJIA; however, they were not regarded as an early presentation for an onset of MAS 34. Hemorrhagic manifestations were often seen in a full-blown MAS, which ranged from an easy bruising to a mucosal bleeding, a gastrointestinal bleeding, and a disseminated intravascular coagulation.…”

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

“…3 More recently, glucocorticoids, along with other immunosuppressive therapy, were reported to be effective in 13 children with sJIA in China 71 and in 6 children with sJIA in India. 72 Hence, high dose steroids are routinely used to treat MAS as part of sJIA.…”

“…Success of intravenous immunoglobulin requires treatment early in the course of MAS. 82 Although often useful for the treatment of infection-triggered MAS 83,84 and a few children with sJIA, 72,85 children with MAS as part of sJIA are often refractory to intravenous immunoglobulin. 86 A more aggressive approach using anti-thymocyte globulin has been proposed for FHLH 87 and has been used successfully in two patients with probable MAS.…”

Diagnosing haemophagocytic syndrome

“…Although termed ‘secondary’, on some occasions HLH can be the presenting manifestation of the underlying condition in a previously well child. It can be difficult to distinguish from systemic sepsis or autoimmune diseases 5 6. Given the risk of significant morbidity and mortality, recognition of HLH/MAS and its early management are important skills for all paediatricians.…”