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Recognising macrophage activation syndrome

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A profound drop in platelets indicates macrophage activation syndrome (MAS) in rheumatic disease in children. This is just one observation by Sawhney et al to emerge from a retrospective review over 20 years in a tertiary paediatric rheumatology unit.

The review of nine children—the largest reported series so far—presents valuable information on this rare, poorly understood, and potentially fatal complication of systemic onset juvenile idiopathic arthritis (SOJIA) and other forms of juvenile idiopathic arthritis. Better understanding is vital for correct early diagnosis and treatment.

Seven children had SOJIA, one enthesis related arthritis, and one chronic infantile neurological cutaneous articular syndrome. Mean duration before MAS was about four years, but it also occurred at presentation of the rheumatic disease. Characteristic features were intense, persistent fever, new onset enlarged liver and spleen, and swollen lymph glands; an early drastic drop in platelet count; and drops of varying extent in white blood cells and haemoglobin. Phagocytosis of platelets by macrophages in bone marrow confirmed the diagnosis in four of seven children. Abnormal liver function was also observed. Treatment was with high dose steroids in all children plus other immunosuppressants in some; two children with renal disease died.

A preceding infection occurred in most children. The authors found no trigger for MAS among the treatments received at or before its onset. As they describe, MAS in rheumatic disease has special features which help to distinguish it from other macrophage disorders and the flare ups characteristic of SOJIA—essential to avoid delayed diagnosis.

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