title: An important complication of a child with juvenile idiopathic arthritis: macrophage activation syndrome.
abstract: A 4-year-old girl, a known case of juvenile idiopathic arthritis for 2 years presented to us with high-grade fever and abdominal distension for 2 months. On examination, her temperature was 104 ° F and she was found to be pale with bilateral cervical lymphadenopathy of up to 3 × 3 cm in size. Her liver was enlarged with a firm consistency and a span of 12 cm. Her spleen was enlarged up to 3 cm along its long axis. The rest of her systemic examination was normal. Laboratory investigations revealed leucocytosis, anaemia and thrombocytopenia with a mildly elevated erythrocyte sedimentation rate. Serum ferritin was 16 500 ng/dL and lactate dehydrogenase was 2311 U/L. A bone marrow aspirate showed macrophages showing ingested nuclei. She was diagnosed as having macrophage activation syndrome and was initiated on intravenous methylprednisolone 300 mg daily for 3 days and was switched over to oral prednisolone 2 mg/kg/day. She is currently doing well on follow-up.
authors.affiliation: Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu, India.
authors.forename: Yuvaram N V
authors.initials: YN
authors.lastname: Reddy
authors.forename: Navin
authors.initials: N
authors.lastname: Bhatia
authors.forename: Julius Xavier
authors.initials: JX
authors.lastname: Scott
authors.forename: Priyathersini
authors.initials: P
authors.lastname: Nagarajan
mesh_terms: D001171:Arthritis, Juvenile / Q000150:complications*; D001853:Bone Marrow / Q000473:pathology; D002675:Child, Preschool; D005260:Female; D006801:Humans; D055501:Macrophage Activation Syndrome / Q000209:etiology* / Q000473:pathology
references.citation: JAMA. 2011 Dec 7;306(21):2340-7
references.pmid: 22056397
references.citation: Inflamm Res. 2000 Jan;49(1):20-6
references.pmid: 10778917
references.citation: Arthritis Rheum. 2011 Feb;63(2):545-55
references.pmid: 21280009
references.citation: J Pediatr. 1996 Nov;129(5):630-2
references.pmid: 8917224
references.citation: Curr Opin Rheumatol. 2002 Sep;14(5):548-52
references.pmid: 12192253
references.citation: Mediators Inflamm. 1999;8(4-5):253-9
references.pmid: 10704080
references.citation: Blood. 1991 Dec 1;78(11):2918-22
references.pmid: 1954380
references.citation: Arch Dis Child. 2001 Nov;85(5):421-6
references.pmid: 11668110
references.citation: Minerva Pediatr. 2011 Dec;63(6):459-66
references.pmid: 22075800
references.citation: Med Pediatr Oncol. 1997 Sep;29(3):157-66
references.pmid: 9212839
references.citation: J Rheumatol. 2001 Apr;28(4):865-7
references.pmid: 11327264