Background Macrophage activation syndrome (MAS) can, at times, be the presentation of pediatric lupus and diagnosis requires high index of suspicion.
Methods We retrospectively studied 140 pediatric lupus patients from January 1993- November 2018 and collected clinical and laboratory data of patients (3) who had MAS as presenting manifestation.
Results Case 1 was 11-year-old girl with fever for 4 months associated with rash and generalized body swelling for 1 month. Examination revealed rash over malar area and ear lobules, anasarca, hepatomegaly, bilateral pleural and pericardial effusion. In view of multisystem involvement a possibility of lupus was considered which was confirmed by investigations (table 1). She had elevated ferritin and fasting triglyceride and low fibrinogen. A clinical possibility of lupus with associated MAS was considered. She received pulses of methylprednisolone, one dose of intravenous immunoglobulin following which she improved. In view of nephrotic range proteinuria she was started on induction regimen with cyclophosphamide and shifted to mycophenolate in maintenance. Her initial SLEDAI-2k was 32- this decreased to 4 at 3 year follow-up.
Case 2 was a 9-year-old girl with fever, rash, generalized body swelling for 1 month and altered sensorium for 4 days. Examination revealed pallor, oral ulcers and hepatomegaly. She was in shock at presentation. In view of multisystem involvement a possibility of lupus was considered which was confirmed by investigations (table 1). She had pericardial effusion and low ejection fraction (25%). A possibility of MAS was considered and investigations revealed hyperferritinemia, elevated triglyceride and hypofibrinoginemia. She was given methylprednisolone pulses and continued on oral prednisolone, mycophenolate and hydroxychloroquine. Her initial SLEDAI-2k was 17- this decreased to 0 at 3 year follow-up.
Case 3 was an 8-year-old girl who had fever, rash and body swelling for 15 days. She had tachycardia, tachypnea, pallor, anasarca, subconjunctival bleed and frontal alopecia. She had pleural and pericardial effusion. In view of multisystem involvement a possibility of lupus was considered which was confirmed by investigations (table1). She had high ferritin and triglyceride. So a possibility of MAS was considered and she received pulse methyl prednisolone and intravenous immunoglobulin. She also had hematuria and proteinuria (renal biopsy could not be performed as she was sick and had thrombocytopenia) so was given pulse cyclophosphamide followed by mycophenolate. Her initial SLEDAI-2k was 25- this decreased to 0 at 2 year follow-up.
Conclusions MAS can be the presenting manifestation of pediatric lupus and may contribute to disease severity and requires aggressive management
Funding Source(s): None
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