Macrophage activation syndrome presenting in a child with concomitant systemic lupus erythematosus and HIV infection: A case report

Abstract

          Macrophage activation syndrome (MAS) is a life threatening complication of several autoimmune diseases. Systemic lupus erythematosus (SLE) is infrequently reported in association with HIV; the incidence of MAS in coexistence of SLE and HIV is extremely rare. Herein, we report a case of SLE coexisting with HIV infection in a child manifesting as MAS. A 13-year-old girl diagnosed with a congenital HIV infection, undetectable viral load, presented with fever and edema for 2 weeks. Physical examination revealed a body temperature of 39.5°C, puffy eyelids and splenomegaly. Her complete blood count showed hemoglobin at 10.8 g/dL, WBC 1,600 /mm³ (absolute neutrophil count 660/mm³) and platelets 57,000/mm³. Further investigations revealed hyperferritinemia (31,132.3 ng/mL), hypofibrinogenemia (130.6 mg/dL), hypertriglyceridemia (537 mg/dL) and increased hemophagocytic activity in bone marrow. Her blood urea nitrogen and creatinine were 61.4 and 2.58 mg/dL, respectively. Autoimmune profiles (ANA and anti-dsDNA)  were positive with high titers; hypocomplementemia (C3 and C4) was observed. A kidney biopsy was compatible with lupus glomerulonephritis class IV. She was diagnosed as MAS with SLE and subsequently treated with HLH-2004 treatment protocol consisting of IVIG, dexamethasone and etoposide. She did not respond to treatment and died of multiple organ failure 10 days after initiation of treatment. Macrophage activation syndrome in concomitant systemic lupus erythematous and HIV infection is serious condition and can be fatal. Physicians taking care of children with HIV infection should be vigilant regarding these conditions, particularly in those presenting with prolonged fever and deterioration of organ function.