Macrophage activation syndrome as a complication of systemic juvenile idiopathic arthritis
Keywords:
systemic juvenile idiopathic arthritis, Still's disease, rituximab, macrophage activation syndromeAbstract
Systemic Juvenile Idiopathic Arthritis, also known as Still's disease, is considered an autoinflammatory disorder and is often the most complex and severe of all clinical forms of the disease. It usually takes the form of repeated bouts of activity, interspersed with periods of remission. We present the case of a 4-year-old female patient, diagnosed with Still's disease at 2 years of age. Currently undergoing treatment with triple induction therapy: chloroquine, methotrexate and salazosulfapyridine with persistently high disease activity due to JADAS 27. He comes to the clinic due to fever, general condition, and respiratory manifestations of three days of evolution interpreted as an infectious respiratory process under. Antibiotic treatment is started without signs of improvement. At 7 days the clinical picture worsens, and the diagnosis of Macrophage Activation Syndrome is raised. A steroid treatment protocol is started in combination with other drugs of proven efficacy for this clinical situation (ethopside, cyclosporine, methotrexate). Antibiotic policy was reassessed without achieving a satisfactory response and it was decided to introduce rituximab, which provides excellent results. After three months of difficult management, the patient was released from the hospital recovered from this complication and with a low level of activity of the underlying disease.
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