Relapsing macrophage activating syndrome in a 15-year-old girl with Still's disease: a case report

Abstract

IntroductionMacrophage activating syndrome is a severe, potentially life-threatening condition that may accompany Still's disease. It is characterized by fever, hepatosplenomegaly, lymphadenopathy, severe cytopenia, serious liver dysfunction, coagulopathy and neurologic involvement. The principal treatment for patients with this syndrome includes etoposide 150 mg/2 M twice a week for two weeks, dexamethasone 10 mg/2 M for two weeks and cyclosporine 3 mg/kg to 5 mg/kg for a longer period. Cases of relapse of macrophage activating syndrome are relatively rare.Case presentationWe describe the case of a 15-year-old Iraqi girl with Still's disease who developed macrophage activating syndrome with acute respiratory distress syndrome that required resuscitation and mechanical ventilation. Following intensive treatment, including high dose steroids and cyclosporine, the patient improved significantly. Two weeks after cyclosporine was discontinued, however, she was readmitted with an acute relapse of macrophage activating syndrome manifested by spiking fever, arthralgias, maculopapular rash and leukocytosis. This time the patient recovered following the reintroduction of treatment with cyclosporine and the addition of mycophenolate mofetil (Cellcept).ConclusionWe believe that cyclosporine is a cornerstone for the treatment of Still's disease. We recommend continuing this medication for several weeks following the patient's clinical recovery in order to prevent macrophage activating syndrome relapses.

The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review

“…Even though there are reports of CyA efficacy as steroid sparing agent with a rapid effect and a low recurrence rate [153], its use in adult onset Still's disease is currently limited to some of the severe manifestations of Still's disease [154]. Indeed, CyA, alone or in combination with other pharmacological tools, might be considered as an option in the management of disseminated intravascular coagulation [155,156], macrophage activation syndrome (MAS) [157,158], acquired amegakariocytic thrombocytopenia [159] and severe hepatic failure [160]. Details of studies investigating CyA in adult onset Still's disease are presented in Table 8.…”

2013 Update of the 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for the Medical Therapy of Children With Systemic Juvenile Idiopathic Arthritis and Tuberculosis Screening Among Children Receiving Biologic Medications

“…Use of a calcineurin inhibitor was recommended as one therapeutic option for patients with features concerning for MAS (level C) (65)(66)(67)(68)(69).…”

Recurrent Macrophage Activation Syndrome Associated With Heterozygous Perforin W374X Gene Mutation in a Child with Systemic Juvenile Idiopathic Arthritis

“…Recurrent MAS is rarely reported. 22,23 Moreover, the family's noncompliance with treatment may have played a role in the poor outcome, as previously reported. 23 Initial genetic evaluations of patients with MAS secondary to sJIA focused primarily on UNC13D, one of the genes responsible for familial HLH.…”