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Rescue Therapy With Continuous Intravenous Anakinra Infusion and Plasma Exchange in Refractory Cytokine Storm of
Ruqaiya Al Jashmi1, Aza Al Sawafi1, Safiya Al Abrawi1
1Paediatric Rheumatology Unit, Child Health Department, Royal Hospital, Muscat, Oman, moh.gov.om.
Introduction:
Macrophage activation syndrome (MAS) is one of the most common fatal complications of inflammatory diseases in children. In recent studies, Interleukin 1 inhibitors have been shown to be effective in suppressing the cytokine storm in MAS. There is limited literature describing the role of plasmapheresis in the cytokine storm syndromes as in MAS.
Case Description:
A six-year-old boy presented with a 1-month history of daily fever, arthritis, cervical lymphadenopathy, skin rash, and elevated inflammatory markers. After multidisciplinary evaluation, he was diagnosed with systemic onset Juvenile Idiopathic Arthritis (sJIA) and started on IV methylprednisolone and SC anakinra. He later developed status epilepticus, required intubation, and progressed to multiorgan failure, needing inotropes and dialysis. Following a brief afebrile period, he worsened again, and labs confirmed MAS relapse with ferritin levels peaking at 26,000. Escalated treatment included switching to IV continuous anakinra, adding ciclosporin, and starting plasmapheresis. After three sessions of plasma exchange, he significantly improved, was extubated, and weaned off inotropes. However, attempts to switch anakinra back to SC failed twice, with recurrence of fever, rash, and elevated ferritin. He remained in PICU on IV continuous anakinra for over 3 weeks. When ferritin dropped to 900, an IV bolus regimen was trialed for a week, followed by a successful switch to SC anakinra. The child was discharged home asymptomatic with normalized labs.
Conclusion:
Anakinra has been used off-label in critically ill patients with MAS, thrombocytopenia, subcutaneous edema, and neurological dysfunction. Tapering from intravenous Anakinra to subcutaneous was challenging. This case taught us that tapering must begin at the right time to avoid hiccups or unfavorable outcomes. As reported in a few studies, plasmapheresis combined with immunosuppressants can reduce MAS-associated mortality by rapidly removing cytokines from the body.
Insights
Plasmapheresis effectively treated a child with macrophage activation syndrome (MAS) relapse, a severe complication of juvenile idiopathic arthritis. Combining plasmapheresis with anakinra and other immunosuppressants rapidly reduced severe symptoms and normalized inflammatory markers.
Area of Science:
- Pediatric Rheumatology
- Critical Care Medicine
- Immunology
Background:
- Macrophage activation syndrome (MAS) is a life-threatening complication in pediatric inflammatory diseases.
- Interleukin-1 inhibitors show promise in managing MAS by suppressing cytokine storms.
- Limited data exists on plasmapheresis for cytokine storm syndromes like MAS.
Purpose of the Study:
- To describe the successful use of plasmapheresis in a pediatric patient with refractory MAS.
- To highlight the challenges and successful strategies in managing critically ill patients with MAS.
Main Methods:
- A six-year-old boy diagnosed with systemic Juvenile Idiopathic Arthritis (sJIA) developed MAS with multiorgan failure.
- Treatment included escalating doses of anakinra, ciclosporin, and plasmapheresis.
- Plasmapheresis was administered over three sessions, followed by careful management of anakinra tapering.
Main Results:
- Plasmapheresis led to significant clinical improvement, including resolution of multiorgan failure and extubation.
- Tapering from intravenous to subcutaneous anakinra proved challenging, requiring careful timing to prevent relapse.
- The patient was eventually discharged asymptomatic with normalized laboratory markers.
Conclusions:
- Plasmapheresis, in conjunction with immunosuppressive therapy, can be a vital tool in reducing mortality in MAS.
- Optimal timing for tapering immunosuppressive therapy, such as anakinra, is crucial for sustained recovery.
- This case underscores the potential of plasmapheresis in managing severe cytokine storm syndromes in children.
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