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Therapeutic plasma exchange in refractory macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a case-based review

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Abstract

Juvenile idiopathic arthritis (JIA) is a heterogeneous group of arthritis of autoimmune aetiology. Systemic-onset juvenile idiopathic arthritis (soJIA) presents with fever, transient erythematous rash, hepatomegaly, splenomegaly, lymphadenopathy, and serositis. SoJIA presents multiple complications, and the most severe is the macrophage activation syndrome (MAS); the timely treatment of MAS must be established early and aggressively to avoid a fatal outcome. Therapeutic plasma exchange has anecdotally been used in refractory cases. A 66-month-old male with a 1-year illness characterized by evening-predominant, intermittent fever, adenomegalies, urticarial-like rash, arthralgia, and arthritis. Biochemical analysis revealed anaemia, leukocytosis, neutrophilia, hypertriglyceridemia, hyperferritinemia, and hypofibrinogenemia; bone marrow aspirate showed hemophagocytosis. He was diagnosed with SoJIA complicated with MAS. He received multiple treatments with IV human gammaglobulin, cyclosporine, dexamethasone, and tocilizumab without improvement. Plasma replacement treatment was performed. Afterwards, he presented significant improvement. After 3-year-follow-up, he remains in good general condition. We present a refractory case of soJIA complicated with MAS successfully treated with plasma exchange.

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Funding

The work presented in this case report did not receive any funding. No funding was received for this work.

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Authors and Affiliations

  1. Pediatric Allergy and Clinical Immunology Resident, National Institute of Pediatrics (INP), Mexico City, Mexico

    Eduardo Liquidano-Perez

  2. Pediatric dermatologist, The Mayo Clinic Care Network, Mexico City, Mexico

    Gibert Maza-Ramos

  3. Blood Bank Department, INP, Mexico City, Mexico

    José Luis Salazar-Bailón

  4. Immunology Service, National Institute of Pediatrics (INP), Mexico City, Mexico

    Marco Antonio Yamazaki-Nakashimada & Francisco Rivas-Larrauri

Authors
  1. Eduardo Liquidano-Perez
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  2. Gibert Maza-Ramos
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  3. José Luis Salazar-Bailón
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  4. Marco Antonio Yamazaki-Nakashimada
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  5. Francisco Rivas-Larrauri
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All authors made substantial contributions to the conception, design of the work, analysis, and interpretation of data for the work; drafted and revised the work critically for important intellectual content; approved the final version to be published; and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

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Correspondence to Francisco Rivas-Larrauri.

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Liquidano-Perez, E., Maza-Ramos, G., Salazar-Bailón, J.L. et al. Therapeutic plasma exchange in refractory macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a case-based review. Rheumatol Int 43, 183–189 (2023). https://doi.org/10.1007/s00296-022-05231-z

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