Role of plasma exchange, leukocytapheresis, and plasma diafiltration in management of refractory macrophage activation syndrome

Macrophage activation syndrome (MAS) is a life‐threating complication of systemic juvenile idiopathic arthritis (s‐JIA). Steroid and cyclosporine (CsA) are effective for MAS, but, treatment for steroid‐ and CsA‐resistant patients is still challenging. We report the case of steroid and CsA resistant...

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Bibliographic Details
Published in:Journal of clinical apheresis 2018-02, Vol.33 (1), p.117-120
Main Authors: Kinjo, Noriko, Hamada, Kazuya, Hirayama, Chika, Shimizu, Masaki
Format: Article
Language:English
Subjects:
Apheresis
Arthritis, Juvenile - complications
Arthritis, Juvenile - diagnosis
Cytokines
Disease Management
Filtration
Humans
Kawasaki disease
Leukapheresis
leukocytapheresis
macrophage activation syndrome
Macrophage Activation Syndrome - etiology
Macrophage Activation Syndrome - therapy
Plasma
plasma diafiltration
Plasma Exchange
proinflammatory cytokines
systemic juvenile idiopathic arthritis
Treatment Outcome
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Summary:Macrophage activation syndrome (MAS) is a life‐threating complication of systemic juvenile idiopathic arthritis (s‐JIA). Steroid and cyclosporine (CsA) are effective for MAS, but, treatment for steroid‐ and CsA‐resistant patients is still challenging. We report the case of steroid and CsA resistant s‐JIA associated MAS misdiagnosed as Kawasaki disease (KD), who was successfully treated with the combination of plasma exchange (PE) and leukocytapheresis (LCAP) followed by plasma diafiltration (PDF). PE + LCAP effectively removed proinflammatory cytokines and reduced the number of peripheral white blood cells. Furthermore, PDF also removed proinflammatory cytokines as effectively as PE + LCAP. Early diagnosis of s‐JIA is necessary to avoid developing MAS. The measurement of serum ferritin and IL‐18 levels are useful for differentiating s‐JIA from KD. Apheresis therapies are an alternative option to induce remission for severe patients with steroid‐ or CsA‐resistant MAS.
ISSN:0733-2459
1098-1101
DOI:10.1002/jca.21570