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Role of plasma exchange, leukocytapheresis, and plasma diafiltration in management of refractory macrophage activation syndrome
Author(s) -
Kinjo Noriko,
Hamada Kazuya,
Hirayama Chika,
Shimizu Masaki
Publication year - 2018
Publication title -
journal of clinical apheresis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.697
H-Index - 46
eISSN - 1098-1101
pISSN - 0733-2459
DOI - 10.1002/jca.21570
Subject(s) - diafiltration , medicine , refractory (planetary science) , plasmapheresis , leukapheresis , apheresis , macrophage , immunology , membrane , antibody , microbiology and biotechnology , platelet , biochemistry , stem cell , cd34 , biology , microfiltration , chemistry , physics , astrobiology , in vitro
Abstract 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.