A Case Report of Neonatal Onset Multisystemic Inflammatory Disease in Japan Treated With Continuous Hemodiafiltration and Steroid Pulse Therapy

Authors: Kawashima, Hisashi; Sato, Akiko¹; Nishimata, Shigeo¹; Yamada, Naoto¹; Kashiwagi, Yasuyo¹; Watanabe, Kiyoko¹; Takekuma, Kouji¹; Hoshika, Akinori¹; Ozawa, Takurou²

Source: Therapeutic Apheresis and Dialysis, Volume 11, Number 3, June 2007 , pp. 232-234(3)

Publisher: Wiley-Blackwell

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Abstract:

Neonatal onset multisystem inflammatory disease (NOMID), which is also known as chronic infantile neurological cutaneous and articular syndrome, is a rare disease that is characterized by the triad of cutaneous rash, chronic meningitis and arthropathy. The long-term prognosis is poor, with progressive deafness and visual impairment, and worsening of the central nervous system manifestations. Some fatal cases have been reported secondary to infection, vasculitis and amyloidosis. We recently managed a typical case of NOMID where the combined treatment of continuous hemodiafiltration and steroid pulse therapy was effective. The patient showed repeated flare ups of fever, arthralgia and meningitis accompanied with high levels of cytokines. The effect was temporary, but useful in recovery from such a serious condition.

Keywords: Cold-induced autoinflammatory syndrome; Interleukin-2; Neonatal onset multisystem inflammatory disease; Prednisolone

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1744-9987.2007.00465.x

Affiliations: 1: Departments of Pediatrics and 2: Anesthesia, Tokyo Medical University, Tokyo, Japan

Publication date: 2007-06-01