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EXTREME EFFICIENCY OF ANTI-INTERLEUKIN 1 AGENT (ANAKINRA) IN A JAPANESE CASE OF CINCA SYNDROME
  1. M Sato1,
  2. H Kawashima1,
  3. H Ioi1,
  4. S Nishimata1,
  5. G Yamanaka1,
  6. Y Kashiwagi1,
  7. K Takekuma1,
  8. A Hoshika1
  1. 1Department of Paediatrics, Tokyo Medical University, Tokyo, Japan

Abstract

Objective Neonatal Onset Multisystem Inflammatory Disease (NOMID) which is also known as Chronic Infantile Neurological Cutaneous and Articular (CINCA) syndrome is characterized by the triad of cutaneous urticarial rash, chronic meningitis and arthropathy, which starts most often soon after birth. The long-term prognosis is poor, with progressive deafness and visual impairment, and worsening of the central nervous system manifestations.

Methods We report on a Japanese boy with NOMID who was admitted and discharged several times. When high fever continued, methylprednisolone 30 mg/kg was given for 2–3 days. In October he had a high fever with low blood pressure and heart failure, which could not be explained. Since Il-6 was extremely high, a combined treatment of continuous hemodiafiltration (CHDF) with steroid pulse therapy was done in order to remove cytokines. After the treatment commenced his ejection fraction recovered and the levels of IL-6 and CRP normalized.

Two weeks later IL-6 increased gradually again. As given for juvenile idiopathic arthritis, ibuprofen, steroids and methotrexate were used. We tried anti-TNF antibody intravenously, however there was no improvement in his symptoms. Anti-interleukin (IL)-1 agent (Anakinra) is currently used as a new type of treatment.

Results Anakinra was very effective in reducing the dose of predonisolon. His CRP and IL-6 levels were normal for three months with mild flare.

Conclusions We report on a Japanese boy with NOMID where a combined treatment of CHDF and steroid pulse therapy was effective. The effect was temporary but useful for recovery from serious conditions.

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