[MIM 607 115]

(CINCA: Chronic Infantile Neurologic Cutaneous Articular syndrome)

Acronym for Neonatal-Onset Multi-system Inflammatory Disease

This rare disorder belongs to the group of autoinflammatory diseases including pyoderma gangrenosum, mediterranean periodic fever, Blau syndrome, Muckle-Wells syndrome.

In 60% of cases, it is due to a mutation of the CIAS1 gene on chromosome 1q44 which codes for crypyrine. This protein is involved in the formation of inflammasomes that activate caspase-1, resultng in the formation of interleukine 1. The mutation results in the production of inflammasomes that is independent of the presence of infection.

Childhood: urticarian rash, fever, neurologic symptoms (headache, aseptic meningitis, chronic, developmental retardation, progressive hearing loss) and skeletal anomalies (enlargement of the patella). Adult mortality is estimated at 20%.

Treatment: daily subcutaneous injections of IL-1 receptor antagonist, corticosteroid therapy

Anesthetic implications:

intracranial hypertension, high temperature (differential diagnosis between periodic fever and intercurrent infection), eye protection, laryngeal edema; neuraxial blocks are effective but keep in mind the pharmacology of the LA in a context of chronic aseptic meningitis; corticosteroid supplementation. Provide the daily dose of IL1 receptor antagonist; monitor CRP (C Reactive Protein).

References : 

• Lauro CF, Goldbasch-Mansky R, Schmidt M, Quezado ZMN. 
  The anesthetic management of children with neonatal-onset multi-system inflammatory disease. 
  Anesth Analg 2007; 105: 351-7.
• Höhne C,  Burkhardt U. 
  Anesthesia in an infant with CINCA syndrome. 
  Pediatr Anesth 2008; 18: 575-7.

Updated: April 2017