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Chronic Infantile Neurological Cutaneous Articular syndrome


Other Names for this Disease
  • CINCA
  • CINCA syndrome
  • Infantile Onset Multisystem Inflammatory Disease
  • IOMID
  • Multisystem inflammatory disease, neonatal-onset
More Names
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Overview


Chronic infantile neurologic cutaneous and articular (CINCA) syndrome, also known as 'neonatal onset multisystem inflammatory disease,' or NOMID, is a congenital inflammatory disorder characterized by a triad of neonatal onset of cutaneous symptoms (skin rash), chronic meningitis, and joint pain with recurrent fever and inflammation.[1] CINCA is the most severe form of the cryopyrin associated periodic syndromes (CAPS) caused by mutations in the CIAS1/NLRP3 gene. About 50% of affected individuals have mutations in this gene. This condition is inherited in an autosomal dominant fashion.[2][3]
Last updated: 7/15/2009

References

  1. CINCA Syndrome. Online Mendelian Inheritance in Man (OMIM). 2006; http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=607115. Accessed 7/15/2009.
  2. Hoffman H. Neonatal-onset Multisystem Inflammatory Disease. National Organization for Rare Disorders (NORD). 2007; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Neonatal-onset%20Multisystem%20Inflammatory%20Disease. Accessed 7/15/2009.
  3. Periodic Fever Syndrome. Cleveland Clinic. 2009; http://my.clevelandclinic.org/disorders/periodic_fever_syndrome/rheumatology_overview.aspx. Accessed 7/15/2009.
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Basic Information

  • The Cleveland Clinic provides an overview of the different types of periodic fever syndromes.
  • Genetics Home Reference (GHR) contains information on Chronic Infantile Neurological Cutaneous Articular syndrome. This website is maintained by the National Library of Medicine.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Chronic Infantile Neurological Cutaneous Articular syndrome. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles