Neonatal onset multisystem inflammatory disease

Common Name(s)

Neonatal onset multisystem inflammatory disease, Chronic infantile neurological cutaneous articular syndrome, CINCA, Infantile multisystem inflammatory disease, NOMID

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.  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.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Neonatal onset multisystem inflammatory disease" for support, advocacy or research.

The NOMID Alliance

The NOMID Alliance is a 501 (c)(3) non-profit public charity dedicated to promoting awareness, proper diagnosis and treatment, and improved care for people with autoinflammatory diseases.

Last Updated: 16 Aug 2013

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Neonatal onset multisystem inflammatory disease" for support, advocacy or research.

The NOMID Alliance

The NOMID Alliance is a 501 (c)(3) non-profit public charity dedicated to promoting awareness, proper diagnosis and treatment, and improved care for people with autoinflammatory diseases.

http://www.nomidalliance.org

Last Updated: 16 Aug 2013

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General Resources

Comparison Chart of Systemic Autoinflammatory DIseases

This comparative chart of Autoinflammatory Diseases lists symptoms, lab findings, genetic mutations and images for each disease.

Uploaded By: The NOMID Alliance

Updated 15 Aug 2013

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Neonatal onset multisystem inflammatory disease" returned 10 free, full-text research articles on human participants. First 3 results:

Sustained response and prevention of damage progression in patients with neonatal-onset multisystem inflammatory disease treated with anakinra: a cohort study to determine three- and five-year outcomes.
 

Author(s): Cailin H Sibley, Nikki Plass, Joseph Snow, Edythe A Wiggs, Carmen C Brewer, Kelly A King, Christopher Zalewski, H Jeffrey Kim, Rachel Bishop, Suvimol Hill, Scott M Paul, Patrick Kicker, Zachary Phillips, Joseph G Dolan, Brigitte Widemann, Nalini Jayaprakash, Frank Pucino, Deborah L Stone, Dawn Chapelle, Christopher Snyder, John A Butman, Robert Wesley, Raphaela Goldbach-Mansky

Journal: Arthritis Rheum.. 2012 Jul;64(7):2375-86.

 

Blocking interleukin-1 with anakinra in patients with the autoinflammatory syndrome neonatal-onset multisystem inflammatory disease (NOMID) reduces systemic and organ-specific inflammation. However, the impact of long-term treatment has not been established. This study was undertaken ...

Last Updated: 28 Jun 2012

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A somatic NLRP3 mutation as a cause of a sporadic case of chronic infantile neurologic, cutaneous, articular syndrome/neonatal-onset multisystem inflammatory disease: Novel evidence of the role of low-level mosaicism as the pathophysiologic mechanism underlying mendelian inherited diseases.
 

Author(s): Juan I Aróstegui, Ma Dolores Lopez Saldaña, Mariona Pascal, Daniel Clemente, Marta Aymerich, Francesc Balaguer, Ajay Goel, Concepción Fournier del Castillo, Josefa Rius, Susana Plaza, Juan Carlos López Robledillo, Manel Juan, Mercedes Ibañez, Jordi Yagüe

Journal: Arthritis Rheum.. 2010 Apr;62(4):1158-66.

 

Chronic infantile neurologic, cutaneous, articular syndrome (CINCA), also known as neonatal-onset multisystem inflammatory disease (NOMID), is a severe, early-onset autoinflammatory disease characterized by an urticaria-like rash, arthritis/arthropathy, variable neurologic involvement, ...

Last Updated: 14 Apr 2010

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Long-term efficacy of the interleukin-1 receptor antagonist anakinra in ten patients with neonatal-onset multisystem inflammatory disease/chronic infantile neurologic, cutaneous, articular syndrome.
 

Author(s): Bénédicte Neven, Isabelle Marvillet, Celine Terrada, Alice Ferster, Nathalie Boddaert, Vincent Couloignier, Graziella Pinto, Anne Pagnier, Christine Bodemer, Bahram Bodaghi, Marc Tardieu, Anne Marie Prieur, Pierre Quartier

Journal: Arthritis Rheum.. 2010 Jan;62(1):258-67.

 

Cryopyrin-associated periodic syndromes (CAPS) are a group of rare autoinflammatory diseases. Neonatal-onset multisystem inflammatory disease (NOMID)/chronic infantile neurologic, cutaneous, articular syndrome (CINCA syndrome) is the most severe phenotype, with fever, rash, articular ...

Last Updated: 13 Jan 2010

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Neonatal onset multisystem inflammatory disease" returned 0 free, full-text review articles on human participants.

 
 
 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Efficacy, Safety and Tolerability of ACZ885 in Pediatric Patients With the Following Cryopyrin-associated Periodic Syndromes: Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome, or Neonatal Onset Multisystem Inflammatory Disease
 

Status: Recruiting

Condition Summary: Cryopyrin-associated Periodic Syndromes; Familial Cold Autoinflammatory Syndrome; Muckle-Wells Syndrome; Neonatal Onset Multisystem Inflammatory Disease

 

Last Updated: 25 Aug 2014

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Clinical Outcomes and Safety: A Registry Study of Ilaris (Canakinumab) Patients
 

Status: Recruiting

Condition Summary: Cryopyrin-associated Periodic Syndromes (CAPS); Familial Cold Autoinflam Syn (FCAS); Muckle-wells Syn (MWS); Neonatal Onset Multisystem Inflam Disease (NOMID)

 

Last Updated: 6 Mar 2014

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Study of Autoinflammatory Diseases (NOMID/CAPS, DIRA, CANDLE, SAVI, CRMO, Still's Disease, Behcet's Disease, and Other Undifferentiated Autoinflammatory Diseases)
 

Status: Recruiting

Condition Summary: Urticaria; Arthropathy; Lymphadenopathy; Nervous System Anomalies

 

Last Updated: 28 Oct 2014

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