Published in:
Open Access
01-11-2013 | Meeting abstract
PW02-038 - Treatment-resistant NOMID with autoantibodies
Authors:
L Broderick, J Chang, I Szer, HM Hoffman
Published in:
Pediatric Rheumatology
|
Special Issue 1/2013
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Excerpt
Neonatal Onset Multisystem Inflammatory Disorder (NOMID) is the most severe of the Cryopyrin-Associated Periodic Syndromes (CAPS) spectrum [
1]. Given the persistent nature of CAPS, symptoms may be confused with autoimmune diseases. However, unlike autoimmune diseases, autoinflammatory disorders are not associated with high-titer autoantibodies or antigen-specific T cells. Here we describe a patient with severe and early onset features of NOMID confirmed by NLRP3 mutation, hepatitis, neonatal antibodies and a limited response to high dose anakinra. …