Mutant Adenosine Deaminase 2 in a Polyarteritis Nodosa Vasculopathy


Elkan P. N., Pierce S. B., Segel R., Walsh T., Barash J., Padeh S., ...More

NEW ENGLAND JOURNAL OF MEDICINE, vol.370, no.10, pp.921-931, 2014 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 370 Issue: 10
  • Publication Date: 2014
  • Doi Number: 10.1056/nejmoa1307362
  • Journal Name: NEW ENGLAND JOURNAL OF MEDICINE
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.921-931
  • Istanbul University Affiliated: Yes

Abstract

BackgroundPolyarteritis nodosa is a systemic necrotizing vasculitis with a pathogenesis that is poorly understood. We identified six families with multiple cases of systemic and cutaneous polyarteritis nodosa, consistent with autosomal recessive inheritance. In most cases, onset of the disease occurred during childhood.