Sequence-Based Screening of Patients With Idiopathic Polyarteritis Nodosa, Granulomatosis With Polyangiitis, and Microscopic Polyangiitis for Deleterious Genetic Variants in ADA2.
Oskar SchnappaufNatalia Sampaio MouraIvona AksentijevichMonique StoffelsAmanda K OmbrelloPatrycja HoffmannKaryl BarronElaine F RemmersMichael HershfieldSusan J Kellynull nullDavid CuthbertsonSimon CaretteSharon A ChungLindsy ForbessNader A KhalidiCurry L KoeningCarol A LangfordCarol A McAlearPaul A MonachLarry W MorelandChristian PagnouxPhilip SeoJason M SpringerAntoine G SreihKenneth J WarringtonSteven R YtterbergDaniel L KastnerPeter C GraysonPeter A Merkelnull nullPublished in: Arthritis & rheumatology (Hoboken, N.J.) (2021)
A subset of patients with idiopathic PAN meet genetic criteria for DADA2. Given that tumor necrosis factor inhibition is efficacious in DADA2 but is not conventional therapy for PAN, these findings suggest that ADA-2 testing should strongly be considered in patients with hepatitis B virus-negative idiopathic PAN.