Biomarkers and clinical characteristics of autoimmune chronic spontaneous urticaria: Results of the PURIST Study

Nicole Schoepke, Riccardo Asero, André Ellrich, Marta Ferrer, Ana Gimenez-Arnau, Clive E H Grattan, Thilo Jakob, George N Konstantinou, Ulrike Raap, Per Stahl Skov, Petra Staubach, Arno Kromminga, Ke Zhang, Carsten Bindslev-Jensen, Alvaro Daschner, Tamar Kinaciyan, Edward F Knol, Michael Makris, Nadine Marrouche, Peter Schmid-GrendelmeierGordon Sussman, Elias Toubi, Martin K Church, Marcus Maurer

Research output: Contribution to journalArticleAcademicpeer-review


Background: Autoimmune chronic spontaneous urticaria (aiCSU) is an important subtype of chronic spontaneous urticaria (CSU) in which functional IgG autoantibodies to IgE or its high-affinity receptor (FcεRI) induces mast cell degranulation and subsequent symptom development. However, it has not been tightly characterized. This study aimed to better define the clinical and immunological features and to explore potential biomarkers of aiCSU. Methods: This was a multinational, multicenter study of 182 CSU patients. The clinical features studied included: urticaria activity and impact (UAS7 and quality of life); autologous serum skin test (ASST); IgG anti-FcεRI and IgG anti-IgE; IgG-anti-thyroperoxidase (IgG anti-TPO); total serum IgE; and basophil reactivity (BASO) using the basophil activation test (BAT) and basophil histamine release assay (BHRA). Results: Of the 182 patients, 107 (59%) were ASST+, 46 (25%) were BASO+, and 105 (58%) were IgG anti-FcεRI+/IgE+. Fifteen patients (8%) fulfilled all three criteria of aiCSU. aiCSU patients appeared more severe (UAS7 21 vs 9 P < 0.016) but showed no other clinical or demographic differences from non-aiCSU patients. aiCSU patients also had markedly lower total IgE levels (P < 0.0001) and higher IgG anti-TPO levels (P < 0.001). Of biomarkers, positive BAT and BHRA tests were 69% and 88% predictive of aiCSU, respectively. Conclusions: aiCSU is a relatively small but immunologically distinct subtype of CSU that cannot be identified by routine clinical parameters. Inclusion of BHRA or BAT in the diagnostic workup of CSU patients may aid identification of aiCSU patients, who may have a different prognosis and benefit from specific management.

Original languageEnglish
Pages (from-to)2427-2436
Number of pages10
Issue number12
Early online date22 Jun 2019
Publication statusPublished - Dec 2019


  • IgG autoantibodies
  • autoimmune CSU
  • autologous serum skin test
  • basophil activation assays
  • chronic spontaneous urticaria


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