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*

*Corresponding author for this work

Research output: Contribution to journalJournal articleResearchpeer-review

Abstract

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
JournalAllergy: European Journal of Allergy and Clinical Immunology
Volume74
Issue number12
Pages (from-to)2427-2436
ISSN0105-4538
DOIs
Publication statusPublished - Dec 2019

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Urticaria
Histamine Release
Serum
Cell Degranulation
Routine Diagnostic Tests
Mast Cells
Multicenter Studies

Keywords

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

Cite this

Schoepke, Nicole ; Asero, Riccardo ; Ellrich, André ; Ferrer, Marta ; Gimenez-Arnau, Ana ; E. H. Grattan, Clive ; Jakob, Thilo ; Konstantinou, George N. ; Raap, Ulrike ; Skov, Per Stahl ; Staubach, Petra ; Kromminga, Arno ; Zhang, Ke ; Bindslev-Jensen, Carsten ; Daschner, Alvaro ; Kinaciyan, Tamar ; Knol, Edward F. ; Makris, Michael ; Marrouche, Nadine ; Schmid-Grendelmeier, Peter ; Sussman, Gordon ; Toubi, Elias ; Church, Martin K. ; Maurer, Marcus. / Biomarkers and clinical characteristics of autoimmune chronic spontaneous urticaria : Results of the PURIST Study. In: Allergy: European Journal of Allergy and Clinical Immunology. 2019 ; Vol. 74, No. 12. pp. 2427-2436.
@article{c27128aa9a8e4d59b5d3f548e9c047e4,
title = "Biomarkers and clinical characteristics of autoimmune chronic spontaneous urticaria: Results of the PURIST Study",
abstract = "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.",
keywords = "autoimmune CSU, autologous serum skin test, basophil activation assays, chronic spontaneous urticaria, IgG autoantibodies",
author = "Nicole Schoepke and Riccardo Asero and Andr{\'e} Ellrich and Marta Ferrer and Ana Gimenez-Arnau and {E. H. Grattan}, Clive and Thilo Jakob and Konstantinou, {George N.} and Ulrike Raap and Skov, {Per Stahl} and Petra Staubach and Arno Kromminga and Ke Zhang and Carsten Bindslev-Jensen and Alvaro Daschner and Tamar Kinaciyan and Knol, {Edward F.} and Michael Makris and Nadine Marrouche and Peter Schmid-Grendelmeier and Gordon Sussman and Elias Toubi and Church, {Martin K.} and Marcus Maurer",
year = "2019",
month = "12",
doi = "10.1111/all.13949",
language = "English",
volume = "74",
pages = "2427--2436",
journal = "Allergy: European Journal of Allergy and Clinical Immunology",
issn = "0105-4538",
publisher = "Wiley Online",
number = "12",

}

Schoepke, N, Asero, R, Ellrich, A, Ferrer, M, Gimenez-Arnau, A, E. H. Grattan, C, Jakob, T, Konstantinou, GN, Raap, U, Skov, PS, Staubach, P, Kromminga, A, Zhang, K, Bindslev-Jensen, C, Daschner, A, Kinaciyan, T, Knol, EF, Makris, M, Marrouche, N, Schmid-Grendelmeier, P, Sussman, G, Toubi, E, Church, MK & Maurer, M 2019, 'Biomarkers and clinical characteristics of autoimmune chronic spontaneous urticaria: Results of the PURIST Study', Allergy: European Journal of Allergy and Clinical Immunology, vol. 74, no. 12, pp. 2427-2436. https://doi.org/10.1111/all.13949

Biomarkers and clinical characteristics of autoimmune chronic spontaneous urticaria : Results of the PURIST Study. / Schoepke, Nicole; Asero, Riccardo; Ellrich, André; Ferrer, Marta; Gimenez-Arnau, Ana; E. H. Grattan, Clive; Jakob, Thilo; Konstantinou, George N.; Raap, Ulrike; Skov, Per Stahl; Staubach, Petra; Kromminga, Arno; Zhang, Ke; Bindslev-Jensen, Carsten; Daschner, Alvaro; Kinaciyan, Tamar; Knol, Edward F.; Makris, Michael; Marrouche, Nadine; Schmid-Grendelmeier, Peter; Sussman, Gordon; Toubi, Elias; Church, Martin K.; Maurer, Marcus.

In: Allergy: European Journal of Allergy and Clinical Immunology, Vol. 74, No. 12, 12.2019, p. 2427-2436.

Research output: Contribution to journalJournal articleResearchpeer-review

TY - JOUR

T1 - Biomarkers and clinical characteristics of autoimmune chronic spontaneous urticaria

T2 - Results of the PURIST Study

AU - Schoepke, Nicole

AU - Asero, Riccardo

AU - Ellrich, André

AU - Ferrer, Marta

AU - Gimenez-Arnau, Ana

AU - E. H. Grattan, Clive

AU - Jakob, Thilo

AU - Konstantinou, George N.

AU - Raap, Ulrike

AU - Skov, Per Stahl

AU - Staubach, Petra

AU - Kromminga, Arno

AU - Zhang, Ke

AU - Bindslev-Jensen, Carsten

AU - Daschner, Alvaro

AU - Kinaciyan, Tamar

AU - Knol, Edward F.

AU - Makris, Michael

AU - Marrouche, Nadine

AU - Schmid-Grendelmeier, Peter

AU - Sussman, Gordon

AU - Toubi, Elias

AU - Church, Martin K.

AU - Maurer, Marcus

PY - 2019/12

Y1 - 2019/12

N2 - 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.

AB - 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.

KW - autoimmune CSU

KW - autologous serum skin test

KW - basophil activation assays

KW - chronic spontaneous urticaria

KW - IgG autoantibodies

U2 - 10.1111/all.13949

DO - 10.1111/all.13949

M3 - Journal article

C2 - 31228881

AN - SCOPUS:85070185417

VL - 74

SP - 2427

EP - 2436

JO - Allergy: European Journal of Allergy and Clinical Immunology

JF - Allergy: European Journal of Allergy and Clinical Immunology

SN - 0105-4538

IS - 12

ER -