Serological diagnostics in the detection of IgG autoantibodies against human collagen VII in epidermolysis bullosa acquisita: a multicentre analysis
| Autor: | S.S. Lofti Jad, G. Di Zenzo, Takashi Hashimoto, Minori Kaneda, Miklós Sárdy, Farzan Solimani, Norito Ishii, M. Hoch, Cassian Sitaru, R. Eming, Hana Jedličková, Brandon Greene, Thomas Schmidt, Regine Gläser, Johannes S. Kern, E. Cozzani, Angelo V. Marzano, I. Lakos Jukic, Soner Uzun, G. Göpel, Maria-Elisabeth Goebeler, Michael Hertl |
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| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Epidermolysis bullosa acquisita Pemphigoid Pathology medicine.medical_specialty Dermatology Immunoglobulin G Serology 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine parasitic diseases Medicine biology business.industry Pemphigus vulgaris Autoantibody IIf medicine.disease 3. Good health 030104 developmental biology Immunology biology.protein Bullous pemphigoid business |
| Zdroj: | British Journal of Dermatology. 177:1683-1692 |
| ISSN: | 0007-0963 |
| Popis: | SummaryBackground Epidermolysis bullosa acquisita is a rare, potentially devastating autoimmune disease of the skin. IgG autoantibodies directed against type VII collagen (Col7), the major component of anchoring fibrils, induce skin fragility leading to cutaneous and mucocutaneous blister formation which is mostly of a scaring phenotype. Thus, powerful and reproducible diagnostic assays are critical to establish the diagnosis of EBA early to avoid irreversible sequelae. Objectives The present international, retrospective multicenter study includes a large cohort of EBA patients and evaluated the diagnostic power of four different diagnostic assays for the detection of anti-Col7 IgG autoantibodies. Methods 95 EBA sera and 200 control sera consisting of 100 bullous pemphigoid sera, 50 pemphigus vulgaris sera and 50 sera of healthy controls were tested for anti-Col7 IgG autoantibodies by indirect immunofluorescence (IIF), two commercial ELISA systems and Western blot (WB) analysis. EBA sera were from patients with positive DIF and IgG reactivity in at least one of the immune serological assays (IIF, ELISA, WB). Results A Col7-NC1/NC2 ELISA (MBL, Japan) showed the highest sensitivity (97.9%), followed by a Col7-NC1 ELISA (Euroimmun, Germany; 89.5%), WB with Col7-NC1 (85.3%), and IIF on saline split human skin (74.7%). The specificities of both ELISA systems were comparable (NC1: 98.7%, NC1/NC2: 99.3%). Furthermore, WB was more sensitive than IIF which was more specific. Conclusions The two commercially available ELISA systems allow for a highly sensitive and specific diagnosis of EBA. The sensitivity of the Col7-NC1/NC2 ELISA is significantly higher compared to the ELISA which is based on the Col7-NC1-domain only. This article is protected by copyright. All rights reserved. |
| Databáze: | OpenAIRE |
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