Laboratory diagnosis of paraneoplastic pemphigus

Poot, A. M.; Diercks, G. F. H.; Kramer, D.; Schepens, Isabelle; Klunder, G.; Hashimoto, T.; Borradori, Luca; Jonkman, M. F.; Pas, H. H. (2013). Laboratory diagnosis of paraneoplastic pemphigus. British journal of dermatology, 169(5), pp. 1016-1024. Wiley-Blackwell 10.1111/bjd.12479

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BACKGROUND Paraneoplastic pemphigus (PNP) is a multiorgan disease characterized by antibodies against plakins, desmogleins and the α2-macroglobulin-like-1 (A2ML1) protein, in association with an underlying neoplasm. Accurate diagnosis relies on the demonstration of these autoantibodies in serum. OBJECTIVES To evaluate the value of different laboratory techniques in the serological diagnosis of PNP. METHODS We performed immunoblotting, envoplakin (EP) enzyme-linked immunosorbent assay (ELISA), indirect immunofluorescence (IIF) on rat bladder, radioactive immunoprecipitation and a nonradioactive combined immunoprecipitation-immunoblot assay. Additional assays included BP180 ELISA and BP230 ELISA. We included the sera of 19 patients with PNP and 40 control subjects. RESULTS The sensitivities were 63% for anti-EP ELISA, 74% for rat bladder IIF, 89% for immunoblotting, 95% for radioactive immunoprecipitation and 100% for nonradioactive immunoprecipitation. Specificities ranged from 86% to 100%. The BP180 and BP230 ELISAs had low sensitivity and specificity for PNP. The combination of rat bladder IIF and immunoblot showed 100% sensitivity and specificity. The analysis of sequential PNP sera showed that antibody titres may decrease over time, possibly resulting in negative outcomes for EP ELISA and rat bladder IIF studies. CONCLUSIONS The detection of autoantibodies against EP and periplakin, or A2ML1 by immunoprecipitation is most sensitive for PNP. The combination of rat bladder IIF and immunoblotting is equally sensitive and highly specific, and represents an alternative valuable and relatively easy approach for the serological diagnosis of PNP.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Dermatology
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > DBMR Forschung Mu35 > Forschungsgruppe Radio-Onkologie
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > DBMR Forschung Mu35 > Forschungsgruppe Radio-Onkologie

UniBE Contributor:

Schepens, Isabelle and Borradori, Luca

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0007-0963

Publisher:

Wiley-Blackwell

Language:

English

Submitter:

Monika Schenk

Date Deposited:

16 Jun 2014 08:02

Last Modified:

19 Oct 2015 11:32

Publisher DOI:

10.1111/bjd.12479

PubMed ID:

23796242

BORIS DOI:

10.7892/boris.45607

URI:

https://boris.unibe.ch/id/eprint/45607

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