S2k guidelines on the management of paraneoplastic
pemphigus/paraneoplastic autoimmune multiorgan syndrome
initiated by the European Academy of Dermatology and
Venereology (EADV)

J 2023

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)

ANTIGA, Emiliano, Rikke BECH, Roberto MAGLIE, Giovanni GENOVESE, Luca BORRADORI et. al.

Basic information

Original name

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)

Authors

ANTIGA, Emiliano, Rikke BECH (guarantor), Roberto MAGLIE, Giovanni GENOVESE, Luca BORRADORI, Barbara BOCKLE, Marzia CAPRONI, Frederic CAUX, Nisha Suyien CHANDRAN, Alberto CORRA, Francesco AMORE, Maryam DANESHPAZHOOH, Dipankar DE, Dario DIDONA, Marian DMOCHOWSKI, Kossara DRENOVSKA, Jan EHRCHEN, Claudio FELICIANI, Matthias GOEBELER, Richard GROVES, Claudia GUENTHER, Sanjeev HANDA, Silke C HOFMANN, Barbara HORVATH, Dimitrios IOANNIDIS, Hana JEDLIČKOVÁ (203 Czech Republic, belonging to the institution), Cezary KOWALEWSKI, Khalaf KRIDIN, Pascal JOLY, Yen Loo LIM, Branka MARINOVIC, Emanual MAVERAKIS, Joost MEIJER, Aikaterini PATSATSI, Carlo PINCELLI, Catherine PROST, Jane SETTERFIELD, Eli SPRECHER, Dusan SKILJEVIC, Kaisa TASANEN, Soner UZUN, Van Beek NINA, Snejina VASSILEVA, Artem VOROBYEV, Igor VUJIC, Gang WANG, Mingyue WANG, Katarzyna WOZNIAK, Savas YAYLI, Giovanna ZAMBRUNO, Takashi HASHIMOTO, Enno SCHMIDT, Mascaro Jr Jose MANUEL and Angelo Valerio MARZANO

Edition

Journal of the European Academy of Dermatology and Venereology, HOBOKEN, WILEY, 2023, 0926-9959

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30216 Dermatology and venereal diseases

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 9.200 in 2022

RIV identification code

RIV/00216224:14110/23:00131034

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1111/jdv.18931

UT WoS

000958925300001

Keywords in English

paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome

Abstract

V originále

BackgroundParaneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. ObjectivesThese guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. ResultsChronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. ConclusionsThese are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies.

Citovat

ANTIGA, Emiliano, Rikke BECH, Roberto MAGLIE, Giovanni GENOVESE, Luca BORRADORI, Barbara BOCKLE, Marzia CAPRONI, Frederic CAUX, Nisha Suyien CHANDRAN, Alberto CORRA, Francesco AMORE, Maryam DANESHPAZHOOH, Dipankar DE, Dario DIDONA, Marian DMOCHOWSKI, Kossara DRENOVSKA, Jan EHRCHEN, Claudio FELICIANI, Matthias GOEBELER, Richard GROVES, Claudia GUENTHER, Sanjeev HANDA, Silke C HOFMANN, Barbara HORVATH, Dimitrios IOANNIDIS, Hana JEDLIČKOVÁ, Cezary KOWALEWSKI, Khalaf KRIDIN, Pascal JOLY, Yen Loo LIM, Branka MARINOVIC, Emanual MAVERAKIS, Joost MEIJER, Aikaterini PATSATSI, Carlo PINCELLI, Catherine PROST, Jane SETTERFIELD, Eli SPRECHER, Dusan SKILJEVIC, Kaisa TASANEN, Soner UZUN, Van Beek NINA, Snejina VASSILEVA, Artem VOROBYEV, Igor VUJIC, Gang WANG, Mingyue WANG, Katarzyna WOZNIAK, Savas YAYLI, Giovanna ZAMBRUNO, Takashi HASHIMOTO, Enno SCHMIDT, Mascaro Jr Jose MANUEL and Angelo Valerio MARZANO. S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV). Journal of the European Academy of Dermatology and Venereology. HOBOKEN: WILEY, 2023, vol. 37, No 6, p. 1118-1134. ISSN 0926-9959. Available from: https://dx.doi.org/10.1111/jdv.18931.

@article{2293063,
   author = {Antiga, Emiliano and Bech, Rikke and Maglie, Roberto and Genovese, Giovanni and Borradori, Luca and Bockle, Barbara and Caproni, Marzia and Caux, Frederic and Chandran, Nisha Suyien and Corra, Alberto and Amore, Francesco and Daneshpazhooh, Maryam and De, Dipankar and Didona, Dario and Dmochowski, Marian and Drenovska, Kossara and Ehrchen, Jan and Feliciani, Claudio and Goebeler, Matthias and Groves, Richard and Guenther, Claudia and Handa, Sanjeev and Hofmann, Silke C and Horvath, Barbara and Ioannidis, Dimitrios and Jedličková, Hana and Kowalewski, Cezary and Kridin, Khalaf and Joly, Pascal and Lim, Yen Loo and Marinovic, Branka and Maverakis, Emanual and Meijer, Joost and Patsatsi, Aikaterini and Pincelli, Carlo and Prost, Catherine and Setterfield, Jane and Sprecher, Eli and Skiljevic, Dusan and Tasanen, Kaisa and Uzun, Soner and Nina, Van Beek and Vassileva, Snejina and Vorobyev, Artem and Vujic, Igor and Wang, Gang and Wang, Mingyue and Wozniak, Katarzyna and Yayli, Savas and Zambruno, Giovanna and Hashimoto, Takashi and Schmidt, Enno and Manuel, Mascaro Jr Jose and Marzano, Angelo Valerio},
   article_location = {HOBOKEN},
   article_number = {6},
   doi = {http://dx.doi.org/10.1111/jdv.18931},
   keywords = {paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome},
   language = {eng},
   issn = {0926-9959},
   journal = {Journal of the European Academy of Dermatology and Venereology},
   title = {S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)},
   url = {https://onlinelibrary.wiley.com/doi/10.1111/jdv.18931},
   volume = {37},
   year = {2023}
}

TY  - JOUR
ID  - 2293063
AU  - Antiga, Emiliano - Bech, Rikke - Maglie, Roberto - Genovese, Giovanni - Borradori, Luca - Bockle, Barbara - Caproni, Marzia - Caux, Frederic - Chandran, Nisha Suyien - Corra, Alberto - Amore, Francesco - Daneshpazhooh, Maryam - De, Dipankar - Didona, Dario - Dmochowski, Marian - Drenovska, Kossara - Ehrchen, Jan - Feliciani, Claudio - Goebeler, Matthias - Groves, Richard - Guenther, Claudia - Handa, Sanjeev - Hofmann, Silke C - Horvath, Barbara - Ioannidis, Dimitrios - Jedličková, Hana - Kowalewski, Cezary - Kridin, Khalaf - Joly, Pascal - Lim, Yen Loo - Marinovic, Branka - Maverakis, Emanual - Meijer, Joost - Patsatsi, Aikaterini - Pincelli, Carlo - Prost, Catherine - Setterfield, Jane - Sprecher, Eli - Skiljevic, Dusan - Tasanen, Kaisa - Uzun, Soner - Nina, Van Beek - Vassileva, Snejina - Vorobyev, Artem - Vujic, Igor - Wang, Gang - Wang, Mingyue - Wozniak, Katarzyna - Yayli, Savas - Zambruno, Giovanna - Hashimoto, Takashi - Schmidt, Enno - Manuel, Mascaro Jr Jose - Marzano, Angelo Valerio
PY  - 2023
TI  - S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)
JF  - Journal of the European Academy of Dermatology and Venereology
VL  - 37
IS  - 6
SP  - 1118-1134
EP  - 1118-1134
PB  - WILEY
SN  - 09269959
KW  - paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome
UR  - https://onlinelibrary.wiley.com/doi/10.1111/jdv.18931
N2  - BackgroundParaneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. ObjectivesThese guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. ResultsChronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. ConclusionsThese are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies.
ER  -

ANTIGA, Emiliano, Rikke BECH, Roberto MAGLIE, Giovanni GENOVESE, Luca BORRADORI, Barbara BOCKLE, Marzia CAPRONI, Frederic CAUX, Nisha Suyien CHANDRAN, Alberto CORRA, Francesco AMORE, Maryam DANESHPAZHOOH, Dipankar DE, Dario DIDONA, Marian DMOCHOWSKI, Kossara DRENOVSKA, Jan EHRCHEN, Claudio FELICIANI, Matthias GOEBELER, Richard GROVES, Claudia GUENTHER, Sanjeev HANDA, Silke C HOFMANN, Barbara HORVATH, Dimitrios IOANNIDIS, Hana JEDLIČKOVÁ, Cezary KOWALEWSKI, Khalaf KRIDIN, Pascal JOLY, Yen Loo LIM, Branka MARINOVIC, Emanual MAVERAKIS, Joost MEIJER, Aikaterini PATSATSI, Carlo PINCELLI, Catherine PROST, Jane SETTERFIELD, Eli SPRECHER, Dusan SKILJEVIC, Kaisa TASANEN, Soner UZUN, Van Beek NINA, Snejina VASSILEVA, Artem VOROBYEV, Igor VUJIC, Gang WANG, Mingyue WANG, Katarzyna WOZNIAK, Savas YAYLI, Giovanna ZAMBRUNO, Takashi HASHIMOTO, Enno SCHMIDT, Mascaro Jr Jose MANUEL and Angelo Valerio MARZANO. S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV). \textit{Journal of the European Academy of Dermatology and Venereology}. HOBOKEN: WILEY, 2023, vol.~37, No~6, p.~1118-1134. ISSN~0926-9959. Available from: https://dx.doi.org/10.1111/jdv.18931.

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