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

Autoři	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
Rok publikování	2023
Druh	Článek v odborném periodiku
Časopis / Zdroj	Journal of the European Academy of Dermatology and Venereology
Fakulta / Pracoviště MU	Lékařská fakulta
Citace
www	https://onlinelibrary.wiley.com/doi/10.1111/jdv.18931
Doi	http://dx.doi.org/10.1111/jdv.18931
Klíčová slova	paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome
Popis	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.