S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)
Jazyk angličtina Země Anglie, Velká Británie Médium print-electronic
Typ dokumentu směrnice, časopisecké články
Grantová podpora
P30 CA093373
NCI NIH HHS - United States
PubMed
36965110
PubMed Central
PMC10806824
DOI
10.1111/jdv.18931
Knihovny.cz E-zdroje
- MeSH
- autoimunitní nemoci MeSH
- krysa rodu Rattus MeSH
- nádory komplikace MeSH
- paraneoplastické neurologické syndromy * diagnóza etiologie terapie MeSH
- paraneoplastické syndromy * diagnóza etiologie terapie MeSH
- společnosti lékařské MeSH
- zvířata MeSH
- Check Tag
- krysa rodu Rattus MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- směrnice MeSH
BACKGROUND: Paraneoplastic 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. OBJECTIVES: These 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. RESULTS: Chronic 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. CONCLUSIONS: These 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.
Azrieli Faculty of Medicine Bar Ilan University Safed Israel
Clinic of Dermatovenereology University Clinical Center of Serbia Belgrade Serbia
Clinical Immunodermatology St John's Institute of Dermatology Guy's Hospital London UK
Department Dermatology and Immunodermatology Medical University of Warsaw Warsaw Poland
Department of Dermatology and Allergology Philipps University Marburg Germany
Department of Dermatology and Referral Center for Autoimmune Bullous Diseases Bobigny France
Department of Dermatology and Venereology Akdeniz University Faculty of Medicine Antalya Turkey
Department of Dermatology and Venereology Medical Faculty Medical University Sofia Bulgaria
Department of Dermatology and Venerology Aarhus University Hospital Aarhus Denmark
Department of Dermatology Groupe Hospitalier Paris Seine Saint Denis AP HP Bobigny France
Department of Dermatology Hospital Clínic of Barcelona Universitat de Barcelona Barcelona Spain
Department of Dermatology Inselspital Bern University Hospital University of Bern Bern Switzerland
Department of Dermatology Klinik Landstraße Vienna Austria
Department of Dermatology Koç University School of Medicine Istanbul Turkey
Department of Dermatology Osaka Metroplitan University Graduate School of Medicine Osaka Japan
Department of Dermatology Peking University 1st Hospital Beijing China
Department of Dermatology PGIMER Chandigarh India
Department of Dermatology University of California Davis Sacramento California USA
Department of Dermatology University of Lübeck Lübeck Germany
Department of Dermatology University of Münster Münster Germany
Department of Dermatology Venereology and Allergology Innsbruck Medical University Innsbruck Austria
Department of Dermatology Venereology and Allergology University Hospital Würzburg Würzburg Germany
Department of Dermatology Xijing Hospital 4th Military Medical University Xi'an China
Department of Dermatovenereology Faculty of Medicine University of Belgrade Belgrade Serbia
Department of Dermatovenereology Masaryk University St Anna Hospital Brno Czech Republic
Department of Haematology University Hospital of Aarhus Aarhus Denmark
Department of Medicine NUS Yong Loo Lin School of Medicine Singapore Singapore
Department of Pathophysiology and Transplantation Università degli Studi di Milano Milan Italy
Dermatology Unit Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy
Division of Dermatology Department of Medicine National University Hospital Singapore Singapore
Division of Dermatology Tel Aviv Sourasky Medical Center Tel Aviv Israel
Faculty of Medicine and Dentistry Danube Private University Krems Austria
Lübeck Institute of Experimental Dermatology University of Lübeck Lübeck Germany
National Clinical Research Center for Skin and Immune Diseases Beijing China
National Skin Centre Singapore Singapore
Rare Diseases Unit Azienda USL Toscana Centro European Reference Network Skin Member Florence Italy
Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel
Section of Dermatology Department of Health Sciences University of Florence Florence Italy
Unit of Dermatology and Skin Research Laboratory Baruch Padeh Medical Center Poriya Israel
University Hospital Center Zagreb School of Medicine University of Zagreb Zagreb Croatia
Yong Loo Lin School of Medicine Lee Kong Chian School of Medicine Duke NUS Singapore Singapore
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