Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV).
Journal
Journal of the European Academy of Dermatology and Venereology : JEADV
ISSN: 1468-3083
Titre abrégé: J Eur Acad Dermatol Venereol
Pays: England
ID NLM: 9216037
Informations de publication
Date de publication:
Sep 2020
Sep 2020
Historique:
received:
20
03
2020
accepted:
29
05
2020
pubmed:
25
8
2020
medline:
15
5
2021
entrez:
25
8
2020
Statut:
ppublish
Résumé
Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, pemphigus was almost always fatal. Due to its rarity, only few randomized controlled therapeutic trials are available. Recently, rituximab has been approved as first-line treatment for moderate and severe pemphigus vulgaris in Europe and the United States. The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology (EADV) has initiated a throughout update of the guideline for the management of patients with pemphigus. The guidelines for the management of pemphigus were updated, and the degree of consent among all task force members was included. The final version of the guideline was consented by the European Dermatology Forum (EDF) and several patient organizations.
Sections du résumé
BACKGROUND
BACKGROUND
Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, pemphigus was almost always fatal. Due to its rarity, only few randomized controlled therapeutic trials are available. Recently, rituximab has been approved as first-line treatment for moderate and severe pemphigus vulgaris in Europe and the United States.
OBJECTIVES
OBJECTIVE
The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology (EADV) has initiated a throughout update of the guideline for the management of patients with pemphigus.
RESULTS
RESULTS
The guidelines for the management of pemphigus were updated, and the degree of consent among all task force members was included. The final version of the guideline was consented by the European Dermatology Forum (EDF) and several patient organizations.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1900-1913Subventions
Organisme : ERN
Organisme : EADV
Commentaires et corrections
Type : CommentIn
Type : CommentIn
Informations de copyright
© 2020 European Academy of Dermatology and Venereology.
Références
Schmidt E, Kasperkiewicz M, Joly P. Pemphigus. Lancet Lond Engl 2019; 394: 882-894.
Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol. 2011; 29: 432-436.
Kneisel A, Hertl M. Autoimmune bullous skin diseases. Part 1: Clinical manifestations. J Dtsch Dermatol Ges J Ger Soc Dermatol JDDG 2011; 9: 844-856; quiz 857.
Kneisel A, Hertl M. Autoimmune bullous skin diseases. Part 2: diagnosis and therapy. J Dtsch Dermatol Ges J Ger Soc Dermatol JDDG 2011; 9: 927-947.
Kasperkiewicz M, Ellebrecht CT, Takahashi H et al. Pemphigus. Nat Rev Dis Primer 2017; 3: 17026.
Amagai M, Klaus-Kovtun V, Stanley JR. Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion. Cell 1991; 67: 869-877.
Jelti L, Cordel N, Gillibert A et al. Incidence and mortality of pemphigus in France. J Invest Dermatol. 2019; 139: 469-473.
Hübner F, Recke A, Zillikens D, Linder R, Schmidt E. Prevalence and age distribution of pemphigus and pemphigoid diseases in germany. J Invest Dermatol. 2016; 136: 2495-2498.
Harman KE, Seed PT, Gratian MJ, Bhogal BS, Challacombe SJ, Black MM. The severity of cutaneous and oral pemphigus is related to desmoglein 1 and 3 antibody levels. Br J Dermatol. 2001; 144: 775-780.
Schmidt E, Dähnrich C, Rosemann A et al. Novel ELISA systems for antibodies to desmoglein 1 and 3: correlation of disease activity with serum autoantibody levels in individual pemphigus patients. Exp Dermatol 2010; 19: 458-463.
Martin LK, Werth V, Villanueva E, Segall J, Murrell DF. Interventions for pemphigus vulgaris and pemphigus foliaceus. Cochrane Database Syst Rev 2009; 1: CD006263.
Martin LK, Werth VP, Villaneuva EV, Murrell DF. A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol 2011; 64: 903-908.
Hertl M, Jedlickova H, Karpati S et al. Pemphigus. S2 Guideline for diagnosis and treatment-guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol JEADV 2015; 29 :405-414.
Tavakolpour S, Mahmoudi H, Balighi K, Abedini R, Daneshpazhooh M. Sixteen-year history of rituximab therapy for 1085 pemphigus vulgaris patients: A systematic review. Int Immunopharmacol 2018; 54: 131-138.
Schmidt E, Goebeler M Zillikens D. Rituximab in severe pemphigus. Ann N Y Acad Sci 2009; 1173: 683-691.
Joly P, Mouquet H, Roujeau J-C et al. A single cycle of rituximab for the treatment of severe pemphigus. N Engl J Med 2007; 357: 545-552.
Ahmed AR, Spigelman Z, Cavacini LA, Posner MR. Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med 2006; 355: 1772-1779.
Joly P, Maho-Vaillant M, Prost-Squarcioni C et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet Lond Engl 2017; 389: 2031-2040.
Sebaratnam DF, Hanna AM, Chee S et al. Development of a quality-of-life instrument for autoimmune bullous disease: the Autoimmune Bullous Disease Quality of Life questionnaire. JAMA Dermatol 2013; 149: 1186-1191.
Tjokrowidjaja A, Daniel BS, Frew JW et al. The development and validation of the treatment of autoimmune bullous disease quality of life questionnaire, a tool to measure the quality of life impacts of treatments used in patients with autoimmune blistering disease. Br J Dermatol 2013; 169: 1000-1006.
Murrell DF, Dick S, Ahmed AR et al. Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus. J Am Acad Dermatol 2008; 58: 1043-1046.
Hébert V, Boulard C, Houivet E et al. Large international validation of ABSIS and PDAI pemphigus severity scores. J Invest Dermatol. 2019; 139: 31-37.
Boulard C, Duvert Lehembre S, Picard-Dahan C et al. Calculation of cut-off values based on the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) and Pemphigus Disease Area Index (PDAI) pemphigus scoring systems for defining moderate, significant and extensive types of pemphigus. Br J Dermatol 2016; 175:142-149.
Basset N, Guillot B, Michel B, Meynadier J Guilhou JJ. Dapsone as initial treatment in superficial pemphigus. Report of nine cases. Arch Dermatol 1987; 123: 783-785.
Almugairen N, Hospital V, Bedane C et al. Assessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium- and high-dose corticosteroids. J Am Acad Dermatol 2013; 69: 583-588.
Werth VP, Fivenson D, Pandya AG et al. Multicenter randomized, double-blind, placebo-controlled, clinical trial of dapsone as a glucocorticoid-sparing agent in maintenance-phase pemphigus vulgaris. Arch Dermatol 2008; 144: 25-32.
Ingen-Housz-Oro S, Valeyrie-Allanore L, Cosnes A et al. First-line treatment of pemphigus vulgaris with a combination of rituximab and high-potency topical corticosteroids. JAMA Dermatol 2015; 151: 200-203.
Cianchini G, Lupi F, Masini C, Corona R, Puddu P, De Pità O. Therapy with rituximab for autoimmune pemphigus: results from a single-center observational study on 42 cases with long-term follow-up. J Am Acad Dermatol 2012; 67: 617-622.
Beissert S, Mimouni D, Kanwar AJ, Solomons N, Kalia V, Anhalt GJ. Treating pemphigus vulgaris with prednisone and mycophenolate mofetil: a multicenter, randomized, placebo-controlled trial. J Invest Dermatol 2010; 130: 2041-2048.
Beissert S, Werfel T, Frieling U et al. A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of pemphigus. Arch Dermatol 2006; 142: 1447-1454.
Hébert V, Vermeulin T, Tanguy L et al. Comparison of real costs in the French healthcare system in newly diagnosed patients with pemphigus for first-line treatment with rituximab vs. standard corticosteroid regimen: data from a national multicentre trial. Br J Dermatol. 2019; 183: 121-127. https://doi.org/10.1111/bjd.18563. Epub ahead of print.
Chen DM, Odueyungbo A, Csinady E et al. Rituximab is an effective treatment in patients with pemphigus vulgaris and demonstrates a steroid-sparing effect. Br J Dermatol. 2019; 182: 1111-1119. https://doi.org/10.1111/bjd.18482. Epub ahead of print.
Jelti L, Prost-Squarcioni C, Ingen-Housz-Oro S et al. Update of the French recommendations for the management of pemphigus. Ann Dermatol Venereol 2019; 146: 279-286.
Mignard C, Maho-Vaillant M, Golinski ML et al. Factors associated with short-term relapse in patients with pemphigus who receive rituximab as first-line therapy: A post hoc analysis of a randomized clinical trial. JAMA Dermatol. 2020; 156: 1-8.
Colliou N, Picard D, Caillot F et al. Long-term remissions of severe pemphigus after rituximab therapy are associated with prolonged failure of desmoglein B cell response. Sci Transl Med 2013; 5: 175ra30.
Sanchez J, Ingen-Housz-Oro S, Chosidow O, Antonicelli F Bernard P. Rituximab as Single Long-term Maintenance Therapy in Patients With Difficult-to-Treat Pemphigus. JAMA Dermatol 2018; 01: 363-365.
Werth VP. Treatment of pemphigus vulgaris with brief, high-dose intravenous glucocorticoids. Arch Dermatol 1996; 132: 1435-1439.
Amagai M, Ikeda S, Shimizu H et al. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol 2009; 60: 595-603.
Behzad M, Möbs C, Kneisel A et al. Combined treatment with immunoadsorption and rituximab leads to fast and prolonged clinical remission in difficult-to-treat pemphigus vulgaris. Br J Dermatol 2012; 166: 844-852.
Kasperkiewicz M, Shimanovich I, Meier M et al. Treatment of severe pemphigus with a combination of immunoadsorption, rituximab, pulsed dexamethasone and azathioprine/mycophenolate mofetil: a pilot study of 23 patients. Br J Dermatol 2012; 166: 154-160.
Meggitt SJ, Anstey AV, Mohd Mustapa MF, Reynolds NJ Wakelin S. British Association of Dermatologists’ guidelines for the safe and effective prescribing of azathioprine 2011. Br J Dermatol 2011; 165: 711-734.
Guslandi M. Steroid ulcers: Any news? World J Gastrointest Pharmacol Ther 2013; 4: 39-40.
Abasq C, Mouquet H, Gilbert D et al. ELISA testing of anti-desmoglein 1 and 3 antibodies in the management of pemphigus. Arch Dermatol. 2009; 145:529-535.