Actualisation des recommandations françaises de traitement du pemphigus

Jelti, L.; Prost-Squarcioni, Catherine; Ingen‐Housz‐Oro, Saskia; Caux, F.; Bernard, Philippe; Bédane, C.; Alexandre, M.; Dereure, O.; Quéreux, G.; Bidre, Emmanuelle Le; Plée, Julie; Picard‐Dahan, C.; Roux–Villet, C. Le; Duvert‐Lehembre, S.; Ma, Rong; Delaporte, E.; Debarbieux, S.; Jullien, D.; D’Incan, M.; Konstantinou, M-P; Jd, Bouaziz; Tancrède‐Bohin, Emmanuelle; Doutre, M-S; Villada, I. Bourgault; Cordel, N.; Sassolas, B.; Viguier, M.; Mellottee, Benoït; Jouen, F.; Hébert, V.; Joly, P.

doi:10.1016/j.annder.2019.01.018

Cited by 14 publications

(5 citation statements)

References 20 publications

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“…This study identified 2 factors associated with short-term relapse after initial treatment with rituximab: the initial PDAI score, which reflects the severity of pemphigus, and the evolution of anti-DSG antibody values 3 months after the initial cycle of rituximab. These findings are important because rituximab has been recently approved as first-line treatment of moderate to severe pemphigus . The set of criteria identified in the present study provided a 50% positive predictive value.…”

Section: Discussionmentioning

confidence: 62%

Factors Associated With Short-term Relapse in Patients With Pemphigus Who Receive Rituximab as First-line Therapy

et al. 2020

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for the French Study Group on Autoimmune Bullous Skin Diseases IMPORTANCE Rituximab and short-term corticosteroid therapy are the criterion standard treatments for patients with newly diagnosed moderate to severe pemphigus. OBJECTIVE To examine factors associated with short-term relapse in patients with pemphigus treated with rituximab. DESIGN, SETTING, AND PARTICIPANTS This post hoc analysis of a randomized clinical trial (Comparison Between Rituximab Treatment and Oral Corticosteroid Treatment in Patients With Pemphigus [RITUX 3]) conducted from January 1, 2010, to December 31, 2015, included patients from 20 dermatology departments of tertiary care centers in France from the RITUX 3 trial and 3 newly diagnosed patients treated according to the trial protocol. Data analysis was performed from February 1 to June 30, 2019.EXPOSURE Patients randomly assigned to the rituximab group in the RITUX 3 trial and the 3 additional patients were treated with 1000 mg of intravenous rituximab on days 0 and 14 and 500 mg at months 12 and 18 combined with a short-term prednisone regimen.MAIN OUTCOMES AND MEASURES Baseline (pretreatment) clinical and biological characteristics (Pemphigus Disease Area Index [PDAI] score, ranging from 0-250 points, with higher values indicating more severe disease) and changes in anti-desmoglein (DSG) 1 and anti-DSG3 values as measured by enzyme-linked immunosorbent assay during the 3 months after rituximab treatment were compared between patients with disease relapse and those who maintained clinical remission during the first 12 months after treatment. The positive and negative predictive values of these factors were calculated. RESULTS Among 47 patients (mean [SD] age, 54.3 [17.0] years; 17 [36%] male and 30 [64%] female) included in the study, the mean (SD) baseline PDAI score for patients with relapsing disease was higher than that of the patients with nonrelapsing disease (54 [33] vs 28 [24]; P = .03). At month 3, 7 of 11 patients with relapsing disease (64%) vs 7 of 36 patients with nonrelapsing disease (19%) had persistent anti-DSG1 antibody values of 20 IU/mL or higher and/or anti-DSG3 antibody values of 130 IU/mL or higher (P = .01). A PDAI score of 45 or higher defining severe pemphigus and/or persistent anti-DSG1 antibody values of 20 IU/mL or higher and/or anti-DSG3 antibody values of 130 IU/mL or higher at month 3 provided a positive predictive value of 50% (95% CI, 27%-73%) and a negative predictive value of 94% (95% CI, 73%-100%) for the occurrence of relapse after rituximab. CONCLUSIONS AND RELEVANCEThe findings suggest that initial PDAI score and changes in anti-DSG antibody values after the initial cycle of rituximab might help differentiate a subgroup of patients with high risk of relapse who might benefit from maintenance rituximab infusion at month 6 from a subgroup of patients with low risk of relapse who do not need early maintenance therapy. TRIAL REGISTRATION NCT00784589

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Section: Discussionmentioning

confidence: 62%

Factors Associated With Short-term Relapse in Patients With Pemphigus Who Receive Rituximab as First-line Therapy

et al. 2020

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“…1 attempts from countries such as UK, China, Brazil, France and Germany to standardise the management of pemphigus have been made. 7,10,12,[27][28][29] The similarities and differences of these various guidelines was outlined well in a 2021 review by Zhao and colleagues. 30 The largest similarity was the widely accepted use of corticosteroids as standard first-line therapy, with azathioprine and mycophenolate mofetil the most widely recommended steroid-sparing drugs.…”

Section: Global Guidelines and Recommendationsmentioning

confidence: 98%

“…Following the landmark randomised controlled trial conducted by Joly and colleagues 1 . attempts from countries such as UK, China, Brazil, France and Germany to standardise the management of pemphigus have been made 7,10,12,27–29 . The similarities and differences of these various guidelines was outlined well in a 2021 review by Zhao and colleagues 30 .…”

Section: Global Guidelines and Recommendationsmentioning

confidence: 99%

Treatment of pemphigus in Australia: Aligning current practises with global recommendations

2022

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Pemphigus encompasses of a group of rare, and often severe, intraepidermal bullous dermatoses that are mediated by autoantibodies that act against adhesion proteins of the desmosome. The current international consensus is that the use of intravenous CD20 inhibitors should be first-line in the management of moderate-to-severe cases of pemphigus, however Australia is yet to adopt this. Systemic corticosteroids, combined with conventional corticosteroid-sparing immunosuppressive agents such as azathioprine, mycophenolate mofetil, cyclosporin and methotrexate is still recommended as first-line therapy in Australia despite overwhelming evidence that combining rituximab with a rapid corticosteroid tapering regime is more effective in the treatment of moderate-to-severe pemphigus, and leads to fewer severe adverse effects. We propose a therapeutic approach that echoes the international consensus and recommend that rituximab, an anti-CD20 monoclonal antibody, be listed in the formularies of Australian Public Hospitals and on the Pharmaceutical Benefits Scheme for use in moderate to severe pemphigus.

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“…The prevalence of complete remission off oral systemic therapy after a single cycle of rituximab is around 32.4% at 12 months 196 . In patients without complete remission, another cycle of RTX may be recommended (two infusions of 1,000 mg 2 weeks apart); in case of complete remission, a single RTX infusion (500 mg or 1,000 mg) 6–12 month after infusion is suggested to prevent relapses 197,198 . In recalcitrant cases, IVIG (2 g/kg BW/month), 183,199 a monthly administration of methylprednisolone 0.5–1 g/day or dexamethasone 100 mg/day over 3 consecutive days, 183,186 and immunoadsorption (at least 2 cycles 4 weeks apart over 3–4 consecutive days) 200,201 can be tried alone or together with first line medications, including RTX.…”

Section: Therapeutic Approaches In Skin Blistering Diseasesmentioning

confidence: 99%

Pemphigus and pemphigoids: Clinical presentation, diagnosis and therapy

Didona,

Schmidt,

Maglie

et al. 2023

J Deutsche Derma Gesell

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SummaryPemphigus and pemphigoid are two potentially life‐threatening groups of autoimmune diseases, characterized by autoantibodies targeting structural components of desmosomes or hemidesmosomes, respectively. Affected patients typically show itchy/painful plaques or blistering skin lesions and/or impairing mucosal blistering and erosions, which may strongly impact their quality of life. Since the milestone work of Walter Lever in 1953, who differentiated these two groups of diseases by histopathological analysis of the level of antibody‐mediated skin cleavage, enormous progresses occurred. Achievements made in laboratory diagnostics now allow to identify antigen specific structural proteins of the skin that are targeted by pathogenic autoantibodies. These progresses were accompanied by an increased understanding of the pathogenesis of these diseases thanks to the establishment of animal models reproducing disease and on studies on skin and blood of affected individuals, which have been leading to novel and disease‐specific treatments. Yet, given their phenotypical overlap with more common dermatological diseases, correct diagnosis and appropriate treatment are often delayed, in some cases leading to irreversible sequelae, including organ dysfunction (i.e., loss of vision in mucous membrane pemphigoid). Here, we provide a concise overview of the clinical appearance, diagnosis and therapeutic management of pemphigus and pemphigoid diseases.

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Actualisation des recommandations françaises de traitement du pemphigus

Cited by 14 publications

References 20 publications

Factors Associated With Short-term Relapse in Patients With Pemphigus Who Receive Rituximab as First-line Therapy

Factors Associated With Short-term Relapse in Patients With Pemphigus Who Receive Rituximab as First-line Therapy

Treatment of pemphigus in Australia: Aligning current practises with global recommendations

Pemphigus and pemphigoids: Clinical presentation, diagnosis and therapy

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