Abordagens terapêuticas no pênfigo vulgar: revisão da eficácia e segurança em pacientes adultos
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Publicado
May 15, 2025
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Resumo
Objetivo: Avaliar, com base em evidências atualizadas, a eficácia, segurança e estratégias terapêuticas emergentes no tratamento do pênfigo vulgar em adultos. Métodos: Revisão integrativa conduzida segundo a estratégia PVO, incluindo pacientes adultos com diagnóstico confirmado de pênfigo vulgar. Foram selecionados estudos primários de acesso aberto na base PubMed Central, publicados entre 2020 e 2025, que abordaram intervenções terapêuticas e os desfechos clínicos de interesse. Após a triagem e aplicação dos critérios de elegibilidade, 18 estudos foram incluídos. Resultados: Os estudos analisados envolveram 1.433 pacientes, com predominância de investigações sobre o uso de rituximabe e seus biossimilares, além de abordagens adjuvantes como metformina, L-carnitina e DFPP. As taxas de remissão clínica completa variaram entre 40% e 79%, e os eventos adversos mais comuns foram leves. A introdução precoce de agentes biológicos, estratégias de manutenção e individualização do tratamento demonstraram impacto positivo na redução das recaídas e no controle da doença. Considerações finais: O rituximabe destaca-se como uma alternativa eficaz e segura para o tratamento do pênfigo vulgar moderado a grave, com evidências promissoras sobre terapias adjuvantes. Persistem desafios relacionados à resposta vacinal, flares paradoxais e ausência de biomarcadores prognósticos.
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Como Citar
MesquitaV. N. de S., & PigariV. G. (2025). Abordagens terapêuticas no pênfigo vulgar: revisão da eficácia e segurança em pacientes adultos. Revista Eletrônica Acervo Saúde, 25(5), e20774. https://doi.org/10.25248/reas.e20774.2025
Seção
Revisão Bibliográfica
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Referências
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2. AIDI S, et al. Adverse electrocardiographic effects of rituximab infusion in pemphigus patients. Dermatology and Therapy, 2020; 33(6): 14299.
3. AMONCHAISAKDA N, et al. Hospitalization and mortality in Asian autoimmune bullous dermatosis patients: A 17-year retrospective study. Experimental Dermatology, 2024; 33(5): 15095.
4. ARYANIAN Z, et al. Rituximab exhibits a better safety profile when used as a first line of treatment for pemphigus vulgaris: A retrospective study. International Immunopharmacology, 2021; 96: 107755.
5. BHATTACHARJEE R, et al. Assessment of the effects of rituximab monotherapy on different subsets of circulating T-regulatory cells and clinical disease severity in severe pemphigus vulgaris. Dermatology, 2016; 232(5): 572–577.
6. CHEN S, et al. A novel combined use of dupilumab for treatment of aggressive refractory pemphigus vulgaris complicated with pulmonary tuberculosis: A case report and the RNA-seq analysis. Frontiers in Immunology, 2022; 13: 825796.
7. CURA MJ, et al. Pemphigus vulgaris: A retrospective cohort study of clinical features, treatments, and outcomes. Actas Dermosifiliogr, 2020; 111(5): 398-407.
8. DIDONA D, et al. Pemphigus: Current and future therapeutic strategies. Frontiers in Immunology, 2023; 14: 1188-1209.
9. FELDMAN RJ. Paradoxical worsening of pemphigus vulgaris following rituximab therapy. British Journal of Dermatology, 2015; 173(3): 858–859.
10. FORTUNA G, et al. Immunocytometric analysis of oral pemphigus vulgaris patients after treatment with rituximab as adjuvant. Biomolecules, 2021; 11(11): 1634.
11. GUPTA V, et al. Clinical and immunological predictors of post-rituximab paradoxical pemphigus flare: A prospective cohort study. Indian Journal of Dermatology, Venereology and Leprology, 2025; 91(1): 3-8.
12. KAKUTA R, et al. Results of the guideline-based treatment for pemphigus: a single-centre experience with 84 cases. Journal of the European Academy of Dermatology and Venereology, 2020; 34(6): 1324-1330.
13. KANOKRUNGSEE S, et al. Rituximab therapy for treatment of pemphigus in Southeast Asians. Drug Design, Development and Therapy, 2021; 15: 1677-1690.
14. LIU Y, et al. Double-filtration plasmapheresis combined with immunosuppressive treatment for severe pemphigus: 10 years’ experience of a single center in China. Journal of Clinical Apheresis, 2021; 36(1): 20-27.
15. LOUCHEZ A, et al. Maintenance treatment of pemphigus with rituximab in real life: A single-center study of 50 patients. Annales de Dermatologie et de Vénéréologie, 2024; 151(2): 103264.
16. LU KL, et al. Immunosuppressants in dermatology on vaccine immunogenicity: a prospective cohort study of pemphigus patients in the pandemic. Frontiers in Immunology, 2024; 15: 1506962.
17. MAGLIE R, et al. Dramatic exacerbation of bullous pemphigoid following rituximab and successful treatment with omalizumab. European Journal of Dermatology; 2019, 29(2): 213–215.
18. MIYACHI H, et al. Clinical course and outcomes of pemphigus vulgaris and foliaceus: A retrospective study using a nationwide database in Japan. Journal of Dermatology, 2023; 50(2): 212-221.
19. MUSTIN DE, et al. Comparing clinical outcomes of steroid-sparing therapy with rituximab versus rituximab alone in pemphigus. Journal of Drugs in Dermatology, 2024; 23(3): 97-99.
20. NARAYANAN A, et al. A retrospective case-control study of clinical factors associated with paradoxical exacerbation of pemphigus vulgaris following rituximab infusion. International Journal of Dermatology, 2020; 59(12): 459–460.
21. NILI A, et al. Paradoxical reaction to rituximab in patients with pemphigus: A report of 10 cases. Immunopharmacology and Immunotoxicology, 2020; 42(1): 56–58.
22. NOORMOHAMMADI Z, et al. L-carnitine downregulate the muscle wasting effect of glucocorticoids in pemphigus patient: A randomized double-blind placebo-controlled study. Physiological Reports, 2023; 11(8): 15657.
23. PADHIYAR JK. Paradoxical disease flare, skin infection and hypogammaglobulinaemia in patients with pemphigus vulgaris treated with rituximab. Clinical and Experimental Dermatology, 2024; 49(2): 175–177.
24. PERUMAL JS, et al. Disease exacerbation after rituximab induction in neuromyelitis optica. Neurology® Neuroimmunology & Neuroinflammation, 2015; 2(5): 61.
25. PIRES CAA, et al. Therapeutic outcomes of pemphigus in a referral service in Northern Brazil: a retrospective study of 32 patients. Journal of Dermatological Treatment, 2021; 32(8): 1031-1034.
26. QUINTARELLI L, et al. Clinical patterns, survival, comorbidities, and treatment regimens in 149 patients with pemphigus in Tuscany (Italy): A 12-year hospital-based study. Frontiers in Immunology, 2022; 13: 895490.
27. RAHMATPOUR RG, et al. Effects of metformin on autoimmune immunoglobins and interferon-γ in patients with early diagnosed pemphigus vulgaris: a prospective clinical trial. Clinical and Experimental Dermatology, 2022; 47(1): 110-113.
28. RASHID H, et al. The effectiveness of rituximab in pemphigus and the benefit of additional maintenance infusions: Daily practice data from a retrospective study. Journal of the American Academy of Dermatology, 2020; 83(5): 1503-1505.
29. RAVIV T, et al. Prognostic factors predicting remission following rituximab therapy for pemphigus vulgaris. Acta Dermato-Venereologica, 2025; 105: 27140.
30. RUSSO I, et al. Ultra-low dose rituximab for refractory pemphigus vulgaris: a pilot study. Expert Opinion on Biological Therapy, 2020; 20(6): 673-678.
31. SHIN SH, et al. Comparative study of efficacy and safety: Biosimilar rituximab versus originator rituximab in the treatment of pemphigus. Journal of Dermatology, 2024; 51(8): 1104-1107.
32. SNAST I, et al. Treatment of pemphigus vulgaris and foliaceus with adjuvant rituximab compared to immunosuppression alone: Real-life experience. Dermatology, 2021; 237(2): 179-184.
33. SPAŁEK MM, et al. Dapsone as a current option for the treatment of autoimmune bullous diseases with autoimmunity to non-enzymes: A retrospective study from a single Central European referral center. Medicina (Kaunas), 2024; 60(8): 1324.
34. TOOSI R, et al. Efficacy and safety of biosimilar rituximab in patients with pemphigus vulgaris: a prospective observational study. Journal of Dermatological Treatment, 2021; 32(1): 33-40.
35. WANG X, et al. Focus on pemphigus treatment publications: A bibliometric and visual analysis (1992–2022). Heliyon, 2024; 10(7): 28462.
36. WERTH VP, et al. Rituximab versus mycophenolate mofetil in patients with pemphigus vulgaris. New England Journal of Medicine, 2021; 384(24): 2295-2305.
37. WIEDENMAYER N, et al. A novel high-dose intravenous immunoglobulin preparation for the treatment of severe pemphigus vulgaris failing standard therapy: A case report. Journal of Dermatology, 2024; 51(12): 1665-1668.
38. ZHANG W, et al. Short-term intravenous infusion of cyclophosphamide in the treatment of refractory pemphigus vulgaris: A retrospective study. Dermatology, 2021; 237(2): 185-190.
2. AIDI S, et al. Adverse electrocardiographic effects of rituximab infusion in pemphigus patients. Dermatology and Therapy, 2020; 33(6): 14299.
3. AMONCHAISAKDA N, et al. Hospitalization and mortality in Asian autoimmune bullous dermatosis patients: A 17-year retrospective study. Experimental Dermatology, 2024; 33(5): 15095.
4. ARYANIAN Z, et al. Rituximab exhibits a better safety profile when used as a first line of treatment for pemphigus vulgaris: A retrospective study. International Immunopharmacology, 2021; 96: 107755.
5. BHATTACHARJEE R, et al. Assessment of the effects of rituximab monotherapy on different subsets of circulating T-regulatory cells and clinical disease severity in severe pemphigus vulgaris. Dermatology, 2016; 232(5): 572–577.
6. CHEN S, et al. A novel combined use of dupilumab for treatment of aggressive refractory pemphigus vulgaris complicated with pulmonary tuberculosis: A case report and the RNA-seq analysis. Frontiers in Immunology, 2022; 13: 825796.
7. CURA MJ, et al. Pemphigus vulgaris: A retrospective cohort study of clinical features, treatments, and outcomes. Actas Dermosifiliogr, 2020; 111(5): 398-407.
8. DIDONA D, et al. Pemphigus: Current and future therapeutic strategies. Frontiers in Immunology, 2023; 14: 1188-1209.
9. FELDMAN RJ. Paradoxical worsening of pemphigus vulgaris following rituximab therapy. British Journal of Dermatology, 2015; 173(3): 858–859.
10. FORTUNA G, et al. Immunocytometric analysis of oral pemphigus vulgaris patients after treatment with rituximab as adjuvant. Biomolecules, 2021; 11(11): 1634.
11. GUPTA V, et al. Clinical and immunological predictors of post-rituximab paradoxical pemphigus flare: A prospective cohort study. Indian Journal of Dermatology, Venereology and Leprology, 2025; 91(1): 3-8.
12. KAKUTA R, et al. Results of the guideline-based treatment for pemphigus: a single-centre experience with 84 cases. Journal of the European Academy of Dermatology and Venereology, 2020; 34(6): 1324-1330.
13. KANOKRUNGSEE S, et al. Rituximab therapy for treatment of pemphigus in Southeast Asians. Drug Design, Development and Therapy, 2021; 15: 1677-1690.
14. LIU Y, et al. Double-filtration plasmapheresis combined with immunosuppressive treatment for severe pemphigus: 10 years’ experience of a single center in China. Journal of Clinical Apheresis, 2021; 36(1): 20-27.
15. LOUCHEZ A, et al. Maintenance treatment of pemphigus with rituximab in real life: A single-center study of 50 patients. Annales de Dermatologie et de Vénéréologie, 2024; 151(2): 103264.
16. LU KL, et al. Immunosuppressants in dermatology on vaccine immunogenicity: a prospective cohort study of pemphigus patients in the pandemic. Frontiers in Immunology, 2024; 15: 1506962.
17. MAGLIE R, et al. Dramatic exacerbation of bullous pemphigoid following rituximab and successful treatment with omalizumab. European Journal of Dermatology; 2019, 29(2): 213–215.
18. MIYACHI H, et al. Clinical course and outcomes of pemphigus vulgaris and foliaceus: A retrospective study using a nationwide database in Japan. Journal of Dermatology, 2023; 50(2): 212-221.
19. MUSTIN DE, et al. Comparing clinical outcomes of steroid-sparing therapy with rituximab versus rituximab alone in pemphigus. Journal of Drugs in Dermatology, 2024; 23(3): 97-99.
20. NARAYANAN A, et al. A retrospective case-control study of clinical factors associated with paradoxical exacerbation of pemphigus vulgaris following rituximab infusion. International Journal of Dermatology, 2020; 59(12): 459–460.
21. NILI A, et al. Paradoxical reaction to rituximab in patients with pemphigus: A report of 10 cases. Immunopharmacology and Immunotoxicology, 2020; 42(1): 56–58.
22. NOORMOHAMMADI Z, et al. L-carnitine downregulate the muscle wasting effect of glucocorticoids in pemphigus patient: A randomized double-blind placebo-controlled study. Physiological Reports, 2023; 11(8): 15657.
23. PADHIYAR JK. Paradoxical disease flare, skin infection and hypogammaglobulinaemia in patients with pemphigus vulgaris treated with rituximab. Clinical and Experimental Dermatology, 2024; 49(2): 175–177.
24. PERUMAL JS, et al. Disease exacerbation after rituximab induction in neuromyelitis optica. Neurology® Neuroimmunology & Neuroinflammation, 2015; 2(5): 61.
25. PIRES CAA, et al. Therapeutic outcomes of pemphigus in a referral service in Northern Brazil: a retrospective study of 32 patients. Journal of Dermatological Treatment, 2021; 32(8): 1031-1034.
26. QUINTARELLI L, et al. Clinical patterns, survival, comorbidities, and treatment regimens in 149 patients with pemphigus in Tuscany (Italy): A 12-year hospital-based study. Frontiers in Immunology, 2022; 13: 895490.
27. RAHMATPOUR RG, et al. Effects of metformin on autoimmune immunoglobins and interferon-γ in patients with early diagnosed pemphigus vulgaris: a prospective clinical trial. Clinical and Experimental Dermatology, 2022; 47(1): 110-113.
28. RASHID H, et al. The effectiveness of rituximab in pemphigus and the benefit of additional maintenance infusions: Daily practice data from a retrospective study. Journal of the American Academy of Dermatology, 2020; 83(5): 1503-1505.
29. RAVIV T, et al. Prognostic factors predicting remission following rituximab therapy for pemphigus vulgaris. Acta Dermato-Venereologica, 2025; 105: 27140.
30. RUSSO I, et al. Ultra-low dose rituximab for refractory pemphigus vulgaris: a pilot study. Expert Opinion on Biological Therapy, 2020; 20(6): 673-678.
31. SHIN SH, et al. Comparative study of efficacy and safety: Biosimilar rituximab versus originator rituximab in the treatment of pemphigus. Journal of Dermatology, 2024; 51(8): 1104-1107.
32. SNAST I, et al. Treatment of pemphigus vulgaris and foliaceus with adjuvant rituximab compared to immunosuppression alone: Real-life experience. Dermatology, 2021; 237(2): 179-184.
33. SPAŁEK MM, et al. Dapsone as a current option for the treatment of autoimmune bullous diseases with autoimmunity to non-enzymes: A retrospective study from a single Central European referral center. Medicina (Kaunas), 2024; 60(8): 1324.
34. TOOSI R, et al. Efficacy and safety of biosimilar rituximab in patients with pemphigus vulgaris: a prospective observational study. Journal of Dermatological Treatment, 2021; 32(1): 33-40.
35. WANG X, et al. Focus on pemphigus treatment publications: A bibliometric and visual analysis (1992–2022). Heliyon, 2024; 10(7): 28462.
36. WERTH VP, et al. Rituximab versus mycophenolate mofetil in patients with pemphigus vulgaris. New England Journal of Medicine, 2021; 384(24): 2295-2305.
37. WIEDENMAYER N, et al. A novel high-dose intravenous immunoglobulin preparation for the treatment of severe pemphigus vulgaris failing standard therapy: A case report. Journal of Dermatology, 2024; 51(12): 1665-1668.
38. ZHANG W, et al. Short-term intravenous infusion of cyclophosphamide in the treatment of refractory pemphigus vulgaris: A retrospective study. Dermatology, 2021; 237(2): 185-190.