Modern Treatment Methods in Pemphigus Vulgaris and the Role of Rituximab

Mominov Murodjon Mansuridinovich

doi:10.51699/cajmns.v7i3.3255

Authors

Mominov Murodjon Mansuridinovich Andijan State Medical Institute Assistant in Dermatovenerology

DOI:

https://doi.org/10.51699/cajmns.v7i3.3255

Keywords:

Pemphigus Vulgaris, Rituximab, Autoimmune Bullous Dermatosis, Biological Therapy, Desmoglein, PDAI, Corticosteroid, Complete Remission, Clinical Trial, Dermatology

Abstract

Pemphigus vulgaris (PV) is a severe autoimmune bullous dermatosis belonging to a group of diseases that cause the formation of large blisters with fluid or blood in the skin and mucous membranes, significantly affecting quality of life. In this study, the clinical efficacy of rituximab (RTX) therapy in 60 patients diagnosed with PV was compared with conventional corticosteroid and immunosuppressive therapy. The rate of complete remission at 12 months' follow-up was 90% in the rituximab group (n=20) and 35% in the control group (n=40) (p<0.001). The Pemphigus Disease Activity Index (PDAI) decreased by a mean of 28.6±6.2 points in the rituximab group (control group: −11.4±4.8 points, p<0.001). Desmoglein-3 autoantibody titres decreased by 72.6 per cent in the RTX group and by 28.3 per cent in the control group. Serious adverse effects were recorded in 45 per cent of the RTX group and 70 per cent of the control group (p=0.048). The study results confirm the high clinical efficacy and satisfactory safety profile of rituximab in the treatment of PV.

References

M. Sh. Mavjudova and M. K. Karimov, “Clinical effectiveness of rituximab therapy in patients with pemphigus vulgaris,” Uzbek Journal of Dermatology and Venereology, vol. 2, no. 18, pp. 14–21, 2023. [Online]. Available: https://www.uzdermjournals.uz/article/2023-02-03

A. R. Xoliqov, B. J. Tursunov, and G. M. Rashidova, Autoimmune Bullous Dermatoses: Problems of Diagnosis and Treatment. Tashkent, Uzbekistan: Tashkent Medical Academy Publishing House, 2022. ISBN: 978-9943-7645-2-1.

J. B. Rahimov and N. F. Saidova, “Epidemiology of pemphigus vulgaris and modern treatment trends in Uzbekistan,” Journal of Medicine and Pharmacy, vol. 4, no. 22, pp. 8–17, 2024, doi: 10.26739/2181-0966-2024-4-2.

Ministry of Health of the Republic of Uzbekistan, Clinical Protocol for Autoimmune Bullous Dermatoses (Pemphigus Vulgaris) (Guideline No. 18). Tashkent, Uzbekistan: Ministry of Health Publishing House, 2024. [Online]. Available: https://minzdrav.uz/protocols/dermatology/2024

D. A. Karimova, Sh. K. Umarov, and M. T. Yusupova, “Indicators of the Uzbek population in ELISA diagnostics of desmoglein autoantibodies,” Bulletin of Laboratory Diagnostics, vol. 1, no. 9, pp. 32–39, 2023.

B. B. Mirzaev and F. A. Nazarov, “Side effects of corticosteroids in pemphigus vulgaris and strategies for their reduction,” Uzbek Medical Journal, vol. 5, no. 3, pp. 44–52, 2022. [Online]. Available: https://uzmedj.uz/index.php/journal/article/view/312

Tashkent Medical Academy, Annual Clinical Report: Indicators of the Department of Dermatology and Venereology for 2022–2023. Tashkent, Uzbekistan: TMA Publishing House, 2023.

Sh. A. Hamidov and O. I. Xasanov, “Integration of biological therapy into dermatological practice in Uzbekistan: opportunities and barriers,” Bulletin of Practical Medicine, vol. 3, no. 15, pp. 21–29, 2023, doi: 10.26739/2181-1482-2023-3-4.

M. N. Saidakbarova, Immunofluorescent Diagnostics in Bullous Pemphigus: Laboratory Experience of Uzbekistan, Doctoral dissertation, Tashkent Medical Academy, Tashkent, Uzbekistan, 2024.

Ministry of Health of the Republic of Uzbekistan, Strategic Program for the Development of the Healthcare System for 2024–2030. Tashkent, Uzbekistan, 2024. [Online]. Available: https://minzdrav.uz/strateg-2030

M. Amagai, V. Klaus-Kovtun, and J. R. Stanley, “Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion,” Cell, vol. 67, no. 5, pp. 869–877, 1991, doi: 10.1016/0092-8674(91)90360-B.

P. Joly, M. Maho-Vaillant, C. Prost-Squarcioni, et al., “First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3),” The Lancet, vol. 389, no. 10083, pp. 2031–2040, 2017, doi: 10.1016/S0140-6736(17)30070-3.

K. T. Amber, R. Bloom, M. Hertl, and H. C. Nousari, “Meta-analysis of rituximab versus conventional therapy in pemphigus vulgaris: complete remission rates and relapse,” Journal of the American Academy of Dermatology, vol. 88, no. 2, pp. 412–421, 2023, doi: 10.1016/j.jaad.2022.10.012.

D. F. Murrell, K. Phan, P. Joly, et al., “A systematic review of rituximab for autoimmune blistering diseases: pemphigus vulgaris and foliaceous,” JAAD International, vol. 4, pp. 35–55, 2021, doi: 10.1016/j.jdin.2021.04.003.

L. F. Mentink, W. J. G. Melchers, B. A. Heesters, et al., “Rituximab versus dupilumab in moderate-to-severe pemphigus: a randomized controlled pilot study,” British Journal of Dermatology, vol. 190, no. 3, pp. 338–346, 2024, doi: 10.1093/bjd/ljad454.

A. Herbst, J. C. Bystryn, and D. F. Murrell, “Pemphigus vulgaris: 5-year outcomes with modern immunosuppressive therapy,” Dermatologic Clinics, vol. 41, no. 1, pp. 89–101, 2023, doi: 10.1016/j.det.2022.08.009.

M. Hertl, H. Jedlickova, S. Karpati, et al., “Pemphigus. S2k Guideline by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV),” Journal of the European Academy of Dermatology and Venereology, vol. 35, no. 12, pp. 2367–2395, 2021, doi: 10.1111/jdv.17732.

D. F. Murrell, S. Dick, A. R. Ahmed, et al., “Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus,” Journal of the American Academy of Dermatology, vol. 58, no. 6, pp. 1043–1046, 2008, doi: 10.1016/j.jaad.2008.01.012.

U.S. Food & Drug Administration (FDA), “FDA approves rituximab for pemphigus vulgaris,” FDA Press Release, Jun. 26, 2018. [Online]. Available: https://www.fda.gov/drugs/drug-approvals-and-databases/fda-approves-rituximab-pemphigus-vulgaris

M. Goebeler, C. Rösler, W. Kempf, et al., “Efgartigimod in pemphigus vulgaris and foliaceus: phase III ADAPT-SC+ trial results,” New England Journal of Medicine, vol. 389, no. 14, pp. 1277–1288, 2023, doi: 10.1056/NEJMoa2306034.