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A blistering new era for bullous pemphigoid: A scoping review of current therapies, ongoing clinical trials, and future directions.

ABSTRACT: Bullous pemphigoid (BP) is a severe autoimmune blistering skin disorder that primarily causes disease in the older population and is the most prevalent subepidermal variant of the pemphigoid diseases. It manifests as exquisitely pruritic vesiculobullous eruptions and is associated with significant morbidity and mortality. Studies are reporting an increase in prevalence, and, among the elderly, BP is no longer considered to be as rare as previously thought. The pathogenesis involves autoantibodies directed against proteinaceous components of hemidesmosomes, with consequent autoimmune destruction of the dermal-epidermal junction. In recent times, more complex elements of the underlying inflammatory orchestra have been elucidated and are being used to develop targeted immunotherapies. The primary treatment modalities of BP include the use of topical and systemic corticosteroids, certain non-immunosuppressive agents (tetracyclines, nicotinamide, and sulfone), and immunosuppressants (methotrexate, azathioprine, cyclophosphamide, and Mycophenolate). However, in the long term, most of these agents are associated with substantial toxicities while recurrence rates remain high. Such egregious prospects led to significant efforts being directed towards developing newer targeted therapies which work by attenuating specific newly discovered pillars of the inflammatory pathway, and these efforts have garnered hope in providing safer alternatives. Our review focuses on presenting the various therapeutic options that are currently in trial since December 2019, as well as on summarizing presently established treatment guidelines to provide readers with the latest exciting updates.

SUBMITTER: Khalid SN

PROVIDER: S-EPMC8435812 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundBullous pemphigoid (BP) is the most common autoimmune blistering disease in the West. Oral steroids are the standard treatment.This is an update of the review published in 2005.ObjectivesTo assess treatments for bullous pemphigoid.Search strategyIn August 2010 we updated our searches of the Cochrane Skin Group Specialised Register, the Cochrane Central Register of Controlled Trials (Clinical Trials), MEDLINE, EMBASE, and the Ongoing Trials registers.Selection criteriaRandomised controlled trials of treatments for participants with immunofluorescence-confirmed bullous pemphigoid.Data collection and analysisAt least two authors evaluated the studies for the inclusion criteria, and extracted data independently.Main resultsWe included 10 randomised controlled trials (with a total of 1049 participants) of moderate to high risk of bias. All studies involved different comparisons, none had a placebo group. In 1 trial plasma exchange plus prednisone gave significantly better disease control at 1 month (0.3 mg/kg: RR 18.78, 95% CI 1.20 to 293.70) than prednisone alone (1.0 mg/kg: RR 1.79, 95% CI 1.11 to 2.90), while another trial showed no difference in disease control at 6 months.No differences in disease control were seen for different doses or formulations of prednisolone (one trial each), for azathioprine plus prednisone compared with prednisone alone (one trial), for prednisolone plus azathioprine compared with prednisolone plus plasma exchange (one trial), for prednisolone plus mycophenolate mofetil or plus azathioprine (one trial), for tetracycline plus nicotinamide compared with prednisolone (one trial). Chinese traditional medicine plus prednisone was not effective in one trial.There were no significant differences in healing in a comparison of a standard regimen of topical steroids (clobetasol) with a milder regimen (RR 1.00, 95% 0.97 to 1.03) in one trial. In another trial, clobetasol showed significantly more disease control than oral prednisolone in people with extensive and moderate disease (RR 1.09, 95% CI 1.02 to 1.17), with significantly reduced mortality and adverse events (RR 1.06, 95% CI 1.00 to 1.12).Authors' conclusionsVery potent topical steroids are effective and safe treatments for BP, but their use in extensive disease may be limited by side-effects and practical factors. Milder regimens (using lower doses of steroids) are safe and effective in moderate BP. Starting doses of prednisolone greater than 0.75 mg/kg/day do not give additional benefit, lower doses may be adequate to control disease and reduce the incidence and severity of adverse reactions. The effectiveness of adding plasma exchange, azathioprine or mycophenolate mofetil to corticosteroids, and combination treatment with tetracycline and nicotinamide needs further investigation.

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A blistering new era for bullous pemphigoid: A scoping review of current therapies, ongoing clinical trials, and future directions.

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