The Future of Bullous Pemphigoid (BP): New and Promising Drugs May Revolutionize Treatment Course for BP Patients

Ben-Shoshan, Danielle; Litvinov, Ivan V.; Netchiporouk, Elena

doi:10.1177/1203475419888867

Cited by 4 publications

(8 citation statements)

References 9 publications

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“…Further, 7% of patients in our review discontinued the treatment, usually for economic reasons and were considered nonetheless as treatment failures. 1,4,5…”

Section: Discussionmentioning

confidence: 99%

“…Bullous pemphigoid (BP) is an autoimmune blistering disease affecting elderly patients. [1][2][3] Recent studies demonstrated that a subset of BP patients may have a Type 2 predominant disease with Immunoglobin E (IgE) antibodies targeting BP180/or BP 230 and involving mast cells and eosinophils 1,4,5 . The presence of Type 2 immunity can be suspected on the basis on high serum IgE levels (detected in over 75% of untreated BP patients) and/or serum eosinophilia.…”

Section: Introductionmentioning

confidence: 99%

“…4,8 Systemic and/or topical corticosteroids remain the first line therapy for BP, they are often used together with steroid sparing options including antimetabolites (e.g., methotrexate, mycophenolate mofetil) or tetracyclines to allow more rapid steroid tapering. 1,5 Because BP affects vulnerable elderly population, there is a high interest to step away from systemic immunosuppressive agents to favor safer drugs such as biologic agents targeting Type 2 response (e.g., omalizumab and dupilumab) or rituximab. 4,9,10 Given the lack of randomized controlled data, these agents remain offlabel and difficult to obtain for clinical use.…”

Section: Introductionmentioning

confidence: 99%

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Omalizumab for the Treatment of Bullous Pemphigoid: A Systematic Review of Efficacy and Safety

et al. 2022

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Bullous pemphigoid (BP) is an autoimmune blistering skin disease. Current treatment strategies are limited by their efficacy and/or side effect profile and the need for safer and effective alternatives is undeniable. We aimed to conduct a systematic review focusing on the efficacy and safety of omalizumab in BP patients. Embase, PubMed, Cochrane, and clinicaltrials.gov were searched for English and French articles published from inception to July 1, 2021, using search terms “omalizumab” OR “Xolair” OR “IGE025” OR “olizumab” AND “bullous pemphigoid.” Screening and data extraction was performed by two raters independently. The primary outcome was complete response (CR), and secondary outcomes were partial response (PR), flare-ups, adverse events/vital status. In total, 22 articles were included, with a total of 56 patients. All patients had a refractory BP with mean disease duration of 13.5 ± 20.2 months (Standard Deviation (SD)) and failed 3.1 ± 1.6 therapies and many remained corticosteroids dependent. Overall, 87.5% of patients responded to treatment (55.4% CR and 32.1% PR), 7.1% discontinued the protocol and only 5.4% were non responders. A third of patients were able to discontinue all other therapies and most others were able to discontinue or taper systemic corticosteroids to <10 mg daily. Flare-ups occurred in 57.7% of patients upon discontinuation of omalizumab and/or steroid tapering, most patients recaptured response thereafter. Omalizumab was well tolerated by most patients. Omalizumab appears to be a promising treatment for BP with a good response rate and safety profile. However, several limitations were identified in current literature, and highlight the need for randomized controlled trials of omalizumab in BP.

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“…Further, 7% of patients in our review discontinued the treatment, usually for economic reasons and were considered nonetheless as treatment failures. 1,4,5…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Omalizumab for the Treatment of Bullous Pemphigoid: A Systematic Review of Efficacy and Safety

et al. 2022

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“…BP is characterized by urticarial plaques, tense blisters, and intractable pruritus due to pathogenic autoantibodies directed against two structural components of hemidesmosomes, BP180 (BPAG2) and/or BP230 (BPAG1) leading to complement activation, immune cells recruitment and consequent disruption of basement membrane integrity. 80 -83 Classically, these autoantibodies are of IgG (usually IgG4) type, and their presence can be demonstrated by the pathognomonic linear IgG/C3 staining along the basement membrane on direct immunofluorescence as well as by presence in sera of BP patients. 84…”

Section: Introductionmentioning

confidence: 99%

“…101 Given the positive preliminary outcomes of this study, the FDA designated bertilimumab as an orphan drug allowing its use in BP. 83,99 Future studies are needed to clarify whether all or only a specific subset of BP patients (eg, patients with high serum IgE and/or eosinophilia) may benefit from therapeutic Type 2 inhibition.…”

Section: Introductionmentioning

confidence: 99%

Prominent Role of Type 2 Immunity in Skin Diseases: Beyond Atopic Dermatitis

et al. 2021

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Type 2 immunity, illustrated by T helper 2 lymphocytes (Th2) and downstream cytokines (IL-4, IL-13, IL-31) as well as group 2 innate lymphoid cells (ILC2), is important in host defense and wound healing. 1 The hallmark of type 2 inflammation is eosinophilia and/or high IgE counts and is best recognized in atopic diathesis. Persistent eosinophilia, such as seen in hypereosinophilic syndromes, leads to fibrosis and hence therapeutic Type 2 inhibition in fibrotic diseases is of high interest. Furthermore, as demonstrated in cutaneous T cell lymphoma, advanced disease is characterized by Th1 to Th2 switch allowing cancer progression and immunosuppression. Development of targeted monoclonal antibodies against IL-4Rα (eg, dupilumab) led to a paradigm shift for the treatment of atopic dermatitis (AD) and stimulated research to better understand the role of Type 2 inflammation in other skin conditions. In this review, we summarize up to date knowledge on the role of Type 2 inflammation in skin diseases other than AD and highlight whether the use of Type 2 targeted therapies has been documented or is being investigated in clinical trials. This manuscript reviews the role of Type 2 inflammation in dermatitis, neurodermatitis, IgE-mediated dermatoses (eg, bullous pemphigoid, chronic spontaneous urticaria), sclerodermoid conditions and skin neoplasms.

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Bullous Pemphigoid

Stavropoulos,

Larios

2023

European Handbook of Dermatological Treatments

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The Future of Bullous Pemphigoid (BP): New and Promising Drugs May Revolutionize Treatment Course for BP Patients

Cited by 4 publications

References 9 publications

Omalizumab for the Treatment of Bullous Pemphigoid: A Systematic Review of Efficacy and Safety

Omalizumab for the Treatment of Bullous Pemphigoid: A Systematic Review of Efficacy and Safety

Prominent Role of Type 2 Immunity in Skin Diseases: Beyond Atopic Dermatitis

Bullous Pemphigoid

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