Targeting therapy in pemphigus: Where are we now and where are we going?

Nuer, Kalibi; Hu, Fengxia; Liang, J.; Kang, Xiaojing

doi:10.1016/j.heliyon.2023.e16679

Cited by 7 publications

(6 citation statements)

References 64 publications

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“…Targeted therapy with efgartigimod, an antagonist of the neonatal Fc receptor, was able to significantly reduce anti-Dsg antibody levels in a phase 2 clinical trial ( 45 ). CAAR-T therapy could also be a promising treatment option in the future, in particular for patients who are refractory to therapy ( 46 ). In the case of pemphigoid diseases, dapsone is often used for systemic treatment.…”

Section: Discussionmentioning

confidence: 99%

Retrospective analysis of autoimmune bullous diseases in Middle Franconia

Sollfrank,

Schönfelder,

Sticherling

2023

Front. Immunol.

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IntroductionAutoimmune bullous diseases (AIBDs) are a group of rare cutaneous disorders affecting cornified skin and mucous membranes. They are characterized by tense or flaccid blistering and erosions due to autoantibodies against desmosomal and hemidesmosomal structural proteins of the skin. This group of disorders can be divided into those of pemphigoid and those of pemphigus diseases. If left untreated, these autoimmune diseases can cause serious or even life-threatening complications such as loss of fluid, superinfections or impaired food intake. Due to modern standardized serological assays, the diagnosis of AIBDs can usually be confirmed in combination with their clinical appearance. Whereas for a long time corticosteroids were the major players in the treatment of these diseases, with the approval of rituximab and other immunosuppressive agents, the therapy has increasingly improved.MethodsIn this study, we aimed to investigate epidemiologic and clinical features as well as diagnostics and therapy of bullous autoimmune diseases in Middle Franconia, a governorate within the German federal state of Bavaria. Patients diagnosed or treated because of a AIBDs between 01.04.2013 and 31.03.2019 at the dermatological department of the university hospital Erlangen were included in this retrospective study (n = 242). Patients were either diagnosed for the first time (n=176) or the diagnosis has been confirmed (n=66) at the department. The respective incidence was calculated among the 176 subjects who had been diagnosed at the center in this period. Data was taken from patient records and analyzed with Microsoft® Excel. The evaluation included the diagnoses of pemphigus vulgaris (PV), pemphigus foliaceus (PF), bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA dermatosis (LAD), epidermolysis bullosa acquisita (EBA), and dermatitis herpetiformis (DH).ResultsThis study shows that the incidence of each AIBDs in Middle Franconia is low and comparable (PV, PF, LAD, EBA) or lower (BP, MMP, DH) than in other studies and regions. BP is the most common newly diagnosed AIBD in Middle Franconia.DiscussionDue to the chronic and sometimes severe course of AIBDs, repeated in-house treatments are often necessary. To date, mainly topically and systemically applied corticosteroids in combination with immunomodulators are used as first-line therapy.

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Section: Discussionmentioning

confidence: 99%

Retrospective analysis of autoimmune bullous diseases in Middle Franconia

Sollfrank,

Schönfelder,

Sticherling

2023

Front. Immunol.

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“…Ianalumab (VAY736), a novel BAFF inhibitor targeting BAFF receptor, has attracted great attention as a promising therapeutic agent for SLE and Sjögren syndrome ( 119 ). Notably, phase 2 clinical trials are in progress to evaluate its clinical impacts on patients with PV ( 120 ).…”

Section: Current Therapeutics For Inflammatory Skin Diseases and Pote...mentioning

confidence: 99%

“…BTK plays a crucial role in B cell receptor (BCR) activation and both BCR and Fc receptor engagement induce its kinase activity ( 102 ). Although usage of BTK inhibitor has predominantly focused on multiple sclerosis ( 102 ), its potential for cutaneous inflammatory diseases has been also investigated ( 120 ). Remarkably, BTK inhibitors like ibrutinib and rillzabrutinib have shown significant improvement in disease activity in PV patients ( 10 , 122 , 123 ).…”

Section: Current Therapeutics For Inflammatory Skin Diseases and Pote...mentioning

confidence: 99%

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From neglect to spotlight: the underappreciated role of B cells in cutaneous inflammatory diseases

Lee,

2024

Front. Immunol.

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The skin, covering our entire body as its largest organ, manifests enormous complexities and a profound interplay of systemic and local responses. In this heterogeneous domain, B cells were considered strangers. Yet, recent studies have highlighted their existence in the skin and their distinct role in modulating cutaneous immunity across various immune contexts. Accumulating evidence is progressively shedding light on the significance of B cells in maintaining skin health and in skin disorders. Herein, we integrate current insights on the systemic and local contributions of B cells in three prevalent inflammatory skin conditions: Pemphigus Vulgaris (PV), Systemic Lupus Erythematosus (SLE), and Atopic Dermatitis (AD), underscoring the previously underappreciated importance of B cells within skin immunity. Moreover, we address the potential adverse effects of current treatments used for skin diseases, emphasizing their unintentional consequences on B cells. These comprehensive approaches may pave the way for innovative therapeutic strategies that effectively address the intricate nature of skin disorders.

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“…The treatment of PV often requires high-dose systemic steroids and adjuvant steroid-sparing drugs, such as azathioprine or mycophenolate mofetil [ 3 ], which are valuable for effectively reducing recurrence rates when systemic steroids are tapered off or withdrawn [ 4 ]. High-dose steroids in pulse therapy or rituximab are used in cases of an inadequate response to standard treatment or when in relapse (defined as the appearance of at least three new lesions within 1 month that do not resolve within 1 week without treatment, or the worsening of pre-existing lesions in a patient with a controlled disease) [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

Factors Associated with Candidiasis in Pemphigus Vulgaris Patients: Results from a Retrospective Study in Two Second-Care Level Hospitals in Mexico

Tirado-Sánchez,

Bonifaz,

Frías De León

2023

TropicalMed

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Background: Infections are a major cause of morbidity and mortality in patients with pemphigus vulgaris (PV). One of the most common infections in these patients is candidiasis. This is probably due to the use of systemic immunosuppressants, including oral and intravenous corticosteroids, mainly in megadoses (pulse therapy), although it is unknown if there are other associated factors, in addition to immunosuppressive treatment. We determine the factors associated with candidiasis in PV patients in two second-care level hospitals in Mexico. Methods: We reviewed 100 cases with PV. Cases were randomly selected from the databases of two second-care level hospitals between January 2010 and December 2019 (10 years). The primary endpoint was the incidence of candidiasis in patients with PV. Results: One hundred patients with PV were enrolled in this retrospective study. Candidiasis was observed in 79 patients (79%). A maximum corticosteroid dose of 55 mg/day during the last year (p = 0.001) and a higher neutrophil/lymphocyte ratio were associated with candidiasis in patients with PV (p = 0.001). Conclusion: Risk factors favoring candidiasis in patients with PV are not only related to the use of corticosteroids, but also to demographic factors, the activity of the disease, and the systemic inflammation associated with autoimmunity.

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Targeting therapy in pemphigus: Where are we now and where are we going?

Cited by 7 publications

References 64 publications

Retrospective analysis of autoimmune bullous diseases in Middle Franconia

Retrospective analysis of autoimmune bullous diseases in Middle Franconia

From neglect to spotlight: the underappreciated role of B cells in cutaneous inflammatory diseases

Factors Associated with Candidiasis in Pemphigus Vulgaris Patients: Results from a Retrospective Study in Two Second-Care Level Hospitals in Mexico

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