Treatment of pemphigus vulgaris: part 1 – current therapies

Yanovsky, Rebecca L.; McLeod, Michael P.; Ahmed, A. Razzaque

doi:10.1080/1744666x.2020.1672535

Cited by 19 publications

(46 citation statements)

References 73 publications

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“…As in most autoimmune conditions, steroids have - mostly empirically - been shown to be effective in IgG4-autoantibody mediated diseases. Most if not all treatment regimens at least initially contain corticosteroids ( 152 , 154 – 157 ). Some physicians initially apply high-dose iv methylprednisolone and other start with oral steroids (1mg/kg bodyweight followed by tapering).…”

Section: Treatments Of Igg4 Neurological Diseasesmentioning

confidence: 99%

IgG4 Autoantibodies in Organ-Specific Autoimmunopathies: Reviewing Class Switching, Antibody-Producing Cells, and Specific Immunotherapies

et al. 2022

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Organ-specific autoimmunity is often characterized by autoantibodies targeting proteins expressed in the affected tissue. A subgroup of autoimmunopathies has recently emerged that is characterized by predominant autoantibodies of the IgG4 subclass (IgG4-autoimmune diseases; IgG4-AID). This group includes pemphigus vulgaris, thrombotic thrombocytopenic purpura, subtypes of autoimmune encephalitis, inflammatory neuropathies, myasthenia gravis and membranous nephropathy. Although the associated autoantibodies target specific antigens in different organs and thus cause diverse syndromes and diseases, they share surprising similarities in genetic predisposition, disease mechanisms, clinical course and response to therapies. IgG4-AID appear to be distinct from another group of rare immune diseases associated with IgG4, which are the IgG4-related diseases (IgG4-RLD), such as IgG4-related which have distinct clinical and serological properties and are not characterized by antigen-specific IgG4. Importantly, IgG4-AID differ significantly from diseases associated with IgG1 autoantibodies targeting the same organ. This may be due to the unique functional characteristics of IgG4 autoantibodies (e.g. anti-inflammatory and functionally monovalent) that affect how the antibodies cause disease, and the differential response to immunotherapies of the IgG4 producing B cells/plasmablasts. These clinical and pathophysiological clues give important insight in the immunopathogenesis of IgG4-AID. Understanding IgG4 immunobiology is a key step towards the development of novel, IgG4 specific treatments. In this review we therefore summarize current knowledge on IgG4 regulation, the relevance of class switching in the context of health and disease, describe the cellular mechanisms involved in IgG4 production and provide an overview of treatment responses in IgG4-AID.

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Section: Treatments Of Igg4 Neurological Diseasesmentioning

confidence: 99%

IgG4 Autoantibodies in Organ-Specific Autoimmunopathies: Reviewing Class Switching, Antibody-Producing Cells, and Specific Immunotherapies

et al. 2022

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“…In addition to new drug therapies being developed, other treatment methods are being utilized outside of the US. For example, particularly in Europe, extracorporeal immunoadsorption is performed to remove the autoantibodies from the blood [ 16 - 18 ]. PF patients are suffering and they need more effective treatment methods that do not carry an increased risk of harm.…”

Section: Discussionmentioning

confidence: 99%

The First Reported Case of Metoprolol-Induced Pemphigus Foliaceus in the United States: A Critical Report and Review of Literature

Lagziel

Ramos

Rozycki

et al. 2020

Cureus

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Pemphigus is a rare family of autoimmune disorders characterized by epithelial and mucosal blisters. Pemphigus foliaceus (PF) commonly affects the scalp, face, and trunk. Lesions often arise as superficial blisters and develop into scaly, crusted erosions. Management includes corticosteroids with immunosuppressants. Novel therapies include immunoadsorption and active clinical trials. We present the only reported case of metoprolol-induced PF in the United States (US), with an extremely complicated hospital course. A 66-year-old male patient with a history of hypertension, diabetes, and hyperlipidemia presented to his doctor with a blistering, pruritic rash that started after switching to metoprolol for hypertension treatment. PF is very rare in North America. Given its solely superficial penetration, it creates no direct fatal complication. However, the developing blisters and subsequent wounds are susceptible to a wide array of secondary infections, which can be life-threatening.

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“…Існуючий сильний зв'язок між психологічними розладами у хворих на АП і зниженням показників якості життя (ЯЖ) призводить до зниження прихильності пацієнтів до медикаментозної терапії. Низька ЯЖ і низька комплаєнтність пацієнтів додатково погіршують прогноз аутоімунних бульозних дерматозів [8].…”

Section: запорізький державний медичний університет українаunclassified

Peculiarities of Quality of Life in Patients with Pemphigus Vulgaris according to the Results of the Analysis of the SF-36V2 Questionnaires and Dermatological Index of Quality of Life in the Background of Glucocorticosteroid Therapy

Litvinov¹

2021

Ukr. ž. med. bìol. sportu

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The purpose of the study was to carry out a comparative assessment of the parameters of the quality of life in patients with pemphigus vulgaris according to the results of the analysis of the SF-36V2 questionnaires and the dermatological index of the quality of life against the background of glucocorticosteroid therapy. Materials and methods. The results of the study are based on the data of a comprehensive study and further follow-up of 47 patients with pemphigus vulgaris (of which 30 are the main group and 17 are the comparison group) who were hospitalized at the Zaporizhzhya Regional Skin and Venereal Clinical Dispensary, Zaporizhzhia. At the time of observation, the majority of patients were aged 61-70 years old. As a control group, 20 apparently healthy people were examined, randomized by age and sex. Results and discussion. Pemphigus vulgaris is a severe systemic dermatosis, which, according to the literature, accounts for about 1% in the structure of dermatological pathology, the main pathomorphological sign of which is acantholysis. Although the share of pemphigus vulgaris in the structure of dermatological pathology is 0.6 per 100 thousand people per year, the severity of the disease and its outcome determine the relevance of further study of the pathogenesis of dermatosis and the search for new effective therapies. The etiology of true pemphigus still remains unclear. The obtained results of questioning the patients with the SF-36V2 questionnaires and the dermatological index of quality of life indicate a significant negative impact of pemphigus vulgaris on the key parameters of the quality of life of patients in the main group and in the comparison group before treatment – the ability to perform their daily duties, work or study, have personal relationships, and also on the desire to be treated and adhere to all the rules of the therapy. Patients with pemphigus vulgaris in the group of monotherapy with glucocorticosteroids had statistically worse indicators of the quality of life after treatment than patients with additional use of Armadin, which was characterized by a low level of physical activity, an increase in the role of physical and psychoemotional problems in everyday life, general discomfort and subjective health. Conclusion. Evaluation of the quality of life of patients allowed for a more qualified study of the health disorders of patients with pemphigus vulgaris, to see the essence of the clinical problem, to select the most optimal treatment regimen for each patient, and also to assess the expected results in terms of parameters that are on the verge of a scientific approach and evidence-based medicine on the one hand, and also the subjective point of view of patients with pemphigus vulgaris on the other hand

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Treatment of pemphigus vulgaris: part 1 – current therapies

Cited by 19 publications

References 73 publications

IgG4 Autoantibodies in Organ-Specific Autoimmunopathies: Reviewing Class Switching, Antibody-Producing Cells, and Specific Immunotherapies

IgG4 Autoantibodies in Organ-Specific Autoimmunopathies: Reviewing Class Switching, Antibody-Producing Cells, and Specific Immunotherapies

The First Reported Case of Metoprolol-Induced Pemphigus Foliaceus in the United States: A Critical Report and Review of Literature

Peculiarities of Quality of Life in Patients with Pemphigus Vulgaris according to the Results of the Analysis of the SF-36V2 Questionnaires and Dermatological Index of Quality of Life in the Background of Glucocorticosteroid Therapy

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