Retrospective analysis of a single‐center clinical experience toward development of curative treatment of 123 pemphigus patients with a long‐term follow‐up: efficacy and safety of the multidrug protocol combining intravenous immunoglobulin with the cytotoxic immunosuppressor and mitochondrion‐protecting drugs

Grando, Sergei A.

doi:10.1111/ijd.14143

Cited by 24 publications

(23 citation statements)

References 82 publications

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“…First, we determined whether serum levels of these non-Dsg AuAb species correlate with the Pemphigus Disease Area and Activity Index (PDAAI) in the Dsg1/3 AuAb-negative acute PV patients ( Table 1). All patients were treated by our original multidrug protocol described elsewhere (17), and their serum samples were obtained prior to treatment and right after they reached complete remission (i.e. complete healing of erosions with or without secondary skin changes in the absence of new lesions and with negative Nikolskiy sign (i.e.…”

Section: Evaluation Of Auabs To Specific Non-dsg Antigens In Sera Of mentioning

confidence: 99%

“…The mucosal type of PV was diagnosed in seven patients, and the mucocutaneous type was diagnosed in five patients. Prior to initiation of treatment of our Dsg1/3 AuAb-negative acute PV patients with the multidrug protocol detailed elsewhere (17), the mean duration of the disease was 1.6 years. The titer of "intercellular" antibodies determined on monkey esophagus ranged from 1/320 to 1/5120.…”

Section: Pemphigus Seramentioning

confidence: 99%

“…Three patients had refractory disease, and eight had a relapse. The severity of disease was determined using the PDAAI, described elsewhere (17).…”

Section: Pemphigus Seramentioning

confidence: 99%

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Synergy among non-desmoglein antibodies contributes to the immunopathology of desmoglein antibody–negative pemphigus vulgaris

Chernyavsky¹,

Amber

Agnoletti³

et al. 2019

Journal of Biological Chemistry

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Edited by Peter Cresswell Pemphigus vulgaris (PV) is a potentially lethal mucocutaneous blistering disease characterized by IgG autoantibodies (AuAbs) binding to epidermal keratinocytes and inducing this devastating disease. Here, we observed that non-desmoglein (Dsg) AuAbs in the sera of patients with Dsg1/3 AuAb-negative acute PV are pathogenic, because IgGs from these individuals induced skin blistering in neonatal mice caused by suprabasal acantholysis. Serum levels of AuAbs to desmocollin 3 (Dsc3), M3 muscarinic acetylcholine receptor (M3AR), and secretory pathway Ca 2؉/ Mn 2؉ -ATPase isoform 1 (SPCA1) correlated with the disease stage of PV. Moreover, AuAb absorption on recombinant Dsc3, M3AR, or SPCA1 both prevented skin blistering in the passive transfer of AuAbs model of PV in BALB/c mice and significantly decreased the extent of acantholysis in a neonatal mouse skin explant model. Although acantholytic activities of each of these immunoaffinity-purified AuAbs could not induce a PV-like phenotype, their mixture produced a synergistic effect manifested by a positive Nikolskiy sign in the skin of neonatal mice. The downstream signaling of all pathogenic non-Dsg AuAbs involved p38 mitogen-activated protein kinase (MAPK)-mediated phosphorylation and elevation of cytochrome c release and caspase 9 activity. Anti-Dsc3 and anti-SPCA1 AuAbs also activated SRC proto-oncogene, nonreceptor tyrosine kinase (SRC). Of note, although a constellation of non-Dsg AuAbs apparently disrupted epidermal integrity, elimination of a single pathogenic AuAb could prevent keratinocyte detachment and blistering. Therefore, anti-Dsg1/3 AuAb-free PV can be a model for elucidating the roles of non-Dsg antigenspecific AuAbs in the physiological regulation of keratinocyte cell-cell adhesion and blister development.

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Section: Evaluation Of Auabs To Specific Non-dsg Antigens In Sera Of mentioning

confidence: 99%

Section: Pemphigus Seramentioning

confidence: 99%

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Synergy among non-desmoglein antibodies contributes to the immunopathology of desmoglein antibody–negative pemphigus vulgaris

Chernyavsky¹,

Amber

Agnoletti³

et al. 2019

Journal of Biological Chemistry

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“…While these therapies have become more accepted as a standard of care, corticosteroids are still generally required for prolonged periods of time for acute disease . Likewise, relapses are common, especially in shorter treatment protocols, rather than protracted protocols . As such, improved treatment targets are still desperately needed.…”

Section: Introductionmentioning

confidence: 99%

“…[42][43][44][45][46][47][48] Likewise, relapses are common, especially in shorter treatment protocols, rather than protracted protocols. 36,[49][50][51][52][53] As such, improved treatment targets are still desperately needed.…”

Section: Introductionmentioning

confidence: 99%

From bench to bedside: evolving therapeutic targets in autoimmune blistering disease

Kridin

Kowalski

Kneiber

et al. 2019

Acad Dermatol Venereol

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Autoimmune blistering diseases comprise a group of heterogenous conditions characterized by the loss of tolerance and subsequent development of autoantibodies targeting epidermal and subepidermal adhesion proteins. Blisters and erosions form on the skin and mucous membranes leading to significant morbidity and mortality. Traditional therapies rely on systemic immunosuppression. Advancements in our understanding of the pathophysiology of pemphigus and pemphigoid have led to the development of molecules which target specific pathways involved in induction and perpetuation of disease. In this review, we outline the novel therapeutic strategies including B‐cell depletion, T‐regulatory cell repletion, cell signalling inhibitors and small molecular inhibitors, inhibitory monoclonal antibodies, as well as complement inhibition. We additionally review their current level of clinical evidence. We lastly review therapeutics targets gleaned from the experimental epidermolysis bullosa acquisita mouse model. These emerging treatments offer an exciting progression from basic science discoveries that have the potential to transform the treatment paradigm in autoimmune blistering diseases.

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Transient lymphadenopathy during intravenous immunoglobulin (IVIg) in a patient with pemphigus vulgaris

Foulad

Rigas²,

Piracha³

et al. 2021

Clinical Case Reports

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Abstract: While the individualized therapeutic approaches to eradicate the autoreactive B cell clones causing disease in each particular PV or PF patient are being developed, all pemphigus patients can benefit from the treatment protocol described in this study.

Cited by 24 publications

References 82 publications

Synergy among non-desmoglein antibodies contributes to the immunopathology of desmoglein antibody–negative pemphigus vulgaris

Synergy among non-desmoglein antibodies contributes to the immunopathology of desmoglein antibody–negative pemphigus vulgaris

From bench to bedside: evolving therapeutic targets in autoimmune blistering disease

Transient lymphadenopathy during intravenous immunoglobulin (IVIg) in a patient with pemphigus vulgaris

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