Rituximab/IVIG in pemphigus – a 10-year study with a long follow-up

Hamadah, Issam; Chisti, Muzamil; Haider, Mansoor A.; Binamer, Yousef; Alajlan, Saad; Aleyouni, Yousif; Alfadley, Abdullah

doi:10.1080/09546634.2018.1484873

Cited by 10 publications

(7 citation statements)

References 38 publications

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“…Initial IVIG therapy rapidly elevates total circulating Ig levels, which is thought to stimulate homeostatic antibody-catabolic mechanisms, leading to lowering of serum levels of IgG1 and IgG4 anti-Dsg 1 and Dsg 3 antibodies (Green and Bystryn, 2008) while normal antibodies are replaced by those in subsequent IVIG doses: this is a particularly useful treatment when fast onset of therapy and lower risk of infection is needed. Combining rituximab and IVIG can be effective for the treatment of refractory pemphigus cases and may even induce long-term complete remission with lower risk of infection (Hamadah et al, 2019): accordingly, a clinical trial was started in June 2020 to evaluate the efficacy and safety of early use of rituximab with or without IVIGs in patients with moderate to severe pemphigus (NCT04400994). Immunoadsorption, which also rapidly removes anti-Dsg IgG from the circulation, has also been trialled in conjunction with conventional immunosuppressive treatment and intravenous rituximab (Eming and Hertl, 2006;Behzad et al, 2012).…”

Section: Adjunct Therapiesmentioning

confidence: 99%

Autoimmune Pemphigus: Latest Advances and Emerging Therapies

Lim

Bohelay

Hanakawa

et al. 2022

Front. Mol. Biosci.

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Pemphigus represents a group of rare and severe autoimmune intra-epidermal blistering diseases affecting the skin and mucous membranes. These painful and debilitating diseases are driven by the production of autoantibodies that are mainly directed against the desmosomal adhesion proteins, desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1). The search to define underlying triggers for anti-Dsg-antibody production has revealed genetic, environmental, and possible vaccine-driven factors, but our knowledge of the processes underlying disease initiation and pathology remains incomplete. Recent studies point to an important role of T cells in supporting auto-antibody production; yet the involvement of the myeloid compartment remains unexplored. Clinical management of pemphigus is beginning to move away from broad-spectrum immunosuppression and towards B-cell-targeted therapies, which reduce many patients’ symptoms but can have significant side effects. Here, we review the latest developments in our understanding of the predisposing factors/conditions of pemphigus, the underlying pathogenic mechanisms, and new and emerging therapies to treat these devastating diseases.

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Section: Adjunct Therapiesmentioning

confidence: 99%

Autoimmune Pemphigus: Latest Advances and Emerging Therapies

Lim

Bohelay

Hanakawa

et al. 2022

Front. Mol. Biosci.

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“…With the clinical success and subsequent United States Food and Drug Administration's approval of rituximab for pemphigus, reviewed in detail elsewhere, much attention has been given towards optimizing B‐cell targeting . Although small studies suggest rituximab may not be as effective for BP as for pemphigus, at this time rituximab is one of the most effective treatments for EBA .…”

Section: Evolving Therapeutic Targetsmentioning

confidence: 99%

“…The application of rituximab and intravenous immunoglobulin (IVIg) in combination has shown how different aspects of these diseases can be effectively targeted . 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 .…”

Section: Introductionmentioning

confidence: 99%

“…[36][37][38][39][40][41] 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. [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%

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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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“…However, there is no consensus on the dose regimen and duration for PV. A previous report suggested various successful rituximabregimens for pemphigus such as rheumatology, oncology, and IVIG combination protocols [4]. Other reports demonstrated that lowdose rituximab (375 mg/m 2 , 2 times, 1-2 weeks apart) had similar effects in autoimmune diseasescompared to high-dose rituximab (1000mg, 2 times, 2 weeks apart) in the time to achieve rapid improvement [5][6][7].…”

mentioning

confidence: 99%

Successful Treatment of Pemphigus Vulgaris with a Modified Regimen of Rituximab Biosimilar(CT-P10) Combined with IVIG

Kim¹,

Hong²,

Kim³

et al. 2020

CRDOA

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Keywords: Pemphigus vulgaris;Modified Regimen;Rituximab biosimilar; IVIG Pemphigus Vulgaris (PV) has been known as fatal autoimmune bullous diseases affecting the skin and mucous membranes. Here, we report that a patient with PV responded rapidly to a modified regimen of rituximab biosimilar (CT-10, Truxima®)combined with Intravenous Immunoglobulin (IVIG). A 53-year-old man presented with extensive erosions due to blistering on the whole body following initial mucosal erosions which started at 3 years ago. Laboratory tests revealed an eosinophil count of 16.7% (normal range: 0~5%) with normal liver and renal functions. Antinuclear antibody was a nuclear membrane pattern with a titer of 1:40. Anti-dsDNA, anti-Ro/La, anti-Scl-70, and anti-phospholipid antibodies were negative. Myeloperoxidase and PR3 antineutrophil cytoplasmic autoantibody were both negative. Histological examination showed suprabasala cantholysis, while direct and indirect immunofluorescence assays demonstrated a deposition of immunoglobulin G (IgG) on anintercellular surface at 1:640 dilutions (Figure. 1a, b). Based on the blistering on the whole body and characteristic biopsy result, the patient was diagnosed as PV. Although he was initially treated with topical and highdose methylprednisolone (1000 mg/day) pulse therapy for 3 consecutive days, there was no significant improvement in skin blistering lesions. We decided to administer CT-P10 combined with IVIG. We treated the patient with CT-P10 (500, 500, and 1000mg once weekly for 3 weeks) combined with IVIG (1g/ kg at first and third week). During the treatment of CT-P10 combined with IVIG, a clinical response rapidly appeared, and levels of antibodies were decreased from 1:640 to 1:40 (Figure. 1c). Disease activity was dramatically controlled within 3 weeks. It achieved the end of the consolidation phase for PV within 5 weeks after starting CT-P10 combined with IVIG (Figure. 1dg). Then, we tapered oral prednisolone and steroid-sparing agents including mycophenolate mofetil and cyclosporine for maintenance therapy. Until now, there has been no recurrence of PV on 36 months follow up.

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Rituximab/IVIG in pemphigus – a 10-year study with a long follow-up

Abstract: Rituximab is an effective and safe treatment for pemphigus and should be considered earlier in the algorithm of pemphigus treatment.

Cited by 10 publications

References 38 publications

Autoimmune Pemphigus: Latest Advances and Emerging Therapies

Autoimmune Pemphigus: Latest Advances and Emerging Therapies

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

Successful Treatment of Pemphigus Vulgaris with a Modified Regimen of Rituximab Biosimilar(CT-P10) Combined with IVIG

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