Pemphigus vulgaris – still a difficult disease to treat

Menezes, Nuno; Leite, Inês; Tente, David; Couceiro, António; Baptista, Armando; Varela, Paulo

doi:10.1111/j.1365-4632.2009.04192.x

Cited by 6 publications

(6 citation statements)

References 6 publications

(6 reference statements)

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“…Kelesidis et al 22 reported that, unlike patients with lymphoma, the risk of infection does not increase significantly following rituximab therapy in autoimmune diseases like rheumatoid arthritis. Although an acceptable safety profile of rituximab in the treatment of PV has been reported by several authors, [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] 22.5% of our patients experienced infectious complications following rituximab dosing, including disseminated herpes, lung abscess, skin abscess, pneumonia, sepsis, and sinus cavernous thrombosis.…”

Section: Discussionmentioning

confidence: 81%

“…Immediate‐early clinical improvement, durability of remission, and significant prednisolone dose reduction following rituximab therapy in recalcitrant PV are the common points among the most reported studies and ours …”

Section: Discussionmentioning

confidence: 82%

“…Immediate-early clinical improvement, durability of remission, and significant prednisolone dose reduction following rituximab therapy in recalcitrant PV are the common points among the most reported studies and ours. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] The major limitations of our study include lack of a control group and open label treatment with unblinded assessment of clinical response.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 It is a chimeric murine/human monoclonal IgG against CD20 on pre-and mature B-cells, and the key pathogenic role of B-cell in most of the aforementioned disorders may well explain the efficacy of rituximab in such diseases. 1,2 Although an increasing number of studies have shown the therapeutic efficacy and safety of rituximab in recalcitrant pemphigus vulgaris (PV), [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] to date there is still no consensus about its dosing regimens, timing, or safety profile. Recalcitrant PV can be a potentially fatal condition necessitating systemic corticosteroid as the mainstay of treatment along with adjuvants that can potentiate earlier control and improve the clinical response with the lowest possible dose of steroids.…”

Section: Introductionmentioning

confidence: 99%

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Adjuvant rituximab in the treatment of pemphigus vulgaris: a phase II clinical trial

et al. 2013

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Rituximab has been tried increasingly for the treatment of pemphigus vulgaris (PV). However, there is still no consensus about its dosing regimens, efficacy, and side effects due to insufficient clinical trials. The goal of this study was to evaluate its efficacy in the treatment of PV. This is a case series of patients with PV who received rituximab, four doses of 375 mg/m(2) intravenously weekly, plus concomitant oral prednisolone. The primary outcomes were the rate of initial clinical improvement and marked clinical improvement; secondary outcomes included prednisolone doses (mg/d) at baseline, three months, six months and the last visit, as well as the side effects. Forty out of 45 patients completed the study. The mean follow-up time was 12 ± 10.69 months (range of 3-46 months). Following treatment with rituximab, all the analyzed patients with PV had initial clinical improvement after a mean period of 6.35 weeks and a marked clinical improvement after a mean of 10.13 months. The mean prednisolone dose (mg/d) decreased significantly from a baseline level of 48.75 ± 25.86 to 26.50 ± 12.95 at three months, 20.70 ± 17.51 at six months, and 15.26 ± 9.98 at the last visit (P = 0.0001). The encountered side effects following rituximab were lung abscess, sepsis, pneumonia, cavernous sinus thrombosis, skin abscess, deep vein thrombosis, generalized arthralgia, and Stevens-Johnson syndrome. According to our study, rituximab may be an effective adjuvant to prednisolone for the treatment of PV; however, its safety profile remains concerning.

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Section: Discussionmentioning

confidence: 81%

Section: Discussionmentioning

confidence: 82%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Adjuvant rituximab in the treatment of pemphigus vulgaris: a phase II clinical trial

et al. 2013

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“…In total, we recorded data of 155 pemphigus patients, 124 of whom had PV while 31 had PF patients with ages ranging from 4 to 86 years . There was no association between disease type and clinical outcomes, with 80% of both PV and PF patients achieving complete response ( P = 0.95).…”

Section: Resultsmentioning

confidence: 99%

An assessment of treatment history and its association with clinical outcomes and relapse in 155 pemphigus patients with response to a single cycle of rituximab

Amber

Hertl

2014

Acad Dermatol Venereol

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The Emerging Role of Rituximab in Autoimmune Blistering Diseases

Ahmed

Shetty

2015

Am J Clin Dermatol

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Rituximab is a chimeric monoclonal antibody that selectively binds to the CD20 molecule on B cells, resulting in their lysis. In autoimmune blistering diseases, the auto-antibody-producing B cells are destroyed and auto-antibody levels are reduced or eliminated. In the majority of patients, rituximab produces rapid clinical response and early resolution. In part, this accounts for the increased use of rituximab. Rituximab does not distinguish normal from pathologic B cells. Hence, shortly after its use, B-cell levels are zero and remain so for several months. In most patients, the use of systemic corticosteroids and immunosuppressive agents are continued after rituximab therapy, while their dosages are significantly decreased. In the majority of patients rituximab is used according to the protocol used in treating lymphoma patients or patients with rheumatoid arthritis. Approximately 50% of patients experience a relapse, requiring additional therapy. Serious adverse events and fatal outcomes have been reported, although their incidence is less than that observed with conventional therapy. Nonetheless, the causes, i.e. infections and septicemia, are similar. Several gaps exist in our understanding of how to optimally benefit from the use of this valuable biological agent. Future studies need to be targeted in designing and implanting protocols that maximize the benefit of rituximab and result in producing sustained prolonged remissions with minimal adverse events and a high quality of life.

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Pemphigus vulgaris – still a difficult disease to treat

Cited by 6 publications

References 6 publications

Adjuvant rituximab in the treatment of pemphigus vulgaris: a phase II clinical trial

Adjuvant rituximab in the treatment of pemphigus vulgaris: a phase II clinical trial

An assessment of treatment history and its association with clinical outcomes and relapse in 155 pemphigus patients with response to a single cycle of rituximab

The Emerging Role of Rituximab in Autoimmune Blistering Diseases

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