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Journal of the American Academy of... Aug 2015The assumption that adjuvant modalities have added value to oral glucocorticoids in the treatment of pemphigus is intuitively sound but has not been conclusively proven. (Comparative Study)
Comparative Study Meta-Analysis Review
BACKGROUND
The assumption that adjuvant modalities have added value to oral glucocorticoids in the treatment of pemphigus is intuitively sound but has not been conclusively proven.
OBJECTIVE
We sought to compare the efficacy and safety of oral glucocorticoid treatment with or without adjuvants for pemphigus vulgaris and pemphigus foliaceus.
METHODS
We performed a systematic review and meta-analysis of randomized controlled trials. The primary outcome was remission. Secondary outcomes were disease control, time to disease control, relapse, time to relapse, cumulative glucocorticoid dose, withdrawal because of adverse events, and all-cause death. Trials were pooled irrespective of adjuvant type evaluated.
RESULTS
Ten trials (559 participants) were included. Adjuvants evaluated were azathioprine, mycophenolate mofetil, cyclophosphamide, cyclosporine, intravenous immunoglobulin, plasma exchange, and infliximab; not all were included in every analysis. Although adjuvants were not beneficial for achieving remission, they were found to collectively decrease the risk of relapse by 29% (relative risk 0.71, 95% confidence interval 0.53-0.95).
LIMITATIONS
Different adjuvants were pooled together.
CONCLUSION
Adjuvants have a role in pemphigus treatment, at least in reducing the risk of relapse. Further randomized controlled trials of other promising modalities are warranted.
Topics: Antineoplastic Agents; Drug Therapy, Combination; Female; Follow-Up Studies; Glucocorticoids; Humans; Immunosuppressive Agents; Male; Pemphigus; Randomized Controlled Trials as Topic; Recurrence; Remission Induction; Risk Assessment; Role; Severity of Illness Index; Treatment Outcome
PubMed: 26088689
DOI: 10.1016/j.jaad.2015.04.038 -
Frontiers in Immunology 2023Pemphigus foliaceus (PF) differs from pemphigus vulgaris (PV) in that it affects only the skin and mucous membranes are not involved. Pemphigus is commonly treated with...
BACKGROUND
Pemphigus foliaceus (PF) differs from pemphigus vulgaris (PV) in that it affects only the skin and mucous membranes are not involved. Pemphigus is commonly treated with systemic corticosteroids and immunosuppressive agents (ISAs). More recently, biologics have been used. The current literature on biologic therapy often combines treatment of PF with PV, hence it is often difficult for clinicians to isolate the treatment of PF from PV. The purpose of this review was to provide information regarding the use of current biological therapy, specifically in PF.
MATERIALS AND METHODS
A search of PubMed, Embase, and other databases was conducted using keywords pemphigus foliaceus (PF), rituximab (RTX), intravenous immunoglobulin (IVIg), and biologics. Forty-one studies were included in this review, which produced 105 patients with PF, treated with RTX, IVIg, or a combination of both. Eighty-five patients were treated with RTX, eight patients with IVIg, and 12 received both RTX and IVIg.
RESULTS
Most patients in this review had PF that was nonresponsive to conventional immunosuppressive therapies (CIST), and had significant side effects from their use. RTX treatment resulted in complete remission (CR) in 63.2%, a relapse rate of 39.5%, an infection rate of 19.7%, and a mortality rate of 3.9%. Relapse was greater in the lymphoma (LP) protocol than the rheumatoid arthritis (RA) protocol (p<0.0001). IVIg led to CR in 62.5% of patients, with no relapses or infections. Patients receiving both biologics experienced better outcomes when RTX was first administered, then followed by IVIg. Follow-up durations for patients receiving RTX, IVIg, and both were 22.1, 24.8, and 35.7 months, respectively.
DISCUSSION
In pemphigus foliaceus patients nonresponsive to conventional immunosuppressive therapy or in those with significant side effects from CIST, RTX and IVIg appear to be useful agents. Profile of clinical response, as well as relapse, infection, and mortality rates in PF patients treated with RTX were similar to those observed in PV patients. The data suggests that protocols specific for PF may produce better outcomes, less adverse effects, and improved quality of life.
Topics: Humans; Pemphigus; Immunoglobulins, Intravenous; Quality of Life; Immunosuppressive Agents; Rituximab; Recurrence; Biological Products
PubMed: 37901249
DOI: 10.3389/fimmu.2023.1267668 -
Journal of the American Academy of... May 2011A range of interventions has been described for the treatment of pemphigus; however, the optimal therapeutic strategy has not been established. (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
A range of interventions has been described for the treatment of pemphigus; however, the optimal therapeutic strategy has not been established.
OBJECTIVE
We sought to evaluate the safety and efficacy of interventions for pemphigus vulgaris and pemphigus foliaceus.
METHODS
We undertook a systematic review and meta-analysis according to the methodology of the Cochrane Collaboration. We selected randomized controlled trials including participants with the diagnosis of pemphigus vulgaris or pemphigus foliaceus confirmed with clinical, histopathological, and immunofluorescence criteria. All interventions were considered. Primary outcomes studied were remission and mortality. Secondary outcomes included disease control, relapse, pemphigus severity score, time to disease control, cumulative glucocorticoid dose, serum antibody titers, adverse events, and quality of life.
RESULTS
Eleven studies with a total of 404 participants were identified. Interventions assessed included prednisolone dose regimen, pulsed dexamethasone, azathioprine, cyclophosphamide, cyclosporine, dapsone, mycophenolate, plasma exchange, topical epidermal growth factor, and traditional Chinese medicine. We found some interventions to be superior for certain outcomes, although we were unable to conclude which treatments are superior overall.
LIMITATIONS
Many interventions for pemphigus have not been evaluated in controlled trials. All studies were insufficiently powered to establish definitive results.
CONCLUSIONS
There is inadequate evidence available at present to ascertain the optimal therapy for pemphigus vulgaris and pemphigus foliaceus. Further randomized controlled trials are required.
Topics: Azathioprine; Cyclophosphamide; Epidermal Growth Factor; Glucocorticoids; Humans; Immunosuppressive Agents; Mycophenolic Acid; Pemphigus; Randomized Controlled Trials as Topic; Remission Induction; Treatment Outcome
PubMed: 21353333
DOI: 10.1016/j.jaad.2010.04.039 -
Indian Journal of Dermatology,... 2011Pemphigus, bullous pemphigoid, and epidermolysis bullosa acquisita are autoimmune diseases of skin associated with considerable morbidity and sometimes mortality. There... (Review)
Review
BACKGROUND
Pemphigus, bullous pemphigoid, and epidermolysis bullosa acquisita are autoimmune diseases of skin associated with considerable morbidity and sometimes mortality. There is no cure for these diseases.
AIMS
To summarize evidence-based treatments for these diseases by performing a systematic review.
METHODS
The research protocol included the following steps: identification of databases to be searched, defining search strategy, searching the databases for references, first-stage screening of the abstracts, second-stage screening of full texts of articles identified after the first-stage screening, data extraction from the identified articles after second-stage screening, quality appraisal of the studies using the Delphi list, and summarizing the findings.
RESULTS
No randomized controlled trials of interventions in pemphigus vegetans, pemphigus erythematosus, and epidermolysis bullosa acquisita could be found. After the second-stage screening, 12 randomized controlled trials were analyzed, which included patients with pemphigus vulgaris or pemphigus vulgaris and pemphigus foliaceus, and 7 which included patients with bullous pemphigoid.
CONCLUSIONS
Number of high-quality randomized controlled trials conducted on pemphigus and bullous pemphigoid is small. Oral corticosteroid along with a steroid-sparing agent appears to be the most effective treatment for pemphigus. Azathioprine may be most effective as a steroid-sparing agent. Topical corticosteroid therapy (as studied) is effective for bullous pemphigoid and appears to be superior to oral corticosteroid for extensive disease. Some suggestions about future research are made.
Topics: Animals; Evidence-Based Medicine; Humans; Immunosuppressive Agents; Pemphigoid, Bullous; Pemphigus; Randomized Controlled Trials as Topic
PubMed: 21727693
DOI: 10.4103/0378-6323.82400 -
Dermatology (Basel, Switzerland) 2023Pemphigus vulgaris is a life-threatening autoimmune bullous disease characterized by flaccid blister formation. As there has been no macroscopic assessment of... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Pemphigus vulgaris is a life-threatening autoimmune bullous disease characterized by flaccid blister formation. As there has been no macroscopic assessment of epidemiological characteristics, its disease burden in the general population remains unknown.
OBJECTIVES
The aim of this study was to assess the global incidence rate of pemphigus vulgaris in the general population.
METHODS
The search was conducted in databases including Medline, Embase, Web of Science, and the Cochrane Library from inception to May 1, 2022. We included original studies that either reported the incidence of pemphigus vulgaris or provided raw data for calculating. Studies based on a specific population instead of the general population were excluded. Individual studies were summarized using random-effects mode. The pooled incidence rate of pemphigus vulgaris among the general population and subgroups was obtained. Heterogeneity (I2 statistic) was assessed with the χ2 test on Cochrane's Q statistic.
RESULTS
Twenty-nine studies were eligible for final analysis, and the pooled incidence rate of pemphigus vulgaris was 2.83 per million person-years (95% CI, 2.14-3.61). The incidence rate was similar between men and women and remained stable in the past half-century. Southern Asia showed the highest rate among subcontinents that had more than one study conducted as 4.94 per million person-years (95% CI, 2.55-8.10). Economic levels do not seem to have any bearing on incidence.
CONCLUSIONS
Despite the substantial heterogeneity among studies, this meta-analysis revealed the worldwide incidence rate of pemphigus vulgaris for the first time and may assist in assessing global disease burden and promoting health policy.
Topics: Male; Humans; Female; Pemphigus; Incidence; Autoimmune Diseases; Asia, Southern
PubMed: 36944327
DOI: 10.1159/000530121 -
International Journal of Dermatology Jun 2024Pemphigus is a group of autoimmune mucocutaneous bullous disorders characterized by acantholysis resulting from autoantibodies targeting epithelial cell surface... (Review)
Review
Pemphigus is a group of autoimmune mucocutaneous bullous disorders characterized by acantholysis resulting from autoantibodies targeting epithelial cell surface antigens. Studies reflect the presence of nail manifestations in some patients and suggest a potential correlation with clinical severity. This study examines the overall prevalence and characterizes the diverse manifestations of nail changes in pemphigus. We searched Cochrane, MEDLINE, EMBASE, and LILACS from 1990 to June 26, 2023 for studies reporting different nail changes in pemphigus patients. Data were collected and pooled to obtain proportions of the prevalence of nail changes in patients with pemphigus and subgroup analysis for pemphigus foliaceous and pemphigus vulgaris. The risk of bias was assessed with the Joanna Briggs Institute Checklist. Of 321 studies screened, 14 studies with 1,208 patients were included. Paronychia (n = 185) and Beau's lines (n = 104) were the most common nail changes identified. The pooled prevalence of nail disease in pemphigus patients was 0.389 (number of studies; [95% CI]: n = 9; [0.160-0.680], with high heterogeneity between studies (I = 95.0%, P < 0.001). Subgroup analysis revealed the highest prevalence in pemphigus foliaceous at 0.342 (n = 3; [0.109-0.688]) and pemphigus vulgaris at 0.396 (n = 5; [0.114-0.769]). Nail changes exhibited varied temporal relationships with disease onset and flares, preceding, concurrent, or following these events. Correlation with disease severity was noted, although discrepancies between studies were reported. Nail changes in pemphigus, particularly pemphigus vulgaris and pemphigus foliaceous, may be underrecognized. Observations regarding temporal associations and potential correlations with disease severity highlight the diagnostic and prognostic implications of nail changes in pemphigus. The limitations of this study include study heterogeneity and possible bias. Further research to establish the correlation of the presence and severity of nail changes on the overall disease course would be helpful.
PubMed: 38887088
DOI: 10.1111/ijd.17257 -
JAMA Dermatology Dec 2021There is a lack of evidence synthesis on the association between bullous skin disease and depression.
IMPORTANCE
There is a lack of evidence synthesis on the association between bullous skin disease and depression.
OBJECTIVE
To synthesize and interpret the current evidence on the association between bullous skin disease and depression.
EVIDENCE REVIEW
This review was conducted according to PRISMA guidelines and reviewed literature related to bullous skin disease and depression in the PubMed, Embase, PsycInfo, and Cochrane databases published between 1945 and February 2021. The quality of each included article was assessed via the Newcastle-Ottawa Scale. This review was registered with PROSPERO (CRD42021230750).
FINDINGS
A total of 17 articles were identified that analyzed a total of 83 910 patients (55.2% female; specifically, 6951 patients with bullous pemphigoid, 1669 patients with pemphigus, and 79 patients with epidermolysis bullosa were analyzed). The prevalence of depressive symptoms among patients with bullous dermatoses ranged from 40% to 80%. The prevalence of depression diagnosis among patients with bullous dermatoses ranged from 11.4% to 28%.
CONCLUSIONS AND RELEVANCE
In this systematic review, high rates of depression and depressive symptoms existed among patients with bullous skin disease. Adequate treatment of bullous dermatoses may be associated with a decrease in mental health burden on patients.
Topics: Depression; Epidermolysis Bullosa; Female; Humans; Male; Pemphigoid, Bullous; Pemphigus; Skin Diseases, Vesiculobullous
PubMed: 34668929
DOI: 10.1001/jamadermatol.2021.4055 -
Dermatologic Therapy Nov 2022Pemphigus is a group of autoimmune blistering diseases. Given the ulcerative nature of the diseases, the full healing of ulcers and erosions might be challenging. The... (Review)
Review
Pemphigus is a group of autoimmune blistering diseases. Given the ulcerative nature of the diseases, the full healing of ulcers and erosions might be challenging. The aim of this study was to determine various topical wound care options for pemphigus patients, the advantage of each alternative, and to compare their efficacy, safety, and feasibility. In accordance with PRISMA guideline, we performed a systematic search in PubMed/Medline, web of science, and Scopus databases for articles published from inception until February 2, 2022. Out of 703 initially retrieved articles, 11 full texts were included. Our findings were divided into the following categories: silver-containing dressings, paraffin-embedded tulle nets, topical insulin, EPIFIBROIN 0039, platelet gel, and Biobrane®. The most commonly used topical care in pemphigus patients was silver-containing dressings in six studies. All of the included studies reported acceptable outcomes without any severe adverse effects. Due to the few available studies in this field, a definite suggestion cannot be made. We recommend larger randomized controlled studies to identify the best topical care modality in pemphigus patients.
Topics: Humans; Bandages, Hydrocolloid; Silver; Pemphigus; Wound Healing
PubMed: 36070217
DOI: 10.1111/dth.15808 -
Acta Dermato-venereologica Nov 2015This meta-analysis examined the efficacy of different dosing regimens containing rituximab (RTX) in treating pemphigus. The analysis included 578 patients with pemphigus... (Meta-Analysis)
Meta-Analysis Review
This meta-analysis examined the efficacy of different dosing regimens containing rituximab (RTX) in treating pemphigus. The analysis included 578 patients with pemphigus from 30 studies. Seventy-six percent of patients achieved complete remission (CR) after 1 cycle of RTX. Mean time to remission was 5.8 months, with a remission duration of 14.5 months and a 40% relapse rate. Eighteen patients (3.3%) developed serious adverse effects. The pooled estimate showed no significant differences in CR and relapse rates between patients treated with high-dose (near or ≥ 2,000 mg/cycle) vs. low-dose (< 1,500 mg/cycle) RTX. In the fully adjusted analysis, high-dose RTX was associated with longer duration of CR compared with low-dose RTX. No superiority of lymphoma protocol over rheumatoid arthritis or high-dose RTX over low-dose RTX was shown in other outcomes. RTX treatment is efficacious and well-tolerated in treating pemphigus. High-dose RTX treatment may lead to longer duration of remission. However, the choice of optimal regimen depends on the overall condition of the individual patient.
Topics: Humans; Immunologic Factors; Pemphigus; Recurrence; Remission Induction; Rituximab; Time Factors
PubMed: 25881672
DOI: 10.2340/00015555-2116 -
Experimental Dermatology Jul 2023Autoimmune bullous diseases (AIBDs) are a group of rare blistering dermatoses of the mucous membrane and/or skin. The efficacy, safety and treatment durability of... (Review)
Review
A systematic review on efficacy, safety and treatment durability of intravenous immunoglobulin in autoimmune bullous dermatoses: Special focus on indication and combination therapy.
Autoimmune bullous diseases (AIBDs) are a group of rare blistering dermatoses of the mucous membrane and/or skin. The efficacy, safety and treatment durability of intravenous immunoglobulin (IVIg) as an alternative treatment should be explored to systematically review the available literature regarding treatment outcomes with IVIg in AIBD patients. The predefined search strategy was incorporated into the following database, MEDLINE/PubMed, Embase, Scopus and Web of Science on 18 July 2022. Sixty studies were enrolled using Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. The use of IVIg alone or combined with rituximab was reported in 500 patients with pemphigus, 82 patients with bullous pemphigoid, 146 patients with mucous membranes pemphigoid and 19 patients with epidermolysis bullosa acquisita. Disease remission with IVIg therapy and RTX + IVIg combination therapy were recorded as 82.8% and 86.7% in pemphigus, 88.0% and 100% in bullous pemphigoid and 91.3% and 75.0% in mucous membrane pemphigoid, respectively. In epidermolysis bullosa acquisita, treatment with IVIg led to 78.6% disease remission; no data were available regarding the treatment with RTX + IVIg in this group of patients. Among all the included patients, 37.5% experienced at least one IVIg-related side effect; the most common ones were headaches, fever/chills and nausea/vomiting. The use of IVIg with or without rituximab had a favourable clinical response in patients with AIBDs. IVIg has no major influence on the normal immune system, which makes its utilization for patients with AIBDs reasonable.
Topics: Humans; Pemphigoid, Bullous; Immunoglobulins, Intravenous; Epidermolysis Bullosa Acquisita; Pemphigus; Rituximab; Autoimmune Diseases; Skin Diseases, Vesiculobullous; Pemphigoid, Benign Mucous Membrane
PubMed: 37150538
DOI: 10.1111/exd.14829