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Immunologic Research Feb 2019The coexistence of pemphigus and systemic lupus erythematosus (SLE) had been reported anecdotally. Anti-desmoglein (Dsg)1 and anti-Dsg3 antibodies were detected... (Meta-Analysis)
Meta-Analysis
The coexistence of pemphigus and systemic lupus erythematosus (SLE) had been reported anecdotally. Anti-desmoglein (Dsg)1 and anti-Dsg3 antibodies were detected concomitantly with antinuclear autoantibodies among blood donors. The aim of the current study was to study the association between pemphigus and SLE in Israeli patients and to synthesize existing data on this association in the current literature. The current study included two sections. Initially, a cross-sectional study was performed to compare pemphigus patients with age-, sex-, and ethnicity-matched control subjects regarding the prevalence of SLE using a real-life large-scale computerized database. Next, a systematic review and meta-analysis of similar observational studies in Medline, Embase, and Web of Science (1823-2017) was conducted. As for the cross-sectional study, a total of 1985 patients with pemphigus and 9874 controls were included in the study. The prevalence of SLE was slightly higher among patients with pemphigus as compared to controls (OR, 1.85; 95% CI, 0.89-3.82). In a sensitivity analysis that included patients who received pemphigus-related treatments, the association between pemphigus and SLE had been substantiated and was statistically significant (OR, 2.10; 95% CI, 1.00-4.48). In the meta-analysis section, three eligible studies, comprising 10,389 pemphigus patients met the eligibility criteria. The overall pooled multivariate OR was 2.50 (95% CI 1.54-4.07, I = 44.19%, P = 0.167) across all studies. In conclusion, the meta-analysis provides epidemiologic evidence that these B cell-driven diseases are significantly associated. Further research is required to elucidate the molecular mechanism underlying this association.
Topics: Aged; Aged, 80 and over; Antibodies, Antinuclear; Autoantibodies; B-Lymphocytes; Comorbidity; Cross-Sectional Studies; Desmoglein 1; Desmoglein 3; Female; Humans; Israel; Lupus Erythematosus, Systemic; Male; Middle Aged; Pemphigus; Prevalence
PubMed: 30637663
DOI: 10.1007/s12026-019-9065-4 -
American Journal of Reproductive... Jan 2024Pemphigus vulgaris may worsen during pregnancy, leading to both maternal and fetal complications. The relationship between pemphigus vulgaris and pregnancy remains...
PROBLEM
Pemphigus vulgaris may worsen during pregnancy, leading to both maternal and fetal complications. The relationship between pemphigus vulgaris and pregnancy remains unclear, and the outcomes and treatments of pemphigus vulgaris during pregnancy have not been extensively discussed.
METHOD OF STUDY
This article systematically reviews the literature, focusing on the relationship between pemphigus vulgaris and pregnancy. We conducted comprehensive searches in PubMed, Embase, Cochrane Library, and Web of Science databases, identifying 42 studies reporting the disease course, pregnancy outcomes, and management of both pregnancy and pemphigus vulgaris.
RESULTS
A total of 57 cases were included in the analysis, categorized into three distinct forms: pemphigus vulgaris onset before pregnancy (n = 33), onset during pregnancy (n = 20), and onset during the postpartum period (n = 4). Fifty four cases reported treatment strategies, among them, 44 cases (81.5%) initially received systemic corticosteroid therapy during pregnancy. Out of these cases, 7 (15.9%) did not achieve successful remission and required alternative treatment approaches. In terms of pregnancy outcomes, 23 out of 62 neonates (37.1%) exhibited skin lesions or tested positive for anti-dsg IgG in their serum, while 16 neonates (25.8%) experienced other complications.
CONCLUSIONS
These findings highlight the importance of effectively managing pemphigus vulgaris during pregnancy to ensure optimal outcomes.
Topics: Pregnancy; Female; Infant, Newborn; Humans; Pemphigus
PubMed: 38282607
DOI: 10.1111/aji.13813 -
The Journal of Dermatological Treatment Feb 2015What is known and objective: Pemphigus is a severe, potentially life-threatening autoimmune blistering disease. The use of corticosteroids has dramatically improved the... (Review)
Review
UNLABELLED
What is known and objective: Pemphigus is a severe, potentially life-threatening autoimmune blistering disease. The use of corticosteroids has dramatically improved the prognosis and changed its course. However, current morbidity of pemphigus is largely iatrogenic, caused by side effects of the long-term, high-dose corticosteroid therapy that is necessary to sustain disease control. In order to minimize side effects, a range of corticosteroid-sparing immunosuppressive agents have been introduced, including mycophenolate mofetil (MMF) and enteric-coated mycophenolate sodium (EC-MPS). A systematic review was performed to evaluate the effectiveness of MMF and EC-MPS in the treatment of pemphigus vulgaris and pemphigus foliaceus.
METHODS
A retrospective literature search was conducted through multiple electronic databases (PubMed, Medline, The Cochrane database of systematic reviews) for reports on the use of mycophenolic acid (MPA) in the treatment of pemphigus vulgaris and pemphigus foliaceus.
RESULTS
Sixteen studies with a total of 239 patients have evaluated the treatment of pemphigus vulgaris and pemphigus foliac;eus with MPA. The majority of patients had refractory disease treated with corticosteroids as monotherapy or associated to adjuvant agents.
DISCUSSION
The results of this review suggest that MPA, as MMF or EC-MPS, may be a promising adjuvant or alternative therapy for the treatment of pemphigus vulgaris and pemphigus foliaceus. It appears safe, at least in the medium term and its adverse events seem to be dose dependent.
WHAT IS NEW AND CONCLUSION
The use of mycophenolate is first-line adjuvant therapy in the treatment of pemphigus vulgaris and pemphigus foliaceus.
Topics: Dermatologic Agents; Drug Therapy, Combination; Glucocorticoids; Humans; Immunosuppressive Agents; Mycophenolic Acid; Pemphigus; Prognosis; Treatment Outcome
PubMed: 24521072
DOI: 10.3109/09546634.2014.880395 -
Journal of the European Academy of... Aug 2018Epidemiological evidence suggests that smoking cigarettes may be beneficial in pemphigus, but no systematic evaluation exists to corroborate this assumption. Therefore,...
Epidemiological evidence suggests that smoking cigarettes may be beneficial in pemphigus, but no systematic evaluation exists to corroborate this assumption. Therefore, a systematic literature review with pooled data analysis of the smoking status in patients with pemphigus was conducted. Electronic searches using PubMed from inception to November 2017 identified 13 reports meeting predetermined inclusion and exclusion criteria. Most were case-control studies partly reporting that pemphigus vulgaris and foliaceus occurred less frequently in current and former smokers. Studies also indicated that duration of smoking and number of cigarettes smoked were lower in patients with pemphigus than controls and that remission may be achieved sooner in those who smoke. However, although a generally low prevalence of smoking was demonstrated in patients with pemphigus, which was lower than in controls by pooled analysis, some investigations found no difference regarding the smoking status compared with non-pemphigus subjects. One study demonstrated more severe mucosal involvement in non-smoking patients with pemphigus, whereas another observed no difference in the rate of cutaneous or mucosal lesions between smokers and non-smokers with pemphigus. This review indicates that smoking may be a possible protective factor in pemphigus, although some compromised study methodologies yet hinder any firm conclusion. Further investigations with a refined quality design are required to resolve the so far partly conflicting results in this area.
Topics: Cigarette Smoking; Humans; Pemphigus; Protective Factors
PubMed: 29478302
DOI: 10.1111/jdv.14886 -
Current Opinion in Pediatrics Aug 2016Neonatal blistering diseases are rare yet potentially fatal. Therefore, it is crucial for clinicians to know its broad range of differential diagnoses. This review... (Review)
Review
PURPOSE OF REVIEW
Neonatal blistering diseases are rare yet potentially fatal. Therefore, it is crucial for clinicians to know its broad range of differential diagnoses. This review discusses the recent literature on the causes and the most appropriate clinical approach to neonatal blistering diseases.
RECENT FINDINGS
Neonatal infections are the commonest causes for neonatal blistering. On the other hand, autoimmune blistering diseases are extremely rare with the literature limited to case reports and one systematic review only. Inherited genodermatoses are also rare, with recent developments in epidermolysis bullosa classification.
SUMMARY
In conclusion, as neonatal infections are the commonest cause for blistering, any neonate with blistering should have their blister fluid investigated for infection, while an antimicrobial should be initiated early. Autoimmune blistering diseases should be considered in neonates with a maternal history of autoimmune blistering disease. Although pemphigus and bullous pemphigoid have overall good prognoses, linear IgA bullous dermatoses has a poor prognosis and requires aggressive treatment. Inherited genodermatoses should be suspected when there is a family history of genodermatoses or consanguinity. In this case, the clinician should not hesitate to seek dermatology advice, perform a skin biopsy and consider genetic testing.
Topics: Anti-Infective Agents; Autoimmune Diseases; Diagnosis, Differential; Genetic Testing; Humans; Immunosuppressive Agents; Infant, Newborn; Practice Guidelines as Topic; Skin Diseases, Vesiculobullous
PubMed: 27386969
DOI: 10.1097/MOP.0000000000000381 -
Journal of Oral Pathology & Medicine :... Sep 2021To assess the prevalence of oral mucosal lesions in patients with Pemphigus Vulgaris. (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To assess the prevalence of oral mucosal lesions in patients with Pemphigus Vulgaris.
METHODS
Observational studies reporting the prevalence of oral lesions in pemphigus vulgaris patients, without restriction to language and year of publication, were selected in a two-phase process. Search strategies were applied to PubMed, Scopus, Livivo, Web of Science, LILACS, Google Scholar, and OpenGrey databases. Articles assessing the prevalence of oral lesions in patients with conditions other than pemphigus vulgaris were excluded. Risk of bias analysis was performed using the Joanna Briggs Institute's Critical Appraisal Checklist for Studies Reporting Prevalence Data. Synthesis of results was calculated by the software R Statistics version 4.0.2 (The R Foundation). Confidence in cumulative evidence was assessed with the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) criteria.
RESULTS
From 1957 studies identified, 40 were included in qualitative synthesis and 38 in meta-analyses. The pooled prevalence of patients with oral lesions solely or concurrent with other mucocutaneous lesions was 90.3%. The prevalence of patients with exclusive oral mucosal lesions was 50.8%. Risk of bias was considered low, and the certainty of evidence was very low.
CONCLUSION
Oral lesions were present in approximately nine out of 10 patients with pemphigus vulgaris. The oral mucosa was the most common site of disease onset. Further longitudinal studies are urged to assess the prevalence of oral lesions at different disease stages.
Topics: Humans; Mouth Mucosa; Pemphigus; Prevalence
PubMed: 33713362
DOI: 10.1111/jop.13167 -
Archives of Dermatological Research Mar 2023Previous studies have found conflicting results about the association of autoimmune blistering disease (AIBD) with cardiovascular disease (CVD) risk. The objective of... (Meta-Analysis)
Meta-Analysis
Association of autoimmune blistering disease, and specifically, pemphigus vulgaris, with cardiovascular disease and its risk factors: a systematic review and meta-analysis.
Previous studies have found conflicting results about the association of autoimmune blistering disease (AIBD) with cardiovascular disease (CVD) risk. The objective of the study was to systematically review the relationship of AIBD, including pemphigus vulgaris (PV), and its treatment with CVD and CVD risk factors. MEDLINE, EMBASE, Cochrane, LILACS, SCOPUS, and Web of Science were searched. We included all studies of CVD and CVD risk factors in AIBD patients. Two reviewers performed title and/or abstract review and data extraction. Pooled random-effects meta-analysis was performed. Forty papers met inclusion criteria. AIBD was associated with higher odds of diabetes (DM) (odds ratio [95% confidence interval]: 1.809 [1.258-2.601]), hypertension (HTN) (1.393 [1.088-1.784]), dyslipidemia (2.177 [1.163-4.073]) and heart failure (1.919 [1.603-2.298]), but was not associated with obesity, stroke, angina, heart attack, or arrhythmia. The pooled random-effects prevalence for treatment-related adverse events (AEs) in AIBD was 13.7% for DM, 10.7% for HTN, and 17.1% for CVD. Sensitivity analysis of high-quality studies revealed similar results. AIBD patients have increased CVD risk factors and heart failure. Systemic corticosteroid treatment results in CVD-related AEs in AIBD. Increased CVD screening and prevention strategies are warranted in AIBD.
Topics: Humans; Pemphigus; Cardiovascular Diseases; Autoimmune Diseases; Blister; Hypertension; Heart Failure; Risk Factors
PubMed: 35262797
DOI: 10.1007/s00403-022-02346-y -
Clinical and Experimental Medicine Aug 2023Pemphigus vulgaris is a potential life-threatening autoimmune bullous disorder. The significant role of autoreactive B cells in the pathogenesis of PV has been explained... (Review)
Review
Pemphigus vulgaris is a potential life-threatening autoimmune bullous disorder. The significant role of autoreactive B cells in the pathogenesis of PV has been explained extensively by producing autoantibodies. Recently, attention has been directed toward the role of T cells in the pathogenesis of PV; in other words, the underlying etiology of PV depends on the interaction between T cells and B cells resulting in antibody secretion. Herein, we systematically review the current literature on the emerging role of T cells in PV. To perform this systematic review, an extensive search through EMBASE, PubMed, Scopus, and ISI databases was performed from 1976 through 2021. Articles investigating the function of T cell subgroups in the pathogenesis or treatment of pemphigus vulgaris were included and reviewed. It is evidenced that T cells play a pivotal role in PV pathogenesis. Th1 and Th2 dichotomy including Th1 suppression and Th2 elevation may induce antibody production against desmoglein in keratinocytes. Furthermore, increased level of Th17 and decreased level of regulatory T cells have been detected in PV patients. However, further studies on the exact role of γδ-T cells in PV are required in order to clarify the pathogenesis of PV. T cells and their subtypes can be involved in the pathogenesis of PV. Thus, they can be considered as tentative targets of novel therapies for PV.
Topics: Humans; Pemphigus; Autoantibodies; Autoimmune Diseases; T-Lymphocytes, Regulatory; T-Lymphocyte Subsets
PubMed: 35925475
DOI: 10.1007/s10238-022-00855-8 -
Autoimmunity Reviews May 2019Bullous pemphigoid and pemphigus constitute two major autoimmune blistering diseases (AIBD) with complicated disease pathomechanisms involving a multitude of cytokines... (Meta-Analysis)
Meta-Analysis
Bullous pemphigoid and pemphigus constitute two major autoimmune blistering diseases (AIBD) with complicated disease pathomechanisms involving a multitude of cytokines and immunological pathways. The purpose of our literature review of the cytokines and chemokines involved in these AIBDs was to allow for a meta-analysis of studies detailing differential cytokine and chemokine changes in these conditions. Elucidation of inflammatory pathways could lead to more targeted therapies, several of which specific monoclonal antibodies already exist and are used safely for other autoimmune diseases. A systematic review of the Pubmed/Medline database was performed for articles characterizing cytokines/chemokines involved in BP and pemphigus. Further, a meta-analysis was carried out using standardized methods, including assessment for heterogeneity. The results of our analysis demonstrated numerous inflammatory alterations in these AIBDs. Significant alterations included serum levels of IL-5, IL-6, IL-8, IL-17, CCL-17, and CCL-26 in patients with BP, and increased blister fluids levels of IL-5, IL-6, IL-8, CCL11, and TNF-α. Blister fluid levels of IL-1α are decreased in BP. In pemphigus, we identified significantly increased serum levels of IL-10, IL-17, and CCL17. We have additionally summarized all studies excluded from meta-analysis to provide a comprehensive summary of cytokine/chemokine alterations in these two conditions.
Topics: Blister; Body Fluids; Chemokines; Cytokines; Humans; Pemphigoid, Bullous; Pemphigus
PubMed: 30844553
DOI: 10.1016/j.autrev.2019.03.009 -
The Cochrane Database of Systematic... Aug 2023Bullous pemphigoid (BP) is the most common autoimmune blistering disease. Oral steroids are the standard treatment. We have updated this review, which was first... (Review)
Review
BACKGROUND
Bullous pemphigoid (BP) is the most common autoimmune blistering disease. Oral steroids are the standard treatment. We have updated this review, which was first published in 2002, because several new treatments have since been tried.
OBJECTIVES
To assess the effects of treatments for bullous pemphigoid.
SEARCH METHODS
We updated searches of the following databases to November 2021: Cochrane Skin Specialised Register, CENTRAL, MEDLINE, and Embase. We searched five trial databases to January 2022, and checked the reference lists of included studies for further references to relevant randomised controlled trials (RCTs).
SELECTION CRITERIA
RCTs of treatments for immunofluorescence-confirmed bullous pemphigoid.
DATA COLLECTION AND ANALYSIS
At least two review authors, working independently, evaluated the studies against the review's inclusion criteria and extracted data from included studies. Using GRADE methodology, we assessed the certainty of the evidence for each outcome in each comparison. Our primary outcomes were healing of skin lesions and mortality.
MAIN RESULTS
We identified 14 RCTs (1442 participants). The main treatment modalities assessed were oral steroids, topical steroids, and the oral anti-inflammatory antibiotic doxycycline. Most studies reported mortality but adverse events and quality of life were not well reported. We decided to look at the primary outcomes 'disease control' and 'mortality'. Almost all studies investigated different comparisons; two studies were placebo-controlled. The results are therefore based on a single study for each comparison except azathioprine. Most studies involved only small numbers of participants. We assessed the risk of bias for all key outcomes as having 'some concerns' or high risk, due to missing data, inappropriate analysis, or insufficient information. Clobetasol propionate cream versus oral prednisone Compared to oral prednisone, clobetasol propionate cream applied over the whole body probably increases skin healing at day 21 (risk ratio (RR 1.08, 95% confidence interval (CI) 1.03 to 1.13; 1 study, 341 participants; moderate-certainty evidence). Skin healing at 21 days was seen in 99.8% of participants assigned to clobetasol and 92.4% of participants assigned to prednisone. Clobetasol propionate cream applied over the whole body compared to oral prednisone may reduce mortality at one year (RR 0.73, 95% CI 0.53 to 1.01; 1 study, 341 participants; low-certainty evidence). Death occurred in 26.5% (45/170) of participants assigned to clobetasol and 36.3% (62/171) of participants assigned to oral prednisone. This study did not measure quality of life. Clobetasol propionate cream may reduce risk of severe complications by day 21 compared with oral prednisone (RR 0.65, 95% CI 0.50 to 0.86; 1 study, 341 participants; low-certainty evidence). Mild clobetasol propionate cream regimen (10 to 30 g/day) versus standard clobetasol propionate cream regimen (40 g/day) A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen probably does not change skin healing at day 21 (RR 1.00, 95% CI 0.97 to 1.03; 1 study, 312 participants; moderate-certainty evidence). Both groups showed complete healing of lesions at day 21 in 98% participants. A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen may not change mortality at one year (RR 1.00, 95% CI 0.75 to 1.32; 1 study, 312 participants; low-certainty evidence), which occurred in 118/312 (37.9%) participants. This study did not measure quality of life. A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen may not change adverse events at one year (RR 0.94, 95% CI 0.78 to 1.14; 1 study, 309 participants; low-certainty evidence). Doxycycline versus prednisolone Compared to prednisolone (0.5 mg/kg/day), doxycycline (200 mg/day) induces less skin healing at six weeks (RR 0.81, 95% CI 0.72 to 0.92; 1 study, 213 participants; high-certainty evidence). Complete skin healing was reported in 73.8% of participants assigned to doxycycline and 91.1% assigned to prednisolone. Doxycycline compared to prednisolone probably decreases mortality at one year (RR 0.25, 95% CI 0.07 to 0.89; number needed to treat for an additional beneficial outcome (NNTB) = 14; 1 study, 234 participants; moderate-certainty evidence). Mortality occurred in 2.4% (3/132) of participants with doxycycline and 9.7% (11/121) with prednisolone. Compared to prednisolone, doxycycline improved quality of life at one year (mean difference 1.8 points lower, which is more favourable on the Dermatology Life Quality Index, 95% CI 1.02 to 2.58 lower; 1 study, 234 participants; high-certainty evidence). Doxycycline compared to prednisolone probably reduces severe or life-threatening treatment-related adverse events at one year (RR 0.59, 95% CI 0.35 to 0.99; 1 study, 234 participants; moderate-certainty evidence). Prednisone plus azathioprine versus prednisone It is unclear whether azathioprine plus prednisone compared to prednisone alone affects skin healing or mortality because there was only very low-certainty evidence from two trials (98 participants). These studies did not measure quality of life. Adverse events were reported in a total of 20/48 (42%) participants assigned to azathioprine plus prednisone and 15/44 (34%) participants assigned to prednisone. Nicotinamide plus tetracycline versus prednisone It is unclear whether nicotinamide plus tetracycline compared to prednisone affects skin healing or mortality because there was only very low-certainty evidence from one trial (18 participants). This study did not measure quality of life. Fewer adverse events were reported in the nicotinamide group. Methylprednisolone plus azathioprine versus methylprednisolone plus dapsone It is unclear whether azathioprine plus methylprednisolone compared to dapsone plus methylprednisolone affects skin healing or mortality because there was only very low-certainty evidence from one trial (54 participants). This study did not measure quality of life. A total of 18 adverse events were reported in the azathioprine group and 13 in the dapsone group.
AUTHORS' CONCLUSIONS
Clobetasol propionate cream applied over the whole body is probably similarly effective as, and may cause less mortality than, oral prednisone for treating bullous pemphigoid. Lower-dose clobetasol propionate cream applied over the whole body is probably similarly effective as standard-dose clobetasol propionate cream and has similar mortality. Doxycycline is less effective but causes less mortality than prednisolone for treating bullous pemphigoid. Other treatments need further investigation.
Topics: Humans; Azathioprine; Prednisone; Clobetasol; Pemphigoid, Bullous; Doxycycline; Methylprednisolone; Dapsone; Niacinamide
PubMed: 37572360
DOI: 10.1002/14651858.CD002292.pub4