Prognostic factors in pemphigus vulgaris and pemphigus foliaceus

Saha, Mrinal; Bhogal, B.S.; Black, Martin M.; Cooper, D. K. C.; Vaughan, R; Groves, RW

doi:10.1111/bjd.12630

Cited by 37 publications

(32 citation statements)

References 23 publications

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“…Svecova et al 17 proposed that these alleles may also contribute to disease severity of the more common variant, PV. However, in a previous study, 18 our group did not find any link in HLA DRB1* or DQB1* allele frequency with prognosis for either PV or PF.…”

Section: Discussioncontrasting

confidence: 83%

Sporadic pemphigus foliaceus and class II human leucocyte antigen allele associations in the white British and Indo-Asian populations in the UK

et al. 2018

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Summary Background Pemphigus foliaceus (PF) has both genetic and environmental susceptibility factors. Current data on human leucocyte antigen (HLA) in patients with sporadic PF are limited. Aim To better define the distribution of HLA alleles in patients with PF in the UK. Methods We recruited 36 patients [26 of white British (WB) descent, 10 of Indo‐Asian (IA) descent] with PF who were living in the UK and 159 ethnically matched normal controls, and analysed their class II HLA DRB1 and DQB1 allele distribution. Results There was an increased frequency of DRB1*1404 in association with DQB1*0503 in IA patients with PF. The DRB1*04 allele group as a whole had an increased frequency (P < 0.001) in the WB patient group compared with controls. The alleles contributing to this significance were DRB1*0401 (P = 0.03) and DRB1*0404 (P < 0.01). Conclusion This is the largest HLA association study in sporadic PF from the UK to date. There appears to be a difference in PF susceptibility alleles between WB and IA patients, highlighting the importance of racial variation in genetic susceptibility to disease development.

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Section: Discussioncontrasting

confidence: 83%

Sporadic pemphigus foliaceus and class II human leucocyte antigen allele associations in the white British and Indo-Asian populations in the UK

et al. 2018

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“…Saha et al . demonstrated that higher antidesmoglein 3 antibody titre at baseline was associated with a longer time to remission in pemphigus vulgaris.…”

Section: Discussionmentioning

confidence: 99%

“…These oral lesions require prolonged treatment with immunosuppressants and as a result puts patients at an increased risk of side‐effects . Earlier studies have implicated the role of demographic factors, such as sex and age at onset, and clinical factors, such as timing of initial treatment, site and severity of initial disease, in the prognosis of pemphigus vulgaris . Some studies have also suggested that patients with pemphigus vulgaris who have herpes simplex virus (HSV) in the oral cavity have an increased chance of having a more intractable course of oral disease.…”

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confidence: 99%

Identification of factors associated with treatment refractoriness of oral lesions in pemphigus vulgaris

Kumar

Handa

et al. 2017

Br J Dermatol

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“…While several studies have been undertaken to evaluate the risk factors for disease course, relapses and remissions in patients with pemphigus [ 5 - 8 ], few investigations evaluated the risk factors for overall mortality in patients with PV [ 9 - 12 ] and none for PF as a separate entity.…”

Section: Introductionmentioning

confidence: 99%

Prediction of survival for patients with pemphigus vulgaris and pemphigus foliaceus: a retrospective cohort study

Baican

Chiorean

Leucuţa

et al. 2015

Orphanet J Rare Dis

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BackgroundFactors associated with survival in pemphigus have not yet been thoroughly addressed. Therefore, in the present study, risk factors for overall mortality in a large group of patients with pemphigus vulgaris and foliaceus were investigated.MethodsA retrospective hospital-based cohort study was carried out, between October 1998 and November 2012, in the Department of Dermatology of the University of Medicine and Pharmacy “Iuliu Hatieganu”, Cluj-Napoca, Romania. The investigated prognostic endpoint was the overall survival of the patients.ResultsA total of 130 patients were studied (108 with pemphigus vulgaris and 22 with pemphigus foliaceus). In pemphigus vulgaris group, univariate analysis found a statistically significant association between the age of onset ≥ 65 years (p < 0.001), presence of coronary heart disease (p = 0.006), presence of cardiac arrhythmia (p = 0.004), level of anti-desmoglein1 autoantibodies ≥ 100 U/mL (p = 0.047) at diagnosis and the survival of the patients. An age-adjusted analysis showed significant results for coronary heart disease. Multivariate analysis identified the age of onset ≥ 65 years and the presence of coronary heart disease at diagnosis as independent risk factors associated with overall mortality. In patients with pemphigus foliaceus, age of onset ≥ 65 years (p = 0.021) was associated with poor survival.ConclusionsIn addition to common prognostic factors, including older age and cardiovascular comorbidities, level of autoantibodies was found to be a disease-specific factor associated with overall mortality in pemphigus vulgaris. The newly identified factors have major implications for the stratification of patients and should greatly facilitate further epidemiological studies in pemphigus. In addition, they provide useful information for the design of personalized therapeutic plans in the clinical setting.

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Prognostic factors in pemphigus vulgaris and pemphigus foliaceus

Abstract: Ethnic group, age at presentation, initial intercellular antibody titre and initial Dsg 3 antibody levels all had a significant impact on prognosis of pemphigus.

Cited by 37 publications

References 23 publications

Sporadic pemphigus foliaceus and class II human leucocyte antigen allele associations in the white British and Indo-Asian populations in the UK

Sporadic pemphigus foliaceus and class II human leucocyte antigen allele associations in the white British and Indo-Asian populations in the UK

Identification of factors associated with treatment refractoriness of oral lesions in pemphigus vulgaris

Prediction of survival for patients with pemphigus vulgaris and pemphigus foliaceus: a retrospective cohort study

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