Abstract
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.
Highlights
Factors associated with survival in pemphigus have not yet been thoroughly addressed
Out of the 130 patients enrolled in the study, 108 (83.1%) were diagnosed with pemphigus vulgaris (PV) and 22 (16.9%) with pemphigus foliaceus (PF)
The hazard ratios (HR) adjusted for age of onset as continuous variable are shown in Table 1 for PV patients and Table 2 for PF patients
Summary
Factors associated with survival in pemphigus have not yet been thoroughly addressed. In the present study, risk factors for overall mortality in a large group of patients with pemphigus vulgaris and foliaceus were investigated. Previous studies provided fragmented information suggesting that an older age of onset [3,10,11,13], concomitant involvement of the skin and mucosal surface at early disease presentation [9,14,15], delayed initiation of corticosteroid treatment [9] and the therapeutic combination of corticosteroids plus azathioprine, vs corticosteroids alone [11] are possible risk factors for overall mortality in patients with pemphigus. In a hospital-based retrospective approach, we aimed to identify possible risk factors for overall mortality in a characterised group of patients with PV and PF
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