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

BACKGROUND: Factors 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. METHODS: A 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. RESULTS: A 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. CONCLUSIONS: In 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.

Pemphigus vulgaris is the most common autoimmune bullous disease in Northwestern Romania.

BACKGROUND: Autoimmune bullous diseases are organ-specific diseases characterized by autoreactive T and B cells specific for structural proteins of the skin. The incidence and prevalence of autoimmune blistering diseases vary in different countries and their epidemiology has not yet been addressed in Romania. METHODS: In this study between 2001 and 2007, we prospectively investigated a total of 116 patients with autoimmune blistering diseases from the Northwestern region of Romania. The diagnosis was based on the clinical, histo- and immunohistological as well as serological findings. RESULTS: Pemphigus was the most common disease representing 58.6% of the case (68 cases); 40 cases (34.5%) were diagnosed with bullous pemphigoid (BP) and eight cases (6.9%) with other autoimmune sub-epidermal diseases. The incidence and prevalence of pemphigus diseases were four patients/1,000,000 inhabitants/year and 0.00248%, respectively. BP occurred in 2.5/1,000,000 inhabitants/year and its prevalence was 0.00146%. While the average onset age for pemphigus vulgaris was 53 years, BP patients were first diagnosed at a mean age of 73.6 years. CONCLUSION: The genetic background of the local population may explain why pemphigus occurs more commonly than BP in Northwestern Romania compared with the population of Western Europe. In addition, the shorter life expectancy in Romania (71.3 years) compared with Western Europe (>80 years) may contribute to this phenomenon.