Prognostic factors in pemphigus vulgaris patients: A retrospective cohort study

Objective: We aimed to identify the prognostic factors affecting the clinical course of PV in addition to describing the demographic, laboratory and clinical features of pemphigus vulgaris (PV) patients that has been treated in Istanbul School of Medicine, Bullous Diseases Clinic, within the years of 1979 and 2014. Materials and Methods: We retrospectively examined the medical files of 426 patients diagnosed with PV with typical clinical features, histopathological and immunological investigations between the years of 1979 and 2014. A total of 184 PV patients that satisfied the inclusion criteria were included in the study. Demographic, laboratory and clinical features as well as treatment history, response to treatment and adverse effect profile were investigated. In order to assess the prognostic factors, patients were compared according to relapse rates and time to cessation of treatment (disease duration). Results: A hundred and eighty-four patients diagnosed with PV were included in the study. Mean age was found to be 42,9 in the study group. Female to male ratio was 1,8. Initial remission was achieved in 98,9% of the cases. In 16,9% of the cases initial remission was permanent; in 83,2% of the cases, at least one relapse took place. Mean time to initial remission was 16 months, whereas mean duration of initial remission was found to be 20,5 months. In the final consultation, in 13% of the patients, the disease was still active, 4,3% of the patients were in partial remission, 32,6% of the patients were in complete remission on therapy, 11,4% of the patients were in complete remission on therapy and 38,6% of the patients were in a long term remission with at least two years. Severe disease and anti-desmoglein 3 IgG (Dsg 3) levels higher than 20 U/ml on initial remission was found to be significantly affecting the relapse rate both on univariate (p=0,05 and p=0,026 respectively) and multivariate analyses (p=0,027 and p=0,022 respectively). Mean disease duration of the population was 164,9 months. Rate of achieving complete remission off treatment was 1,7% on first year, 25,6% on the fifth year, 49,2% on 10th year and 79,2% by the end of the 20th year. Genital involvement (p=0,047), diabetes mellitus (DM) presence (p=0,008), hyperlipidemia presence (p=0,001), use of adjuvant treatment (0,048), positive indirect immuneflouresence (IIF) investigation on remission (p=0,012) and Dsg 3 levels above 20 U/ml on initial remission (p<0,001) were found to be significantly associated with longer disease duration. However, on multivariate analysis, only DM presence (p=0,008), hyperlipidemia presence (p=0,044) and Dsg 3 elevation on remission (p=0,05) was significantly associated with diseases duration. Discussion: Dsg 3 levels above 20 U/ml on remission, DM and hyperlipidemia presence were found to be significantly affecting the disease duration in our study. In addition, severe disease at the time of diagnosis and Dsg 3 levels above 20 U/ml on remission was found to be significantly associated with relapse. These findings are in line with previous reports on the subject. However, the relationship between the presence of DM or hyperlipidemia and response to treatment has not been defined before.