title: Mortality and Cause of Death in Patients with Pemphigus.
abstract: All-cause and cause-specific mortality among patients with pemphigus compared with the general population is yet to be established. This study investigated overall mortality and cause-specific mortality in a large immunopathologically validated cohort of patients with pemphigus. Mortality of patients with pemphigus was compared with age- and gender-matched control subjects in the general population. All-cause and cause-specific standardized mortality ratios (SMRs) were estimated. The study cohort included 245 patients newly-diagnosed with pemphigus between January 1990 and June 2016, contributing 2,679.4 person-years of follow-up. Overall, 48 deaths were observed during a mean follow-up period of 10.9 ± 8.1 years, which was more than twice the number expected (SMR 2.4; 95% confidence interval (95% CI) 1.82-3.20). The SMRs for death due to infections (22.6; 95% CI 13.6-35.3), namely pneumonia (25.7; 95% CI 11.7-48.8) and septicaemia (8.6; 95% CI 1.7-25.0), and due to cardiovascular diseases (2.8; 95% CI 1.0-6.0) were significantly higher than expected. Overall mortality among patients with pemphigus is 2.4-times greater than for the general population, mainly due to infections.
authors.affiliation: Department of Dermatology, Rambam Health Care Campus. POB 9602, 31096 Haifa, Israel. dr_kridin@hotmail.com.
authors.forename: Khalaf
authors.initials: K
authors.lastname: Kridin
authors.forename: Shira Zelber
authors.initials: SZ
authors.lastname: Sagi
authors.forename: Reuven
authors.initials: R
authors.lastname: Bergman
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018912:Arabs; D002318:Cardiovascular Diseases / Q000208:ethnology / Q000401:mortality; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007557:Israel / Q000453:epidemiology; D007585:Jews; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000175:diagnosis / Q000208:ethnology / Q000401:mortality*; D011014:Pneumonia / Q000208:ethnology / Q000401:mortality; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D018805:Sepsis / Q000208:ethnology / Q000401:mortality; D013997:Time Factors; D055815:Young Adult