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INVESTIGATIVE REPORT
Mortality and Cause of Death in Patients with Pemphigus
Khalaf KRIDIN 1 , Shira ZELBER-SAGI 2 and Reuven BERGMAN 1,3
1
Department of Dermatology, Rambam Health Care Campus and 2 School of Public Health, Faculty of Social Welfare and Health Sciences,
University of Haifa, 3 Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel
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 immu-
nopathologically validated cohort of patients with pem-
phigus. Mortality of patients with pemphigus was com-
pared 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-diagno-
sed 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 fol-
low-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 pneu-
monia (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 pem-
phigus is 2.4-times greater than for the general popu-
lation, mainly due to infections.
Key words: mortality; prognosis; survival; cause of death;
pemphigus, Israel.
Accepted Jan 12, 2017; Epub ahead of print Jan 17, 2017
Acta Derm Venereol 2017; 97: 607–611.
Corr: Khalaf Kridin, Department of Dermatology, Rambam Health Care
Campus, POB 9602, Haifa 31096, Israel. E-mail: [email protected]
P
emphigus is a rare, chronic, potentially life-threa-
tening, autoimmune blistering disease of the skin
and mucous membranes. There are 2 major subtypes of
pemphigus: pemphigus vulgaris (PV) and pemphigus
foliaceus (PF). The aetiopathogenesis of pemphigus is
characterized by acantholysis and intraepidermal blister
formation, resulting from IgG autoantibodies directed
against desmoglein (Dsg) 3 (PV) an