ABSTRACT
BACKGROUND: Pemphigus has in the past been associated with a high mortality rate. However, with the discovery of corticosteroids, patient median survival has improved. Our purpose was to assess median survival after confirmed diagnosis of pemphigus in patients in Kerman, a southern province of Iran. METHODS: All patients who were either admitted to the hospital or treated as outpatients in Kerman from 22 September 1987 to 22 September 1999 and who had confirmed pemphigus were included in the study. Survival was estimated using the Kaplan-Meier method, and the following variables were evaluated in a univariate analysis for an association with survival: age, sex, type of pemphigus, and type of therapy. RESULTS: A total of 55 patients (38 female and 17 male) were identified. No significant differences were found between genders. The mean age at the time of diagnosis was 46.0 years. Older groups had a lower survival rate than younger groups (P < 0.001). The majority (82%) of cases were vulgaris/vegetans, and no significant differences were found in 10-year survival for type of pemphigus (P > 0.05). The patients who had been treated with corticosteroids alone had longer median survival times than those who had been treated with corticosteroids plus azathioprine (P < 0.001). A total of 11 patients died; the median follow-up time for those still alive was 5.9 years (range 2-12 years). Estimated survival at 2, 6 and 10 years was 92.7, 86.8 and 61.5%, respectively. CONCLUSION: Overall median survival rate in patients with pemphigus was 10 years, regardless of gender or subtype of pemphigus. Survival was adversely affected by late onset. Those patients treated with immunosuppressives and corticosteroids also appeared to have reduced survival times when compared to those treated with corticosteroids alone.
