Determination of survival and hazard functions for pemphigus patients in Kerman, a southern province of Iran

Abstract

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.

Epidemiology of Pemphigus in Tehran, Iran: A 20-Year Retrospective Study

“…A similar observation regarding age has been reported from other studies in Khuzestan 6 (42.5 years), Tehran 10 (42 years), and Kerman 14 (46 years). It is important to mention that the mean age of patients with pemphigus in the other parts of the world is higher than the present study (Hietanen & Salo 12 , 57 years; Qasem et al 15 , 63 years).…”

“…Many researchers considered that there is a fairly strong genetic background to pemphigus vulgaris. 6 , 10 , 14 …”

Pemphigus Vulgaris and Pemphigus Foliaceus: Differences in Epidemiology and Mortality

“…Pemphigus is associated with a relatively high mortality rate after diagnosis, ranging in the literature from 5% to 30% during various lengths of follow-up (9)(10)(11)(25)(26)(27)(28). Of note, the vast majority of the survival data reported among pemphigus patients did not differentiate between the different variants of the disease as distinct entities.…”

“…The mainstay treatments for pemphigus are systemic corticosteroids and immunosuppressive therapy. The prognosis for patients with pemphigus has improved greatly since the introduction of corticosteroid therapy; nevertheless, pemphigus remains a potentially life-threatening disease (6), with a relatively high mortality rate after diagnosis, ranging from 5% to 30% during various lengths of follow-up (9)(10)(11). In 2 recent studies from the UK and Taiwan, the risk of death was calculated as 2-to-3-fold higher than in the control or general population (12,13).…”

Pemphigus vulgaris: approach to treatment

“…With proper treatment, the mortality rate of PV has been drastically reduced, but still stands at an unacceptable rate of approximately 5-6%, with most deaths being the result of side effects of immunosuppressive agents rather than the result of the disease itself or disease sequelae [2][3][4]. The only known factor that significantly reduces survival rates is a later age of onset of PV [5]. A poorer prognosis for achieving complete remission is seen when the disease is more widespread, more severe at onset, or when it responds slowly to therapy [4].…”