Mortality Rates Among Individuals Diagnosed with Pemphigus: 12-Year Experience in Tehran, Iran.

Background: Pemphigus consists of a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. Pemphigus includes three major forms: pemphigus vulgaris (PV), pemphigus foliaceus, and paraneoplastic pemphigus. Before the advent of systemic corticosteroids (SCSs), pemphigus was usually a fatal disease. Rituximab (RTX), a monoclonal antibody against the CD20+B cells has been approved for the treatment of patients with pemphigus. Previous studies have confirmed the high efficacy and safety profile of RTX in pemphigus patients. We aimed to estimate the overall mortality and causes of death among pemphigus patients who were admitted to the hospitals of Shahid Beheshti University of medical Sciences, Tehran, Iran, before and after administering RTX.

Methods: We included 480 patients admitted to Shahid Beheshti University of medical Sciences hospitals, Tehran, Iran, from October 2010 to October 2022. The diagnosis of all patients was confirmed by direct immunofluorescence and pathological studies. All variables such as age, sex, type of pemphigus, presence of comorbidities, medications, and cause of death were assessed.

Results: The prevalence of pemphigus was 262 (54.58%) in women and 218 (45.41%) in men (P value=0.004). These included 474 (98.75%) PV, 4 (0.83%) pemphigus foliaceus and 2 (0.41%) paraneoplastic pemphigus cases. The most common comorbidities were hypertension and diabetes mellitus (98 [20.41%] and 93 [19.37%], respectively). The overall mortality was 20 (4.16%), including 15 (75%) patients under treatment with high dose SCSs and immunosuppressive agents, and 5 (25%) patients who received at least 500 mg of RTX and low dose SCSs.

Conclusion: The mean age of the disease was found to be a decade earlier than other parts of the world, with a higher preponderance of women. The most common comorbidities were hypertension and diabetes mellitus. Most deaths were due to infectious and cardiovascular diseases. Mortality rate was 1/3 in patients who received RTX compared to those who were treated with high dose corticosteroids and other immunosuppressive agents.