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

Area of Science:

• Immunodermatology
• Autoimmune Diseases
• Clinical Research

Background:

• Pemphigus is a rare group of autoimmune blistering diseases affecting skin and mucous membranes.
• Pemphigus vulgaris (PV), pemphigus foliaceus, and paraneoplastic pemphigus are the main forms.
• Systemic corticosteroids (SCSs) were the primary treatment before the advent of targeted therapies, often with fatal outcomes.

Purpose of the Study:

• To estimate overall mortality and causes of death in pemphigus patients.
• To compare mortality rates before and after the introduction of Rituximab (RTX).
• To analyze patient demographics and comorbidities in a tertiary care setting.

Main Methods:

• Retrospective analysis of 480 pemphigus patients admitted between October 2010 and October 2022.
• Diagnosis confirmed by direct immunofluorescence and pathological studies.
• Assessment of age, sex, pemphigus type, comorbidities, medications, and cause of death.

Main Results:

• Overall mortality was 4.16% (20/480 patients).
• Pemphigus vulgaris constituted 98.75% of cases.
• Patients treated with high-dose SCSs had higher mortality (75%) compared to those receiving RTX (25%).

Conclusions:

• Pemphigus patients in this cohort presented a decade earlier and had a higher prevalence in women compared to global data.
• Hypertension and diabetes mellitus were the most frequent comorbidities.
• Mortality was significantly lower (1/3) in patients treated with RTX compared to high-dose corticosteroids and other immunosuppressants, with infectious and cardiovascular diseases being leading causes of death.