Management of Pemphigus Vulgaris
Rituximab combined with short-term corticosteroids is the most effective first-line treatment for pemphigus vulgaris, achieving 89% complete remission at 2 years compared to only 28% with corticosteroids alone. 1
First-Line Treatment Options
Rituximab-Based Regimen
- Initial treatment: Two 1,000 mg intravenous infusions separated by 2 weeks in combination with tapering glucocorticoids 2
- Maintenance: 500 mg intravenous infusion at Month 12 and every 6 months thereafter 2
- For relapse: 1,000 mg intravenous infusion with consideration of resuming or increasing glucocorticoid dose 2
- Important: Subsequent infusions should not be given sooner than 16 weeks after the previous infusion 1, 2
Alternative First-Line Approach (if rituximab unavailable)
- Systemic corticosteroids (prednisone 0.5-1 mg/kg/day) combined with an adjuvant immunosuppressant 1
- Adjuvant options:
- Azathioprine (1-3 mg/kg/day, titrated according to TPMT activity)
- Mycophenolate mofetil (1 g twice daily)
Topical Treatments
- For oral lesions: Betamethasone sodium phosphate 0.5 mg dissolved in 10 mL water as mouthwash 1
- For skin lesions: Clobetasol propionate 0.05% cream twice daily 1
Monitoring and Treatment Adjustment
Clinical Monitoring
- Weekly to biweekly assessment during induction phase
- Monthly assessment during maintenance phase
- Evaluate:
- New blister formation
- Healing of existing lesions
- Mucosal involvement
- Treatment side effects 1
Laboratory Monitoring
- Complete blood count
- Liver and kidney function tests
- Blood glucose
- Lipid profile
- Anti-desmoglein antibody titers (if available) 1
Management of Relapse
- Up to 65% of patients may experience relapse, typically 13-17 months after rituximab 1
- For relapse on rituximab: Administer rituximab 1,000 mg IV and consider resuming or increasing glucocorticoid dose 1, 2
- For relapse on other regimens: Options include increasing steroids back to previous dose, adding an immunosuppressant if using steroid monotherapy, or replacing a first-line immunosuppressant with another if already on combination therapy 3
Additional Treatment Options for Refractory Cases
Intravenous Immunoglobulin (IVIG)
- Dosage: 2 g/kg divided over 3-5 days
- Frequency: Every 2-4 weeks for 1-34 cycles 1
- Has demonstrated corticosteroid-sparing effects 4
Extracorporeal Photopheresis (ECP)
- Consider for recalcitrant cases where conventional therapy fails
- 2-day cycles every 2-4 weeks 1
Prophylaxis and Supportive Care
Infection Prevention
- Pneumocystis jirovecii pneumonia prophylaxis during treatment and for at least 6 months following 1, 2
- Herpes virus prophylaxis during treatment 1, 2
Bone Health
- Calcium and vitamin D supplementation
- Consider bisphosphonates if systemic corticosteroid duration exceeds 3 months 1
Wound Care and Symptom Management
- Antiseptic baths and non-adherent dressings to reduce bacterial infection and pain 1
- For oral lesions: Topical analgesics/anesthetics, antiseptic mouthwashes, and soluble betamethasone sodium phosphate 1
Special Considerations
Pregnancy
- Prednisolone is the first-line systemic agent during pregnancy
- Avoid mycophenolate mofetil, methotrexate, and cyclophosphamide due to teratogenicity
- Azathioprine may be used if necessary (low teratogenic risk)
- IVIG is safe in pregnancy 1
Treatment Outcomes and Prognosis
- Clinical responses to rituximab with short-term corticosteroids typically begin within 6 weeks of treatment 1
- Complete remission rate of 89% at 2 years with rituximab plus corticosteroids versus 34% with corticosteroids alone 1
- Risk of relapse is reduced if direct immunofluorescence is negative (13-27%) compared to positive (44-100%) 1
- Infection and sepsis are major causes of mortality, emphasizing the importance of prophylaxis and careful monitoring 1
Common Pitfalls to Avoid
- Delaying diagnosis and treatment initiation (early intervention improves prognosis) 5
- Inadequate monitoring for treatment side effects
- Tapering corticosteroids too rapidly
- Not providing appropriate prophylaxis against infections and osteoporosis
- Administering rituximab infusions sooner than 16 weeks after previous infusion 1, 2