What is the management for hematemesis in a patient with pemphigus?

Management of Hematemesis in Pemphigus

Hematemesis in a patient with pemphigus requires urgent endoscopy to identify esophageal involvement, followed by immediate escalation to high-dose corticosteroids (prednisolone 1-1.5 mg/kg/day or pulsed intravenous methylprednisolone 250-1000 mg) combined with immunosuppressants, as esophageal bleeding responds well to aggressive immunosuppression. 1, 2

Immediate Diagnostic Approach

• Perform urgent esophagogastroduodenoscopy within 36 hours of bleeding onset to identify esophageal involvement of pemphigus, which presents as diffuse mucosal exfoliation with oozing bleeding 2, 3
• Esophageal involvement is underdiagnosed because routine endoscopy is not recommended in asymptomatic patients, but hematemesis mandates immediate evaluation 1
• Obtain biopsies during endoscopy for direct immunofluorescence if the diagnosis of pemphigus is not yet established 4

Acute Treatment Protocol

Corticosteroid Escalation

• Initiate high-dose prednisolone at 1-1.5 mg/kg/day (typically 60-100 mg/day) immediately upon diagnosis of esophageal bleeding 5, 2
• If bleeding persists or disease is rapidly progressive, switch to pulsed intravenous methylprednisolone 250-1000 mg daily for 1-3 days 5, 6
• Both cases in the literature with esophageal bleeding responded to this aggressive corticosteroid approach 2

Concurrent Immunosuppression

• Add or continue a steroid-sparing agent immediately to minimize long-term corticosteroid exposure, which is associated with up to 77% of pemphigus-related deaths 7
• Azathioprine 2-3 mg/kg/day (if TPMT normal) or mycophenolate mofetil 2-3 g/day in divided doses are first-line adjuvants 5, 7
• Expect a 6-8 week latent period before these agents demonstrate clinical effect, so maintain adequate corticosteroid coverage during this time 5, 7

Critical Monitoring During Acute Phase

• Maintain vigilance for infection and sepsis, which are major causes of mortality in pemphigus, especially with escalated immunosuppression 4, 7
• Daily washing with antibacterial products can decrease colonization in patients with extensive mucosal erosions 4
• Consider Pneumocystis jirovecii prophylaxis with trimethoprim/sulfamethoxazole 800/160 mg on alternate days if using cyclophosphamide or triple immunosuppression 5, 6

Pain Management

• Provide adequate analgesia for both acute and maintenance pain control, as esophageal involvement causes significant odynophagia 4
• Pain team consultation may be necessary for severe cases 4

Treatment of Refractory Bleeding

If bleeding persists despite high-dose corticosteroids and conventional immunosuppression:

• Consider rituximab 2 x 1000 mg infusions separated by 2 weeks as third-line therapy, which achieves 89% complete remission at 2 years 5, 8, 6
• Clinical improvement with rituximab typically begins within 6 weeks 8
• Dexamethasone-cyclophosphamide pulse (DCP) therapy is an alternative, with 86% remission rates, though it carries risks of amenorrhea, azoospermia, and hemorrhagic cystitis 4, 5

Common Pitfalls to Avoid

• Do not delay endoscopy in pemphigus patients presenting with hematemesis, as esophageal involvement requires specific treatment escalation 1, 2
• Do not reduce corticosteroids prematurely during the 6-8 week latent period before adjuvant immunosuppressants become effective 5, 7
• Do not undertreat the acute bleeding episode, as both published cases required high-dose corticosteroids and immunosuppressants for resolution 2
• Esophageal involvement may herald disease onset and takes longer to heal than cutaneous lesions 9

Tapering After Bleeding Control

• Once hematemesis resolves and endoscopy shows healing, reduce prednisolone by 5-10 mg weekly initially, tapering more slowly below 20 mg daily 5
• Aim for ≤10 mg daily prednisolone while continuing adjuvant immunosuppression 5, 7
• Implement bone protection measures immediately upon starting high-dose corticosteroids 5, 7