What is the treatment for lupus enteritis?

Treatment of Lupus Enteritis

The treatment of lupus enteritis requires high-dose glucocorticoids as first-line therapy, typically methylprednisolone pulses (500-750 mg/day for 3 days) followed by oral prednisone, combined with immunosuppressive agents such as mycophenolic acid analogs or cyclophosphamide. 1

Initial Treatment Approach

First-Line Therapy

• Intravenous glucocorticoids:
  • Methylprednisolone 500-750 mg/day for 3 consecutive days 1
  • Follow with oral prednisone 0.5 mg/kg/day, tapering to ≤10 mg/day by 4-6 months 2

Immunosuppressive Agents (choose one)

1. Mycophenolic acid analogs (MPAA):

   • Mycophenolate mofetil (MMF) at target dose of 3 g/day divided into 2-3 doses 2, 1
   • Preferred for patients at high risk of infertility 2

2. Low-dose intravenous cyclophosphamide:

   • Total dose of 3 g over 3 months 2
   • Alternative for patients with difficulty adhering to oral regimens 2

3. Combination therapy options:

   • Belimumab plus either MPAA or cyclophosphamide 2
   • MPAA plus calcineurin inhibitor (when eGFR >45 ml/min/1.73m²) 2

For Severe Lupus Enteritis

• For cases with gastrointestinal hemorrhage or perforation risk:
  • Higher doses of cyclophosphamide may be required 2
  • Consider intravenous immunoglobulin and plasmapheresis for life-threatening cases 3

Adjunctive Therapy

• Hydroxychloroquine: Recommended for all SLE patients unless contraindicated 2, 1
• Supportive care:
  • Bowel rest and intravenous hydration during acute phase 4
  • Monitor for complications such as bowel perforation or hemorrhage 5

Monitoring and Response Assessment

• Expect significant clinical improvement within 2-3 days of starting high-dose steroids 4
• Monitor for:
  • Resolution of abdominal pain and gastrointestinal symptoms
  • Improvement in laboratory markers (complement levels, anti-DNA)
  • Signs of complications (perforation, hemorrhage)

Maintenance Therapy

After achieving remission (typically within 6 months):

• Transition to maintenance therapy with:
  • Lower dose glucocorticoids (aim for ≤5 mg/day prednisone)
  • MPAA or azathioprine for at least 3 years 2
  • Continue hydroxychloroquine indefinitely 1

Special Considerations

Infection Risk

• Screen for tuberculosis, HBV, HCV, and HIV before starting immunosuppression 2
• Consider Pneumocystis jirovecii prophylaxis 2
• Monitor for opportunistic infections, particularly CMV enteritis which can mimic lupus enteritis and cause perforation in immunosuppressed patients 6

Pregnancy Planning

• Switch from mycophenolate or cyclophosphamide to azathioprine if pregnancy is planned 1
• Continue hydroxychloroquine during pregnancy 1

Treatment-Resistant Cases

• For non-responders to initial therapy:
  • Switch between MPAA and cyclophosphamide 2
  • Consider rituximab as rescue therapy 2, 1
  • Evaluate for concomitant intestinal pseudo-obstruction which may require additional management 7

Preventive Measures

• Calcium and vitamin D supplementation to prevent osteoporosis 2
• Limit ultraviolet light exposure and use broad-spectrum sunscreen 2
• Monitor and manage cardiovascular risk factors 2, 1

Early recognition and aggressive treatment of lupus enteritis are essential to prevent serious complications such as bowel necrosis, perforation, and death 5.