What is the management for a patient with enteritis caused by Henoch-Schonlein purpura (HSP)?

Management of Enteritis Caused by Henoch-Schönlein Purpura

Initial Treatment Approach

Corticosteroids are the first-line therapy for HSP enteritis, with oral prednisone 1-2 mg/kg/day for two weeks recommended to reduce abdominal pain duration and potentially decrease the risk of persistent renal disease. 1

Corticosteroid Therapy Details

• Start oral prednisone at 1-2 mg/kg daily (maximum 60-80 mg/day) for 2 weeks as initial management 1
• A meta-analysis demonstrated that corticosteroids in children reduced the mean time to resolution of abdominal pain 1
• Corticosteroids also decreased the odds of developing persistent renal disease 1
• Most HSP cases (94% in children, 89% in adults) resolve spontaneously, making supportive care the primary intervention alongside steroids 1

Important Caveat About Corticosteroids

• Do not use corticosteroids if severe gastritis or active gastrointestinal bleeding is present, as high-dose steroids may contribute to mucosal injury and perforation risk 2
• Histopathology in one case showed depletion of lymphoid follicles with high-dose corticosteroid use, suggesting steroids may have contributed to intestinal perforation 2

Second-Line Therapy: Intravenous Immunoglobulin (IVIG)

For steroid-refractory cases, severe gastrointestinal involvement, or when steroids are contraindicated due to severe gastritis, IVIG should be administered as second-line therapy. 3, 4

IVIG Treatment Protocol

• IVIG demonstrates complete response within 7 days in 75% (6/8) of severe pediatric HSP cases with gastrointestinal involvement 3
• Partial response occurs in the remaining 25% of cases 3
• IVIG provides an effective alternative to corticosteroids and may be employed as first-line therapy when severe gastritis precludes steroid use 4
• Relapse rate is low (25%), and relapses are typically less severe and respond to a second IVIG dose without further recurrence 3

IVIG Monitoring Considerations

• Monitor for high proteinuria on the day following IVIG infusion, which occurred in 25% (2/8) of patients 3
• Overall tolerance is good despite this potential complication 3
• IVIG induced prompt and sustained resolution in cases of life-threatening gastrointestinal hemorrhage from multiple intestinal sites 4

Third-Line Therapy: Mycophenolate Mofetil

For recurrent, persistent, or steroid-resistant HSP enteritis, mycophenolate mofetil should be considered as an immunosuppressive agent. 5

Mycophenolate Mofetil Indications

• Use in patients with poor response to steroids and recurrent gastrointestinal involvement 5
• Clinical manifestations resolved within days in reported cases 5
• Mycophenolate mofetil is effective for inducing and maintaining remission in persistent, recurrent, or complicated HSP, not just renal involvement 5

Surgical Considerations and Red Flags

Close monitoring with serial abdominal examinations every 4-6 hours is mandatory to detect bowel ischemia before perforation occurs. 2

Indications for Urgent Surgical Consultation

• Hematochezia with persistent localized abdominal tenderness and guarding 2
• Signs of intestinal perforation or peritonitis 2
• Bowel ischemia detected on imaging 2
• Clinical deterioration despite 24-48 hours of appropriate medical therapy 2

Imaging Limitations

• Ultrasonography and contrast-enhanced CT may fail to detect intestinal ischemia prior to perforation 2
• In highly suspicious cases, exploratory laparotomy may be needed for definite diagnosis and prevention of further complications 2
• Intestinal perforation in HSP is rare but life-threatening 2

Supportive Care Measures

• Bowel rest with NPO status until clinical improvement 6
• Intravenous hydration to maintain fluid balance 6
• Correct electrolyte abnormalities aggressively 6
• Hold antimotility agents (loperamide, opiates) until infectious causes are excluded 6

Clinical Presentation to Anticipate

• 60-65% of HSP patients develop abdominal pain 1
• Severe gastrointestinal involvement may include intense pain, digestive bleeding, and protein-losing enteropathy 3
• Atypical presentations exist where gastrointestinal symptoms predominate and classic cutaneous changes may be delayed or absent, leading to diagnostic delay 4
• Adults with HSP are more likely to experience complications than children 1

Long-Term Prognosis Considerations

• Long-term prognosis depends on the severity of renal involvement 1
• End-stage renal disease occurs in 1-5% of patients 1
• 40-50% of HSP patients develop renal disease, necessitating monitoring 1