What is the treatment of choice for gastrointestinal (GI) symptoms in children with Henoch-Schönlein Purpura (HSP)?

Treatment of Gastrointestinal Symptoms in Henoch-Schönlein Purpura (HSP) in Children

Oral corticosteroids are the treatment of choice for severe gastrointestinal symptoms in children with HSP, specifically for severe abdominal pain and gastrointestinal hemorrhage. 1

Initial Management Approach

For children presenting with HSP and GI symptoms, the treatment strategy depends on symptom severity:

• Mild to moderate abdominal pain: Supportive care with analgesics and monitoring is appropriate, as most cases are self-limited with an average disease duration of 4 weeks 1
• Severe abdominal pain or GI bleeding: Oral corticosteroids should be initiated 1
• Life-threatening GI involvement: Escalate to high-dose intravenous methylprednisolone 2

Corticosteroid Therapy

Oral corticosteroids are indicated specifically for severe gastrointestinal pain and gastrointestinal hemorrhage, though they do not alter the overall disease course or prevent nephritis. 1 The evidence shows corticosteroids hasten resolution of abdominal pain but do not prevent recurrences or shorten the overall duration of HSP 2

Key Points About Steroid Use:

• Corticosteroids should not be used prophylactically at HSP onset, as moderate-quality evidence demonstrates they do not prevent renal involvement or decrease risk of severe persistent nephritis 3
• The primary benefit is symptomatic relief of severe GI pain and control of GI bleeding 1, 2

Treatment Escalation for Refractory Cases

When patients fail to respond adequately to oral corticosteroids:

Second-Line: Pulse Methylprednisolone + Cyclophosphamide

• For massive GI involvement refractory to oral prednisolone, administer pulse intravenous methylprednisolone 4, 2
• Add pulse cyclophosphamide if bleeding and severe abdominal pain persist despite high-dose steroids 4

Third-Line: Intravenous Immunoglobulin (IVIG)

• IVIG appears effective for steroid-refractory severe GI involvement, with 6 out of 8 patients showing complete response within 7 days 5
• IVIG is indicated when steroids alone fail to control intense pain, digestive bleeding, or protein-losing enteropathy 5
• Tolerance is generally good, though monitor for proteinuria flare-ups on the day following infusion 5

Fourth-Line: Plasma Exchange

• Reserved for life-threatening GI involvement refractory to steroids, cyclophosphamide, and IVIG 4
• All 7 reported pediatric cases with massive GIS involvement improved after plasma exchange when other therapies failed 4

Critical Pitfalls to Avoid

• Do not delay surgical evaluation: Abdominal symptoms precede the purpuric rash in 14-36% of cases, potentially mimicking an acute surgical abdomen 6
• Use ultrasonography liberally: It is the investigation of choice for detecting complications like intussusception (most common major complication at 4.6% incidence), which is confined to the small bowel in 58% of cases and inaccessible to contrast enema 6
• Do not give prophylactic corticosteroids: This exposes patients to unnecessary steroid side effects without preventing nephritis 3
• Monitor for surgical complications: Intussusception, bowel ischemia/infarction, intestinal perforation, and massive upper GI hemorrhage require prompt surgical intervention 6

Monitoring Requirements

Serial clinical assessment combined with ultrasonography clarifies the nature of GI involvement and reduces unnecessary surgery 6. Earlier diagnosis and prompt treatment of intra-abdominal complications has reduced mortality from 40% to almost zero 6