What is the best management approach for a patient with a history of Henoch-Schonlein Purpura (HSP) presenting with gastrointestinal (GI) bleeding?

Management of Henoch-Schönlein Purpura with Gastrointestinal Bleeding

For patients with a history of Henoch-Schönlein Purpura presenting with GI bleeding, immediate hemodynamic assessment and resuscitation take priority, followed by urgent diagnostic evaluation with colonoscopy for stable patients or CT angiography for unstable patients, with corticosteroids as first-line therapy and intravenous immunoglobulin (IVIG) reserved for severe or steroid-refractory cases.

Initial Assessment and Hemodynamic Stabilization

Calculate the shock index (heart rate/systolic blood pressure) immediately upon presentation, with a shock index >1 indicating hemodynamic instability requiring aggressive resuscitation. 1

• Begin fluid resuscitation and blood transfusion concurrently with initial evaluation if hemodynamic instability is present 2
• Use restrictive transfusion thresholds: hemoglobin trigger of 70 g/L (target 70-90 g/L) for patients without cardiovascular disease, or 80 g/L trigger (target 100 g/L) for those with cardiovascular disease 1
• Correct coagulopathy immediately with fresh frozen plasma if INR >1.5 and platelets if platelet count <50,000/µL 1
• Check for orthostatic hypotension, which indicates significant blood loss and necessitates ICU admission 1

Risk Stratification for Stable Patients

For hemodynamically stable patients (shock index ≤1), calculate the Oakland score to guide disposition decisions 1:

• Oakland score ≤8 points: Consider outpatient management with urgent follow-up 1
• Oakland score >8 points: Hospital admission required for inpatient colonoscopy 1

The Oakland score incorporates age, gender, previous lower GI bleeding admission, digital rectal examination findings, heart rate, systolic blood pressure, and hemoglobin level 1.

Diagnostic Approach Based on Hemodynamic Status

For Hemodynamically Unstable Patients (Shock Index >1)

Proceed immediately to CT angiography (CTA) as the first diagnostic step, as it provides the fastest and least invasive means to localize bleeding before therapeutic intervention. 1

• CTA has a 94% positive rate in hemodynamically unstable patients with lower GI bleeding 1
• Following positive CTA, perform catheter angiography with embolization within 60 minutes in centers with 24/7 interventional radiology services 1
• Do not perform colonoscopy as the initial approach when shock index >1 or patients remain unstable after resuscitation 1
• Consider upper endoscopy if no lower GI source is identified, as 10-15% of severe hematochezia cases originate from upper GI sources 2

For Hemodynamically Stable Patients

Colonoscopy is the diagnostic procedure of choice, with diagnostic accuracy of 72-86%. 2

• Perform urgent colonoscopy after rapid bowel cleansing in patients with severe hematochezia 2
• Consider upper endoscopy first if risk factors for peptic ulcer, portal hypertension, or angiodysplasia are present 2
• If colonoscopy is non-diagnostic and bleeding continues, proceed to radionuclide imaging (99Tcm-labeled red blood cell scan) followed by urgent angiography within 1 hour if positive 2

HSP-Specific Medical Management

First-Line Therapy: Corticosteroids

Corticosteroids are the first-line therapy for severe GI involvement in HSP. 3, 4

• Initiate intravenous methylprednisolone 500 mg daily (or weight-based equivalent in pediatric patients) followed by a steroid taper 3
• Monitor for clinical response within 48-72 hours 3

Second-Line Therapy: Intravenous Immunoglobulin (IVIG)

IVIG should be used as second-line therapy for steroid-refractory cases or as first-line therapy when severe gastritis precludes corticosteroid use. 5, 4

• IVIG induces prompt and sustained resolution in severe intestinal HSP, with 6 out of 8 patients showing complete response within 7 days in one series 4
• IVIG provides an effective alternative when significant gastritis contraindicates corticosteroids 5
• Two important caveats: 2 out of 8 patients may relapse requiring a second dose (though with less severe involvement), and 2 out of 8 may develop high proteinuria on the day following IVIG infusion 4

Third-Line Therapy: Additional Immunosuppression

For incomplete clinical response to steroids alone, add mycophenolate mofetil 1000 mg twice daily and maintain for three months 3.

Interventional Management

Endoscopic Therapy

• Perform therapeutic intervention during colonoscopy if a bleeding source is identified 2
• If continued bleeding occurs despite endoscopic intervention, proceed to angiographic embolization 2

Angiographic Embolization

• Transcatheter embolization provides time to stabilize the patient and prepare the bowel, contributing to better surgical outcomes if surgery ultimately becomes necessary 1
• This approach is preferred over blind segmental resection, which carries rebleeding rates as high as 33% and mortality of 33-57% 1

Surgical Intervention

Surgery should be reserved only for patients who fail angiographic intervention or continue to deteriorate despite all attempts at localization and intervention. 1

• Intraoperative peroral enteroscopy can locate bleeding sources in the small bowel that are not accessible by standard endoscopy 6
• Resection of the affected bowel segment is curative once the bleeding source is localized 6
• Avoid blind segmental resection and emergency subtotal colectomy due to substantial rebleeding rates (33%) and mortality (33-57%) 1

Critical Pitfalls and Caveats

Failure to consider an upper GI source in patients with severe hematochezia and hemodynamic instability can lead to delayed diagnosis and treatment. 2, 1

• HSP can present with isolated GI symptoms before the classic purpuric rash appears, leading to significant diagnostic delay 5
• Secondary infections (such as norovirus) can precipitate worsening of underlying HSP vasculitis and lead to acute clinical decompensation, even after initial improvement 7
• Mortality in lower GI bleeding is generally related to comorbidity rather than exsanguination, with overall in-hospital mortality of 3.4%, but rising to 20% in patients requiring ≥4 units of red cells 1
• The operative mortality rate for emergency surgery is 10%, with mortality rates for total abdominal colectomy ranging from 27-33% 1
• When GI manifestations precede the classic purpuric rash, skin biopsy showing IgA deposition within superficial dermal vessels remains the gold standard for confirming HSP diagnosis 3