Guidelines for the Diagnosis and Management of Childhood Immunoglobulin A Vasculitis (2025, Chinese Version, Published in World Journal of Pediatrics)

Citation: Shuai LJ, Zhang QY, Li XZ, et al. Guidelines for the diagnosis and management of childhood immunoglobulin A vasculitis. World J Pediatr. 2025. DOI:10.1007/s12519‑025‑00974‑8 Issuing body: Subspecialty Group of Immunology, Chinese Society of Pediatrics; Chinese Alliance of Pediatric Rheumatic and Immunologic Diseases. Note: This is structured executive summary for academic reference, not substitute for original full‑text guideline or clinical decision‑making.

Abstract

Immunoglobulin A vasculitis (IgAV, formerly Henoch‑Schönlein purpura) is the most common small‑vessel vasculitis in children. Most patients have favourable prognosis, but severe gastrointestinal or renal involvement may cause long‑term sequelae. This guideline was developed following GRADE methodology, covering definition, diagnostic criteria, laboratory‑imaging work‑up, risk stratification, organ‑specific management, follow‑up and prognosis of paediatric IgAV.

1. General Principles

1. Diagnosis is mainly clinical; skin biopsy is not mandatory for typical cases.
2. Core goals: relieve symptoms, prevent life‑threatening gastrointestinal complications, reduce risk of chronic IgAV‑nephritis (IgAVN).
3. Stratified management by organ‑involvement severity.
4. Avoid over‑treatment for uncomplicated skin‑only IgAV.
5. Long‑term urine monitoring is essential, as renal lesions may occur even after rash resolution.

2. Diagnostic Criteria (adopt EULAR/PRINTO/PReS 2008 Ankara criteria)

Mandatory criterion: Palpable purpura (non‑thrombocytopenic), predominantly on lower extremities. Plus at least one of the following:

1. Abdominal pain: diffuse acute pain, may be associated with gastrointestinal bleeding, intussusception.
2. Musculoskeletal manifestation: arthritis / arthralgia.
3. Renal involvement: haematuria, proteinuria.
4. Histopathology: leukocytoclastic vasculitis with dominant IgA deposition in small vessels.

Differential diagnoses: immune thrombocytopenic purpura, other systemic vasculitides, acute glomerulonephritis, sepsis, drug‑induced vasculitis.

3. Initial Evaluation

Baseline laboratory

• Complete blood count, platelet count, coagulation profile, ESR, CRP, serum creatinine, albumin, liver transaminases.
• Urinalysis with microscopy (critical for detecting renal involvement).
• Stool faecal‑occult‑blood test for gastrointestinal screening.
• Imaging: Abdominal ultrasound for severe abdominal pain (rule out intussusception, bowel wall oedema).

Renal biopsy indications (quantified in this guideline)

Perform renal biopsy in children with any of the following:

1. Persistent proteinuria > 1 g/day for more than 4 weeks.
2. Nephrotic‑range proteinuria, nephrotic syndrome.
3. Rapidly progressive glomerulonephritis (increased creatinine, oliguria, persistent gross haematuria).
4. Persistent mixed haematuria‑proteinuria with progressive deterioration of renal function.

4. Organ‑specific Treatment Recommendations

4.1 Isolated cutaneous IgAV

• Supportive care, rest, symptomatic management.
• Routine corticosteroids are NOT recommended for skin‑only disease.

4.2 Musculoskeletal involvement (arthralgia / arthritis)

• Analgesics (paracetamol as first‑line; NSAIDs with caution when gastrointestinal or renal impairment exists).
• Short‑course oral corticosteroids for severe refractory joint pain.

4.3 Gastro‑intestinal involvement

1. Mild abdominal pain: supportive care, enteral nutrition if tolerated.
2. Moderate‑severe abdominal pain, gastrointestinal bleeding: oral prednisone 1‑2 mg/kg/day or intravenous methylprednisolone.
3. Life‑threatening gastrointestinal manifestations (massive bleeding, bowel necrosis, perforation): intravenous pulse methylprednisolone; combined immunosuppression; surgical consultation.

Corticosteroids cannot prevent subsequent IgAV‑nephritis.

4.4 IgAV‑Nephritis (IgAVN)

1. Mild IgAVN (isolated microscopic haematuria, minimal proteinuria): supportive, monitor urine and renal function; ACE‑I / ARB for proteinuria.
2. Moderate IgAVN: proteinuria 0.5‑1 g/d. Consider oral corticosteroids; MMF as steroid‑sparing option.
3. Severe IgAVN (nephrotic syndrome, crescentic glomerulonephritis):
   • Pulse intravenous methylprednisolone followed by oral steroid taper.
   • Mycophenolate mofetil (MMF) is preferred first‑line immunosuppressant.
   • Calcineurin inhibitors (tacrolimus / cyclosporin) for MMF‑intolerant patients.
   • Cyclophosphamide reserved for rapidly progressive severe cases.

4.5 Macrophage‑Activation Syndrome (MAS, life‑threatening complication)

• High‑dose pulse methylprednisolone.
• Cyclosporine A; anakinra may be considered for refractory MAS.
• Monitor ferritin, triglycerides, fibrinogen, blood counts continuously.

5. Follow‑up Protocol

1. Patients without renal signs at onset: perform urinalysis regularly for at least 6 months after disease onset, since nephritis may develop late.
2. Confirmed IgAV‑Nephritis: serial urinalysis, urine protein‑to‑creatinine ratio, serum creatinine; follow‑up for several years according to severity.
3. Relapse monitoring: skin rash recurrence, abdominal symptoms, new‑onset haematuria / proteinuria.

6. Key Updates of 2025 Chinese Guideline

1. Formal adoption of EULAR/PRINTO/PReS 2008 Ankara diagnostic criteria.
2. Quantified, explicit renal‑biopsy indications.
3. Emphasised: corticosteroids do not prevent IgAV‑nephritis.
4. Standardised stratification for IgAV‑nephritis therapy; MMF prioritised over cyclophosphamide for most moderate‑severe IgAVN.
5. Highlighted early recognition and management of MAS.
6. Standardised minimum urine‑surveillance duration (≥6 months) even for initially non‑renal patients.