What is the initial treatment recommendation for a patient with chronic IgA (Immunoglobulin A) vasculitis?

Treatment of Chronic IgA Vasculitis

For chronic IgA vasculitis in adults, initiate treatment with glucocorticoids (prednisone 1 mg/kg/day, maximum 60-80 mg/day) as first-line therapy, and add immunosuppressive agents (cyclophosphamide or rituximab) when severe organ-threatening manifestations are present, particularly glomerulonephritis with proteinuria >1 g/day or rapidly progressive renal dysfunction. 1

Disease Severity Stratification

The treatment approach depends critically on whether you are dealing with organ-threatening disease versus limited manifestations:

Severe/organ-threatening disease includes: 1

• Glomerulonephritis with rapidly declining renal function
• Proteinuria >1 g/day with progressive renal dysfunction
• Severe gastrointestinal involvement (bleeding, intussusception)
• Pulmonary hemorrhage (rare but life-threatening)

Limited disease includes: 2, 3

• Isolated cutaneous purpura lasting >6 weeks
• Mild arthralgia without functional impairment
• Mild gastrointestinal symptoms without bleeding

Glucocorticoid Protocol for Severe Disease

Initial dosing: Start prednisone at 1 mg/kg/day (maximum 60-80 mg/day) for approximately one month before initiating taper. 1

Structured taper schedule over 4-5 months: 1

• Weeks 2-4: Reduce to 20-30 mg/day
• Months 2-3: Reduce to 15-20 mg/day
• Months 4-5: Reduce to 5 mg/day
• Target maintenance dose of 7.5-10 mg by 3 months, though this may take up to 5 months in practice 4

Immunosuppressive Therapy Selection

Add immunosuppressive agents when: 1

• Glomerulonephritis with proteinuria >1 g/day
• Rapidly progressive renal dysfunction
• Severe gastrointestinal manifestations

Cyclophosphamide Option

• Dose: 2 mg/kg/day (maximum 200 mg/day) for 3-6 months 1
• Critical supportive care: Administer Pneumocystis jirovecii prophylaxis with trimethoprim/sulfamethoxazole (800/160 mg on alternate days or 400/80 mg daily) 4, 1
• Bladder protection: Encourage high fluid intake or give IV fluids on treatment days; consider MESNA to prevent hemorrhagic cystitis 4
• Major limitation: Causes reduced ovarian reserve, ovarian failure, and male infertility 4, 5

Rituximab Option (Emerging as Preferred Alternative)

• Induction regimen: 375 mg/m² IV weekly for 4 weeks OR 1,000 mg on days 1 and 15 1, 5
• Advantages over cyclophosphamide: 5
  • Preserves reproductive potential (no reported fertility concerns)
  • Superior for relapsing disease
  • Preferred in women of childbearing age
• Required monitoring: Pneumocystis prophylaxis with trimethoprim/sulfamethoxazole; monitor immunoglobulin levels every 6 months (hypogammaglobulinemia occurs in 27-58% of patients) 6, 5

Rituximab is particularly preferred when: 6, 5

• Patient wishes to preserve fertility
• Relapsing disease pattern
• Cyclophosphamide poses specific risks or contraindications

Treatment for Limited/Chronic Cutaneous Disease

For chronic cutaneous manifestations lasting >6 weeks without organ involvement:

Dapsone: 1-2 mg/kg once daily for at least 1 week, showing good effectiveness for persistent purpuric lesions 7

• Caveat: Relapse occurs frequently after discontinuation but responds to repeat treatment 7
• Monitor for: Methemoglobinemia 7

Alternative agents for minor manifestations: 8

• Colchicine
• Methotrexate
• These can be useful for controlling symptoms without severe organ involvement

Renal-Specific Management

For patients with proteinuria >1 g/day: 1

• Add ACE inhibitors or ARBs for blood pressure control and proteinuria reduction
• Target blood pressure <125/75 mm Hg

For severe crescentic glomerulonephritis: Consider plasma exchange in addition to glucocorticoids and immunosuppression, though evidence is limited 1

The combination of prednisolone with cyclophosphamide or azathioprine has demonstrated healing of vasculitic lesions and may arrest progression of glomerular scarring in IgA nephropathy with crescentic features. 9

Critical Monitoring Parameters

Do not use laboratory markers alone for disease monitoring. 1

• Perform structured clinical assessment with urinalysis at each visit
• Monitor renal function, proteinuria, and blood pressure regularly
• Assess for gastrointestinal symptoms and cutaneous manifestations

Bone Protection

Consider bone protection therapy for all patients on prolonged glucocorticoid treatment to prevent osteoporosis. 1

Prognosis Considerations

Adults have worse outcomes than children: 2, 3

• Higher frequency of glomerulonephritis (more common and severe)
• Up to one-third of adult patients may progress to end-stage renal failure
• Short-term outcome depends on severity of gastrointestinal manifestations
• Long-term prognosis depends heavily on presence and severity of nephritis

Key pitfall: Self-limited disease requires only symptomatic treatment, but severe manifestations (especially renal) demand aggressive immunosuppression to prevent progression to end-stage renal disease. 2, 3