What is the role of steroids in treating vasculitis?

Steroids Are Essential First-Line Therapy for Vasculitis

Steroids (glucocorticoids) are the cornerstone of initial treatment for all forms of vasculitis and should be initiated immediately upon diagnosis, with high-dose therapy (40-60 mg/day prednisone-equivalent or 1 mg/kg/day) used for induction of remission, always combined with immunosuppressive agents for severe disease. 1, 2

Immediate Initiation Strategy

• Start high-dose glucocorticoids immediately without waiting for biopsy confirmation in rapidly deteriorating patients, particularly those with ANCA-associated vasculitis, as diagnostic delays can result in irreversible organ damage 3, 2
• For severe presentations (rapidly progressive glomerulonephritis, pulmonary hemorrhage, or critical organ involvement), administer intravenous methylprednisolone 1-3 g daily for 3-5 days as pulse therapy before transitioning to oral steroids 1, 3
• Following pulse therapy, initiate oral prednisone 1 mg/kg/day (maximum 60 mg/day) for all vasculitis types 1, 2

Disease-Specific Steroid Protocols

ANCA-Associated Vasculitis

• Never use steroids as monotherapy—always combine with either rituximab or cyclophosphamide for induction 1, 2
• The 2024 KDIGO guidelines demonstrate that reduced-dose glucocorticoids (0.5 mg/kg/day) are noninferior to high-dose (1 mg/kg/day) when combined with rituximab in patients without severe glomerulonephritis (eGFR >15 ml/min/1.73m²) or life-threatening alveolar hemorrhage 1
• Taper to 0.2 mg/kg/day (maximum 10 mg/day) by month 6, with the PEXIVAS trial showing that rapid reduction is as effective but safer than standard tapering in patients with GFR <50 ml/min/1.73m² 1

Large Vessel Vasculitis (Giant Cell Arteritis/Takayasu Arteritis)

• Initiate prednisone 40-60 mg/day (or 1 mg/kg/day) immediately to prevent blindness in GCA or vascular complications in Takayasu arteritis 1, 2
• Maintain high-dose therapy for one month before gradual tapering 2
• Add steroid-sparing agents early: tocilizumab is recommended for GCA patients with refractory disease, relapsing disease, or high risk for glucocorticoid-related adverse events; methotrexate is an alternative 1
• For Takayasu arteritis, all patients should receive non-biological glucocorticoid-sparing agents in combination with glucocorticoids from the outset 1, 2

Anti-GBM Disease

• Requires immediate high-dose corticosteroids plus plasmapheresis and cyclophosphamide without delay once diagnosis is confirmed 1
• Start treatment before confirmation if diagnosis is highly suspected, as outcomes are time-dependent 1

Critical Steroid-Sparing Strategies

Glucocorticoids are major contributors to adverse events, making steroid-sparing approaches essential. 1

• Avacopan (C5a receptor antagonist) 30 mg twice daily is an effective alternative to prednisone in ANCA-associated vasculitis, achieving 72.3% remission versus 70.1% with prednisolone, with potential for improved kidney outcomes and fewer infections 1
• Avacopan was excluded from trials involving patients with eGFR <15 ml/min/1.73m² or alveolar hemorrhage requiring mechanical ventilation, so use traditional glucocorticoids in these severe presentations 1
• Rituximab allows for lower cumulative glucocorticoid exposure compared to cyclophosphamide-based regimens 1

Maintenance Phase Steroid Management

• Continue glucocorticoid maintenance at 5-7.5 mg/day for 2 years, then reduce by 1 mg every 2 months 2
• Maintenance therapy should continue for at least 18-24 months for ANCA-associated vasculitis to prevent relapse 1, 2
• Discontinue maintenance immunosuppression in dialysis-dependent patients without extrarenal manifestations after 3 months 1

Common Pitfalls and Safety Considerations

• Never use alternate-day glucocorticoid therapy—this increases relapse risk 2
• Provide prophylaxis against Pneumocystis jiroveci pneumonia for all patients on combination immunosuppression 3, 2
• Implement osteoporosis prophylaxis in all patients receiving prolonged glucocorticoid therapy 2
• The most serious adverse events from steroids include infections (particularly with pulse therapy), diabetes, osteoporosis, and cardiovascular complications 1
• In mixed cryoglobulinemia syndrome, high-dose pulse glucocorticoid therapy is useful for critical flares (renal/neurological complications), but chronic low-dose therapy should be avoided due to serious irreversible side effects 1

Monitoring During Steroid Therapy

• Monitor for infections aggressively, as they are more common and severe with high-dose glucocorticoids, particularly when combined with rituximab or cyclophosphamide 1, 3
• Track ANCA levels during treatment—persistence or increase may predict future relapse, though treatment changes should not be based on ANCA titers alone 1, 2
• Assess for steroid-related complications: hyperglycemia, hypertension, weight gain, mood changes, and bone density loss 1