Table 5.
Therapeutic approach for vasculitis according to the severity of the manifestations.
| Clinical severity | Findings | Therapeutic approach |
|---|---|---|
| Mild vasculitis limited to the skin | Persistent or recurrent vasculitis and/or symptomatic diseases (burning or pruritus) | Dapsone or colchicine (useful for mild disease, IgAV, UV or Behçet's disease; both drugs have better therapeutic effects after two to four weeks); |
| Dapsone 25–50 mg/day orally at the beginning of treatment. After a complete blood count and liver function laboratory evaluation with normal results, the dosage may be increased to 100–200 mg/day; | ||
| Colchicine: 0.5 mg, two to three times daily, orally; | ||
| Other agents: pentoxifylline 400 mg, three times daily (Behçet's disease or mild cutaneous arteritis). | ||
| Moderate to severe cutaneous vasculitis | Extensive skin involvement, persistent vasculitis, recurrent vasculitis with ulcers, nodules or recalcitrant symptoms | Methotrexate, azathioprine, and/or prednisone (cutaneous arteritis, mild vasculitis limited to the skin, rheumatoid vasculitis, lupus vasculitis); |
| Methotrexate: 5–20 mg/week; | ||
| Azathioprine (AZA; PNS involvement, renal involvement): TPMT (levels); TPMT < 5, not recommended; TPMT 5–13.7 U (AZA 0.5 mg/kg/day); TPMT 13.7–19 U (AZA 1.5 mg/kg/day); TPMT > 19 U (AZA 2.5 mg/kg/day). | ||
| Prednisone: 1–1.5 mg/kg/day orally; | ||
| Hydroxychloroquine: 400 mg/day orally; | ||
| Cyclosporine 2.5–5.0 mg/kg/day orally; | ||
| Cyclophosphamide (extensive skin and ulcerative vasculitis or PNS involvement, or renal involvement): 100 mg/day to 2000 mg/day, orally. | ||
| Systemic vasculitis | Potentially fatal disease or permanent organ damage, serum creatinine levels >150 μmoL/L (severe if >500 μmoL/L) | Prednisone ± cyclophosphamide or azathioprine or cyclosporine or mofetil mycophenolate (AAV, PAN, severe IgAV, lupus vasculitis, large vessel vasculitis); |
| Methylprednisolone (intravenous) 1000 mg/day for three consecutive days, then prednisone 1.0–1.5 mg/kg/day orally; | ||
| Mofetil mycophenolate: 2000 mg/day (HUV, AAV). | ||
IgAV, IgA vasculitis; HUV, hypocomplementemic urticarial vasculitis; PAN, polyarteritis nodosa; TPMT, thiopurine S-methyltransferase; AAV, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis.