Churg-Strauss syndrome medical therapy

The mainstay of management for eosinophilic granulomatosis with polyangiitis is glucocorticoids and cyclophosphamide. Immunosupressive agents (eg, azathioprine and methotrexate) can be used for maintenance therapy. Rituximab, interferon-alpha, anti IgE antibodies and plasma exchange can be used as a second line therapies in the management of eosinophilic granulomatosis with polyangiitis.

• Medical therapy for eosinophilic granulomatosis with polyangiitis is according to the guidelines proposed by:^[1][2]
  • EGPA Consensus Task Force recommendations by EULAR (European League Against Rheumatism)
  • American College of Rheumatology (ACR)
• Pharmacologic therapy for EGPA include systemic glucocorticoids(eg, prednisone), immunosupressive agents (eg, cyclophosphamide, azathioprine, methotrexate), inhaled glucocorticoids, IVIG (intravenous immune globulin), anti-IgE (eg, omalizumab), anti-IL-5 antibodies (eg, mepolizumab), and plasma exchange.
• Five factor score(FFS) and Birmingham vasculitis activity score (BVAS) can be used to assess the vasculitis severity and disease activity. These two scoring systems can be helpful in initiating therapy.^[3][4]

• The first line treatment for all the patients with eosinophilic granulomatosis with polyangiitis are systemic glucocorticoids. Most commonly used drug is prednisone.^[1][5][6]
• Preferred regimen:
  • Oral dose of 0.5 to 1.0 mg/kg/day for 2-3 weeks then gradual tapering the dose to 0.3 mg/kg/day after 3 months and 0.15 mg/kg/day after 6 months.
  • Higher doses of corticosteroids can be given in pulses up to 7.5-15mg/kg/day may be required in severe vasculitis involving heart, kidney, and GIT.
• For life-threatening vasculitis, intravenous glucocorticoids can be administered.
  • Preferred regimen: 1 gm/day/3 days followed by treatment with oral glucocorticoids 0.5-1 mg/kg/day.
• Once remission is achieved, dose is gradually tapered over months. Patients who achieve remission will require long term low dose of corticosteroids.
• Most common side effects:
  • Fluid retention
  • Bloating
  • Weight gain
  • Osteoporosis
  • Cataracts and glaucoma

• Cyclophosphamide can be admistered in EGPA patients with severe and life threatening multiple organ involvement in addition to glucocorticoids.^[7][8]
• Preferred regimen:
  • Oral therapy: 2 mg/kg/day
  • Intravenous pulses: For first 3 doses 15 mg/kg/every 2 weeks, for 4-6 doses 15 mg/kg/ every 3 weeks.
• Common side effects:
  • Severe drug induced neutropenia
  • Opportunistic infection
  • Gonadal toxicity
  • Low renal clearance

• Once induction of remission has occured, maintenance therapy with azathioprine or methotrexate can be recommended.^[5]
• Maintenance with cyclophosphamide is associated with frequent relapses.
• Preferred regimen: 10-30 mg/day for 12-18 months.
• Folic acid supplementation is necessary during immunosuppressant therapy.

• Administration of rituximab can be beneficial in ANCA positive patients with progressieve renal failure, CNS involvement, and refractory disease.^[9]

• Second line therapy drug, may be useful for patients who are not responding to glucocorticoids or immunosuppressieve agents^[10]
• Preferred regimen: Subcutaneous administration of 3 million I.U./3 times weekly
• Relapses are more frequent

• Omalizumab may be administered to control asthma, sinusitis and eosinophilia refractory to systemic and inhaled glucocorticoids.^[11]

• Mepolizumab: Preferred regimen: 300mg given every 4 weeks.

• They can be used to relieve symptoms of upper airway disease.

• High doses of intravenous immunoglobulins may be considered in patients whose flare are refractory to standard therapy and during pregnancy.^[12]
• Preferred regimen: 2 g/kg for 2–5-day cycles for every 3-4 weeks.

• The benificial role of plasma exchange in addition to glucocorticoid or immunosuppressieve therapy to improve survival rate in patients with eosinophilic granulomatosis with polyangiitis is unclear.^[13]
• Plasma exchange may be considered for patients with severe and rapidly progressieve renal failure, and diffuse alveolar hemorrhage.^[14]

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