Churg-Strauss syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [2]

Overview

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Medical Therapy

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), cytotoxic drugs(eg, 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 seveority and disease activity. These two scoring systems can be helpful in initiating therapy.^[3]^[4]

1 Stage 1 - Name of stage
- 1.1 Specific Organ system involved 1
  - 1.1.1 Adult
    - Preferred regimen (1): drug name 100 mg PO q12h for 10-21 days (Contraindications/specific instructions)
    - Preferred regimen (2): drug name 500 mg PO q8h for 14-21 days
    - Preferred regimen (3): drug name 500 mg q12h for 14-21 days
    - Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
    - Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
    - Alternative regimen (3): drug name 500 mg PO q6h for 14–21 days
  - 1.1.2 Pediatric
    - 1.1.2.1 (Specific population e.g. children < 8 years of age)
      - Preferred regimen (1): drug name 50 mg/kg PO per day q8h (maximum, 500 mg per dose)
      - Preferred regimen (2): drug name 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
      - Alternative regimen (1): drug name10 mg/kg PO q6h (maximum, 500 mg per day)
      - Alternative regimen (2): drug name 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
      - Alternative regimen (3): drug name 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
    - 1.1.2.2 (Specific population e.g. 'children < 8 years of age')
      - Preferred regimen (1): drug name 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
      - Alternative regimen (1): drug name 10 mg/kg PO q6h (maximum, 500 mg per day)
      - Alternative regimen (2): drug name 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
      - Alternative regimen (3): drug name 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
- 1.2 Specific Organ system involved 2
  - 1.2.1 Adult
    - Preferred regimen (1): drug name 500 mg PO q8h
  - 1.2.2 Pediatric
    - Preferred regimen (1): drug name 50 mg/kg/day PO q8h (maximum, 500 mg per dose)

2 Stage 2 - Name of stage
- 2.1 Specific Organ system involved 1
  Note (1):
  
  Note (2):
  
  Note (3):
  - 2.1.1 Adult
    - Parenteral regimen
      - Preferred regimen (1): drug name 2 g IV q24h for 14 (14–21) days
      - Alternative regimen (1): drug name 2 g IV q8h for 14 (14–21) days
      - Alternative regimen (2): drug name 18–24 MU/day IV q4h for 14 (14–21) days
    - Oral regimen
      - Preferred regimen (1): drug name 500 mg PO q8h for 14 (14–21) days
      - Preferred regimen (2): drug name 100 mg PO q12h for 14 (14–21) days
      - Preferred regimen (3): drug name 500 mg PO q12h for 14 (14–21) days
      - Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
      - Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
      - Alternative regimen (3):drug name 500 mg PO q6h for 14–21 days
  - 2.1.2 Pediatric
    - Parenteral regimen
      - Preferred regimen (1): drug name 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
      - Alternative regimen (1): drug name 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
      - Alternative regimen (2): drug name 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '(Contraindications/specific instructions)'
    - Oral regimen
      - Preferred regimen (1): drug name 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
      - Preferred regimen (2): drug name (for children aged ≥ 8 years) 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
      - Preferred regimen (3): drug name 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
      - Alternative regimen (1): drug name 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
      - Alternative regimen (2): drug name 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
      - Alternative regimen (3): drug name 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)
- 2.2 'Other Organ system involved 2'
  Note (1):
  
  Note (2):
  
  Note (3):
  - 2.2.1 Adult
    - Parenteral regimen
      - Preferred regimen (1): drug name 2 g IV q24h for 14 (14–21) days
      - Alternative regimen (1): drug name 2 g IV q8h for 14 (14–21) days
      - Alternative regimen (2): drug name 18–24 MU/day IV q4h for 14 (14–21) days
    - Oral regimen
      - Preferred regimen (1): drug name 500 mg PO q8h for 14 (14–21) days
      - Preferred regimen (2): drug name 100 mg PO q12h for 14 (14–21) days
      - Preferred regimen (3): drug name 500 mg PO q12h for 14 (14–21) days
      - Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
      - Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
      - Alternative regimen (3):drug name 500 mg PO q6h for 14–21 days
  - 2.2.2 Pediatric
    - Parenteral regimen
      - Preferred regimen (1): drug name 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
      - Alternative regimen (1): drug name 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
      - Alternative regimen (2): drug name 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
    - Oral regimen
      - Preferred regimen (1): drug name 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
      - Preferred regimen (2): drug name 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
      - Preferred regimen (3): drug name 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
      - Alternative regimen (1): drug name 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
      - Alternative regimen (2): drug name 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
      - Alternative regimen (3): drug name 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)

References

↑ Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, Dalhoff K, Dunogué B, Gross W, Holle J, Humbert M, Jayne D, Jennette JC, Lazor R, Mahr A, Merkel PA, Mouthon L, Sinico RA, Specks U, Vaglio A, Wechsler ME, Cordier JF, Guillevin L (September 2015). "Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management". Eur. J. Intern. Med. 26 (7): 545–53. doi:10.1016/j.ejim.2015.04.022. PMID 25971154.
↑ Maisch B (February 2015). "[Vasculitis : EULAR/ACR guidelines with respect to the clinical cardiological routine]". Herz (in German). 40 (1): 85–98. doi:10.1007/s00059-014-4200-4. PMID 25676009.
↑ Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P (January 2011). "The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort". Medicine (Baltimore). 90 (1): 19–27. doi:10.1097/MD.0b013e318205a4c6. PMID 21200183.
↑ Yumura W, Kobayashi S, Suka M, Hayashi T, Ito S, Nagafuchi H, Yamada H, Ozaki S (March 2014). "Assessment of the Birmingham vasculitis activity score in patients with MPO-ANCA-associated vasculitis: sub-analysis from a study by the Japanese Study Group for MPO-ANCA-associated vasculitis". Mod Rheumatol. 24 (2): 304–9. doi:10.3109/14397595.2013.854075. PMID 24593206.

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[pmid25971154-1] Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, Dalhoff K, Dunogué B, Gross W, Holle J, Humbert M, Jayne D, Jennette JC, Lazor R, Mahr A, Merkel PA, Mouthon L, Sinico RA, Specks U, Vaglio A, Wechsler ME, Cordier JF, Guillevin L (September 2015). "Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management". Eur. J. Intern. Med. 26 (7): 545–53. doi:10.1016/j.ejim.2015.04.022. PMID 25971154.

[pmid25676009-2] Maisch B (February 2015). "[Vasculitis : EULAR/ACR guidelines with respect to the clinical cardiological routine]". Herz (in German). 40 (1): 85–98. doi:10.1007/s00059-014-4200-4. PMID 25676009.

[pmid21200183-3] Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P (January 2011). "The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort". Medicine (Baltimore). 90 (1): 19–27. doi:10.1097/MD.0b013e318205a4c6. PMID 21200183.

[pmid24593206-4] Yumura W, Kobayashi S, Suka M, Hayashi T, Ito S, Nagafuchi H, Yamada H, Ozaki S (March 2014). "Assessment of the Birmingham vasculitis activity score in patients with MPO-ANCA-associated vasculitis: sub-analysis from a study by the Japanese Study Group for MPO-ANCA-associated vasculitis". Mod Rheumatol. 24 (2): 304–9. doi:10.3109/14397595.2013.854075. PMID 24593206.

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Churg-Strauss syndrome medical therapy

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