Churg-Strauss syndrome medical therapy: Difference between revisions

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__NOTOC__
__NOTOC__
{{Churg-Strauss syndrome}}
{{Churg-Strauss syndrome}}
{{CMG}}{{APM}}{{AE}}{{KW}}
{{CMG}}; {{AE}}{{CK}}


==Overview==
==Overview==
Eosinophilic granulomatosis with polyangiitis responds well to treatment with glucocorticoids such as [[prednisone]] when the disease is not in a life threatening state. Patients who present with a life threatening form, are treated with cyclophosphamide together with a glucocorticoid.
There is no treatment for [disease name]; the mainstay of therapy is supportive care.


==Medical Therapy==
OR
Eosinophilic granulomatosis with polyangiitis responds well to treatment with glucocorticoids such as [[prednisone]] when the disease is not in a life threatening state. The dose of glucocorticoid that is given is 1 mg/kg/day for 2-3 weeks. Patients who are unable to taper the dose of glucocorticoid less than 7.5 mg/day for 3 to 4 months. Patients not responding to tapered doses can be given a glucocorticoid-sparing agent. These agents include [[methotrexate]],[[azathioprine]],[[hydroxyurea]], and mycophenolate mofetil.<ref name="pmid15030353">{{cite journal| author=Assaf C, Mewis G, Orfanos CE, Geilen CC| title=Churg-Strauss syndrome: successful treatment with mycophenolate mofetil. | journal=Br J Dermatol | year= 2004 | volume= 150 | issue= 3 | pages= 598-600 | pmid=15030353 | doi=10.1111/j.1365-2133.2003.05807.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15030353  }}</ref> <ref name="pmid25971154">{{cite journal| author=Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V et al.| title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. | journal=Eur J Intern Med | year= 2015 | volume= 26 | issue= 7 | pages= 545-53 | pmid=25971154 | doi=10.1016/j.ejim.2015.04.022 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25971154  }}</ref> <ref name="pmid259711543">{{cite journal| author=Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V et al.| title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. | journal=Eur J Intern Med | year= 2015 | volume= 26 | issue= 7 | pages= 545-53 | pmid=25971154 | doi=10.1016/j.ejim.2015.04.022 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25971154  }}</ref> 
 
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


Patients who present with a life threatening form, are treated with [[cyclophosphamide]] together with a glucocorticoid. The dosage that is given is 1 mg/kg/day for 2-3 weeks and then slowly tapered to a minimal effective dose. Cyclophosphamide is dosed at 2mg/kg/day as an oral or intravenous dose. Cyclophosphamide may also be administered via pulse infusions at 15mg/kg every 2 weeks for the first three infusions, after which cyclophosphamide may be infused every 3 weeks at 15 mg/kg. Cyclophosphamide infusion doses should not exceed 1.2 g. Patients generally receive 6 to 12 pulse infusions of cyclophosphamide.
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].


The maintenance therapy of Eosinophilic granulomatosis with polyangiitis that presents as life threating, patients are treated with azathioprine or methotrexate. Treatment with either of these two agents is given as follows:
OR
{| class="sortable"
!Maintenance therapy
!Azathioprine
!Methotrexate
|-
|
|2 mg/kg/day
|10-30 mg/wk
|}
Either of these treatments can be given after a few days of oral cyclophosphamide or 2 to 3 weeks after pulse administrative doses. The time frame of how long these medications can be administered for is currently unknown; however, 18-24 months of remission have been noted between both of these medications.<ref name="pmid259711542">{{cite journal| author=Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V et al.| title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. | journal=Eur J Intern Med | year= 2015 | volume= 26 | issue= 7 | pages= 545-53 | pmid=25971154 | doi=10.1016/j.ejim.2015.04.022 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25971154  }}</ref> According to Sanders et al, 2005 at 18 months the relapse rate when using azathioprine was 13.3% and after 5 years the relapse rate was 62.3%.<ref>{{cite journal |author=Bosch X, Guilabert A, Espinosa G, Mirapeix E |title=Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review |journal=JAMA |volume=298 |issue=6 |pages=655–69 |year=2007 |pmid=17684188 |doi=10.1001/jama.298.6.655}}</ref>


== Rituximab ==
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
Currently rituximab in patients with Eosinophilic granulomatosis with polyangiitis in not licensed for use and studies are required to test for its effectiveness.


== Intravenous Immunoglobulins ==
==Medical Therapy==
In Eosinophilic granulomatosis with polyangiitis IV immungoglobulins, are used as a second-line therapy. They are administered with glucocorticoids or together with glucocorticoids + immunosuppressive agents, to patients who experience flare-ups due to certain medications they take or during pregnancy.
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
===Disease Name===


== References ==
* '''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 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)
****1.1.2.2 (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  '<nowiki/>'''''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==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Disease]]
[[Category:Pulmonology]]
[[Category:Autoimmune diseases]]
[[Category:Rheumatology]]


{{WH}}
{{WH}}
{{WS}}
{{WS}}
[[Category: (name of the system)]]

Revision as of 18:48, 6 April 2018

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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

  • Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
  • Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
  • Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
  • Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Disease Name

  • 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

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