Churg-Strauss syndrome medical therapy: Difference between revisions

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==Overview==
==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].


The mainstay of management for [[eosinophilic granulomatosis with polyangiitis]] is [[glucocorticoids]] and [[cyclophosphamide]]. [[Immunosuppressive drug|Immunosupressive agents]] (eg, [[azathioprine]] and [[methotrexate]]) can be used for maintenance therapy. [[Rituximab]], [[interferon-alpha]], anti IgE antibodies and [[Plasmapheresis|plasma exchange]] can be used as a second line therapies in the management of [[Eosinophilic granulomatosis with polyangiitis|eosinophilic granulomatosis with polyangiitis.]]
==Medical Therapy==
==Medical Therapy==
*Medical therapy for eosinophilic granulomatosis with polyangiitis is according to the guidelines proposed by:<ref name="pmid25971154">{{cite journal |vauthors=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 |title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management |journal=Eur. J. Intern. Med. |volume=26 |issue=7 |pages=545–53 |date=September 2015 |pmid=25971154 |doi=10.1016/j.ejim.2015.04.022 |url=}}</ref><ref name="pmid25676009">{{cite journal |vauthors=Maisch B |title=[Vasculitis : EULAR/ACR guidelines with respect to the clinical cardiological routine] |language=German |journal=Herz |volume=40 |issue=1 |pages=85–98 |date=February 2015 |pmid=25676009 |doi=10.1007/s00059-014-4200-4 |url=}}</ref>
*Medical therapy for [[eosinophilic granulomatosis with polyangiitis]] is according to the guidelines proposed by:<ref name="pmid25971154">{{cite journal |vauthors=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 |title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management |journal=Eur. J. Intern. Med. |volume=26 |issue=7 |pages=545–53 |date=September 2015 |pmid=25971154 |doi=10.1016/j.ejim.2015.04.022 |url=}}</ref><ref name="pmid25676009">{{cite journal |vauthors=Maisch B |title=[Vasculitis : EULAR/ACR guidelines with respect to the clinical cardiological routine] |language=German |journal=Herz |volume=40 |issue=1 |pages=85–98 |date=February 2015 |pmid=25676009 |doi=10.1007/s00059-014-4200-4 |url=}}</ref>
**EGPA Consensus Task Force recommendations by EULAR (European League Against Rheumatism)
**EGPA Consensus Task Force recommendations by EULAR (European League Against Rheumatism)
**American College of Rheumatology (ACR)
**American College of Rheumatology (ACR)
*Pharmacologic therapy for EGPA include oral 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.
*Pharmacologic therapy for EGPA include systemic [[glucocorticoids]](eg, [[prednisone]]), [[Immunosuppressive drug|immunosupressive agents]] (eg, [[cyclophosphamide]], [[azathioprine]], [[methotrexate]]), inhaled [[glucocorticoids]], [[Intravenous immunoglobulin|IVIG (intravenous immune globulin)]], anti-IgE (eg, [[omalizumab]]), anti-IL-5 antibodies (eg, [[mepolizumab]]), and plasma exchange.
===Disease Name===
* 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.<ref name="pmid21200183">{{cite journal |vauthors=Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P |title=The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort |journal=Medicine (Baltimore) |volume=90 |issue=1 |pages=19–27 |date=January 2011 |pmid=21200183 |doi=10.1097/MD.0b013e318205a4c6 |url=}}</ref><ref name="pmid24593206">{{cite journal |vauthors=Yumura W, Kobayashi S, Suka M, Hayashi T, Ito S, Nagafuchi H, Yamada H, Ozaki S |title=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 |journal=Mod Rheumatol |volume=24 |issue=2 |pages=304–9 |date=March 2014 |pmid=24593206 |doi=10.3109/14397595.2013.854075 |url=}}</ref>
 
==Initiating pharmacotherapy==
* '''1 Stage 1 - Name of stage'''
===Systemic glucocorticosteroids===
** 1.1 '''Specific Organ system involved 1'''
*The first line treatment for all the patients with [[eosinophilic granulomatosis with polyangiitis]] are systemic [[glucocorticoids]]. Most commonly used drug is [[prednisone]].<ref name="pmid25971154">{{cite journal |vauthors=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 |title=Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management |journal=Eur. J. Intern. Med. |volume=26 |issue=7 |pages=545–53 |date=September 2015 |pmid=25971154 |doi=10.1016/j.ejim.2015.04.022 |url=}}</ref><ref name="pmid18240234">{{cite journal |vauthors=Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Lauque D, Puéchal X, Letellier P, Delaval P, Cordier JF, Guillevin L |title=Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients |journal=Arthritis Rheum. |volume=58 |issue=2 |pages=586–94 |date=February 2008 |pmid=18240234 |doi=10.1002/art.23198 |url=}}</ref><ref name="pmid22887848">{{cite journal |vauthors=Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, Matthis C, Metzler C, Nölle B, Richardt G, Gross WL |title=A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients |journal=Ann. Rheum. Dis. |volume=72 |issue=6 |pages=1011–7 |date=June 2013 |pmid=22887848 |doi=10.1136/annrheumdis-2012-201531 |url=}}</ref>
*** 1.1.1 '''Adult'''
*Preferred regimen:
**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)''' 
**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.
**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
**Higher doses of [[Corticosteroid|corticosteroids]] can be given in pulses up to 7.5-15mg/kg/day may be required in severe [[vasculitis]] involving [[heart]], [[kidney]], and [[Gastrointestinal tract|GIT]].
**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
*For life-threatening [[vasculitis]], intravenous [[glucocorticoids]] can be administered.
**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
**Preferred regimen: 1 gm/day/3 days followed by treatment with oral [[glucocorticoids]] 0.5-1 mg/kg/day.
**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
*Once remission is achieved, dose is gradually tapered over months. Patients who achieve remission will require long term low dose of [[Corticosteroid|corticosteroids]].
**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
*Most common [[Adverse effect (medicine)|side effects]]:
*** 1.1.2 '''Pediatric'''
**[[Water retention|Fluid retention]]
**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
**[[Bloating]]
***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose) 
**[[Weight gain]]
***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
**[[Osteoporosis]]
***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
**[[Cataract|Cataracts]] and [[glaucoma]]
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
===Cyclophosphamide===
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
*[[Cyclophosphamide]] can be admistered in EGPA patients with severe and life threatening multiple organ involvement in addition to [[glucocorticoids]].<ref name="pmid1676753">{{cite journal |vauthors=Guillevin L, Jarrousse B, Lok C, Lhote F, Jais JP, Le Thi Huong Du D, Bussel A |title=Longterm followup after treatment of polyarteritis nodosa and Churg-Strauss angiitis with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange. A prospective randomized trial of 71 patients. The Cooperative Study Group for Polyarteritis Nodosa |journal=J. Rheumatol. |volume=18 |issue=4 |pages=567–74 |date=April 1991 |pmid=1676753 |doi= |url=}}</ref><ref name="pmid14680433">{{cite journal |vauthors=Hellmich B, Gross WL |title=Recent progress in the pharmacotherapy of Churg-Strauss syndrome |journal=Expert Opin Pharmacother |volume=5 |issue=1 |pages=25–35 |date=January 2004 |pmid=14680433 |doi=10.1517/14656566.5.1.25 |url=}}</ref>
****1.1.2.2 (Specific population e.g. ''''''children < 8 years of age'''''')
*Preferred regimen:
***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
**Oral therapy: 2 mg/kg/day
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
**[[Intravenous therapy|Intravenous pulses]]: For first 3 doses 15 mg/kg/every 2 weeks, for 4-6 doses 15 mg/kg/ every 3 weeks.  
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose) 
*Common [[Adverse effect (medicine)|side effects]]:
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
**Severe drug induced [[neutropenia]]
** 1.2 '''Specific Organ system involved 2'''
**[[Opportunistic infection]]
*** 1.2.1 '''Adult'''
**Gonadal toxicity
**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
**Low [[Clearance (medicine)|renal clearance]]
*** 1.2.2  '''Pediatric'''
==Maintenance therapy==
**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)
*Once induction of [[Remission (medicine)|remission]] has occured, maintenance therapy with [[azathioprine]] or [[methotrexate]] can be recommended.<ref name="pmid18240234">{{cite journal |vauthors=Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Lauque D, Puéchal X, Letellier P, Delaval P, Cordier JF, Guillevin L |title=Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients |journal=Arthritis Rheum. |volume=58 |issue=2 |pages=586–94 |date=February 2008 |pmid=18240234 |doi=10.1002/art.23198 |url=}}</ref>
 
*Maintenance with [[cyclophosphamide]] is associated with frequent [[Relapse|relapses]].
* 2 '''Stage 2 - Name of stage'''
*Preferred regimen: 10-30 mg/day for 12-18 months.
** 2.1 '''Specific Organ system involved 1 '''
*[[Folic Acid|Folic acid]] supplementation is necessary during immunosuppressant therapy.
**: '''Note (1):'''
==Additional therapy==
**: '''Note (2)''':
===Rituximab===
**: '''Note (3):'''
*Administration of [[rituximab]] can be beneficial in ANCA positive patients with progressieve [[Renal insufficiency|renal failure]], CNS involvement, and refractory disease.<ref name="pmid19740901">{{cite journal |vauthors=Saech J, Owczarczyk K, Owczarzyk K, Rösgen S, Petereit H, Hallek M, Rubbert-Roth A |title=Successful use of rituximab in a patient with Churg-Strauss syndrome and refractory central nervous system involvement |journal=Ann. Rheum. Dis. |volume=69 |issue=6 |pages=1254–5 |date=June 2010 |pmid=19740901 |doi=10.1136/ard.2009.109850 |url=}}</ref>
*** 2.1.1 '''Adult'''
===Interferon-alpha===
**** Parenteral regimen
*Second line therapy drug, may be useful for patients who are not responding to [[glucocorticoids]] or immunosuppressieve agents<ref name="pmid18799051">{{cite journal |vauthors=Metzler C, Schnabel A, Gross WL, Hellmich B |title=A phase II study of interferon-alpha for the treatment of refractory Churg-Strauss syndrome |journal=Clin. Exp. Rheumatol. |volume=26 |issue=3 Suppl 49 |pages=S35–40 |date=2008 |pmid=18799051 |doi= |url=}}</ref>
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
*Preferred regimen: Subcutaneous administration of 3 million I.U./3 times weekly
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
*Relapses are more frequent
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
===Anti-IgE therapy===
**** Oral regimen
*[[Omalizumab]] may be administered to control [[asthma]], [[Rhinosinusitis|sinusitis]] and [[eosinophilia]] refractory to systemic and [[Glucocorticoids|inhaled glucocorticoids]].<ref name="pmid26946346">{{cite journal |vauthors=Jachiet M, Samson M, Cottin V, Kahn JE, Le Guenno G, Bonniaud P, Devilliers H, Bouillet L, Gondouin A, Makhlouf F, Meaux-Ruault N, Gil H, Bienvenu B, Coste A, Groh M, Giraud V, Dominique S, Godeau B, Puéchal X, Khouatra C, Ruivard M, Le Jeunne C, Mouthon L, Guillevin L, Terrier B |title=Anti-IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss): Data on Seventeen Patients |journal=Arthritis Rheumatol |volume=68 |issue=9 |pages=2274–82 |date=September 2016 |pmid=26946346 |doi=10.1002/art.39663 |url=}}</ref>
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
===Anti-IL-5 antibodies===
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
*[[Mepolizumab]]: Preferred regimen: 300mg given every 4 weeks.
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
===Inhaled glucocorticoids===
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
*They can be used to relieve symptoms of upper airway disease.
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
===Intravenous immunoglobulins===
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
* High doses of [[Intravenous immunoglobulin|intravenous immunoglobulins]] may be considered in patients whose flare are refractory to standard therapy and during pregnancy.<ref name="pmid15547090">{{cite journal |vauthors=Danieli MG, Cappelli M, Malcangi G, Logullo F, Salvi A, Danieli G |title=Long term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome |journal=Ann. Rheum. Dis. |volume=63 |issue=12 |pages=1649–54 |date=December 2004 |pmid=15547090 |pmc=1754837 |doi=10.1136/ard.2003.015453 |url=}}</ref>
*** 2.1.2 '''Pediatric'''
*Preferred regimen: 2 g/kg for 2–5-day cycles for every 3-4 weeks.
**** Parenteral regimen
===Plasma exchange===
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
*The benificial role of plasma exchange in addition to [[glucocorticoid]] or [[Immunosuppresive drug|immunosuppressieve therapy]] to improve survival rate in patients with [[eosinophilic granulomatosis with polyangiitis]] is unclear.<ref name="pmid9255326">{{cite journal |vauthors=Guillevin L, Cevallos R, Durand-Gasselin B, Lhote F, Jarrousse B, Callard P |title=Treatment of glomerulonephritis in microscopic polyangiitis and Churg-Strauss syndrome. Indications of plasma exchanges, Meta-analysis of 2 randomized studies on 140 patients, 32 with glomerulonephritis |journal=Ann Med Interne (Paris) |volume=148 |issue=3 |pages=198–204 |date=1997 |pmid=9255326 |doi= |url=}}</ref>
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
*Plasma exchange may be considered for patients with severe and rapidly progressieve [[Renal insufficiency|renal failure]], and diffuse alveolar hemorrhage.<ref name="pmid14655185">{{cite journal |vauthors=Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ |title=Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis |journal=Am. J. Kidney Dis. |volume=42 |issue=6 |pages=1149–53 |date=December 2003 |pmid=14655185 |doi= |url=}}</ref>
***** 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==
==References==

Latest revision as of 18:06, 12 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

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

Initiating pharmacotherapy

Systemic glucocorticosteroids

Cyclophosphamide

Maintenance therapy

Additional therapy

Rituximab

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

Interferon-alpha

  • 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

Anti-IgE therapy

Anti-IL-5 antibodies

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

Inhaled glucocorticoids

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

Intravenous immunoglobulins

  • 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.

Plasma exchange

References

  1. ↑ 1.0 1.1 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.
  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.
  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.
  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.
  5. ↑ 5.0 5.1 Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Lauque D, Puéchal X, Letellier P, Delaval P, Cordier JF, Guillevin L (February 2008). "Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients". Arthritis Rheum. 58 (2): 586–94. doi:10.1002/art.23198. PMID 18240234.
  6. ↑ Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, Matthis C, Metzler C, Nölle B, Richardt G, Gross WL (June 2013). "A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients". Ann. Rheum. Dis. 72 (6): 1011–7. doi:10.1136/annrheumdis-2012-201531. PMID 22887848.
  7. ↑ Guillevin L, Jarrousse B, Lok C, Lhote F, Jais JP, Le Thi Huong Du D, Bussel A (April 1991). "Longterm followup after treatment of polyarteritis nodosa and Churg-Strauss angiitis with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange. A prospective randomized trial of 71 patients. The Cooperative Study Group for Polyarteritis Nodosa". J. Rheumatol. 18 (4): 567–74. PMID 1676753.
  8. ↑ Hellmich B, Gross WL (January 2004). "Recent progress in the pharmacotherapy of Churg-Strauss syndrome". Expert Opin Pharmacother. 5 (1): 25–35. doi:10.1517/14656566.5.1.25. PMID 14680433.
  9. ↑ Saech J, Owczarczyk K, Owczarzyk K, Rösgen S, Petereit H, Hallek M, Rubbert-Roth A (June 2010). "Successful use of rituximab in a patient with Churg-Strauss syndrome and refractory central nervous system involvement". Ann. Rheum. Dis. 69 (6): 1254–5. doi:10.1136/ard.2009.109850. PMID 19740901.
  10. ↑ Metzler C, Schnabel A, Gross WL, Hellmich B (2008). "A phase II study of interferon-alpha for the treatment of refractory Churg-Strauss syndrome". Clin. Exp. Rheumatol. 26 (3 Suppl 49): S35–40. PMID 18799051.
  11. ↑ Jachiet M, Samson M, Cottin V, Kahn JE, Le Guenno G, Bonniaud P, Devilliers H, Bouillet L, Gondouin A, Makhlouf F, Meaux-Ruault N, Gil H, Bienvenu B, Coste A, Groh M, Giraud V, Dominique S, Godeau B, Puéchal X, Khouatra C, Ruivard M, Le Jeunne C, Mouthon L, Guillevin L, Terrier B (September 2016). "Anti-IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss): Data on Seventeen Patients". Arthritis Rheumatol. 68 (9): 2274–82. doi:10.1002/art.39663. PMID 26946346.
  12. ↑ Danieli MG, Cappelli M, Malcangi G, Logullo F, Salvi A, Danieli G (December 2004). "Long term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome". Ann. Rheum. Dis. 63 (12): 1649–54. doi:10.1136/ard.2003.015453. PMC 1754837. PMID 15547090.
  13. ↑ Guillevin L, Cevallos R, Durand-Gasselin B, Lhote F, Jarrousse B, Callard P (1997). "Treatment of glomerulonephritis in microscopic polyangiitis and Churg-Strauss syndrome. Indications of plasma exchanges, Meta-analysis of 2 randomized studies on 140 patients, 32 with glomerulonephritis". Ann Med Interne (Paris). 148 (3): 198–204. PMID 9255326.
  14. ↑ Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ (December 2003). "Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis". Am. J. Kidney Dis. 42 (6): 1149–53. PMID 14655185.

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