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==Overview==
==Overview==
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==
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 7.5 mg/day for 3 to 4 months. After this period, the dose of glucocorticoids in tapered down. However, that all patients respond well when the dose of glucocorticoid is tapered below 7.5 mg/day. Patients not responding well can be given a glucocorticoid-sparing agent along with there glucocorticoid. These agents include methotrexate, azathioprine, hydroxyurea, and meclophenalate 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>
*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)
Patients who present with a life threating form of the disease are treated with [[Cyclophosphamide]] together with 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. Following the 3 infusions of cyclophosphamide, cyclophosphamide is infused at 15 mg/kg every 3 weeks. Infused doses of cyclophosphamide should not exceed doses of 1.2 g. Patients receive 6 to 12 pulse infusions of cyclophosphamide.
**American College of Rheumatology (ACR)
 
*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.
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:
* 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>
{| class="sortable"
==Initiating pharmacotherapy==
!Maintenance therapy
===Systemic glucocorticosteroids===
!Azathioprine
*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>  
!Methotrexate
*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 [[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]].
|2 mg/kg/day
*For life-threatening [[vasculitis]], intravenous [[glucocorticoids]] can be administered.
|10-30 mg/wk
**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 [[Corticosteroid|corticosteroids]].
Either of these treatments can be given after a few days of oral cyclophosphamide; to 2 to 3 weeks after pulse administrative doses. The time frame at which these medications are to be administered to is currently unknown; however, 18-24 months of remission of these 2 agents have been noted.<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>      
*Most common [[Adverse effect (medicine)|side effects]]:
 
**[[Water retention|Fluid retention]]
Treatment for Churg-Strauss syndrome includes glucocorticoids such as [[prednisone]] at a dose of 0.5-1.5 mg/kg per day x 6-12 weeks and other immunosupressive drugs such as [[azathioprine]] and [[cyclophosphamide]]. In many cases the disease can be put into a type of chemical remission through drug therapy, but the disease is chronic and life long.
**[[Bloating]]
 
**[[Weight gain]]
A [[systematic review]] conducted in 2007 indicated that all patients should be treated with high-dose steroids, but that in patients with an FFS of 1 or higher cyclophosphamide pulse therapy should be commenced, with 12 pulses leading to less relapses than 6. Remission can be maintained with a less toxic drug, such as azathioprine or [[methotrexate]].<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>
**[[Osteoporosis]]
 
**[[Cataract|Cataracts]] and [[glaucoma]]
The [[erythrocyte sedimentation rate]] ([[ESR]]) and [[eosinophil]] count can be followed to gauge the response to therapy. Late relapses are uncommon and refractory disease may require [[Cyclophosphamide]], [[Azathioprine]], intravenous immunoglobulin (IVIG) and [[plasmapheresis]].
===Cyclophosphamide===
*[[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>
*Preferred regimen:
**Oral therapy: 2 mg/kg/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.
*Common [[Adverse effect (medicine)|side effects]]:
**Severe drug induced [[neutropenia]]
**[[Opportunistic infection]]
**Gonadal toxicity
**Low [[Clearance (medicine)|renal clearance]] 
==Maintenance therapy==
*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]].
*Preferred regimen: 10-30 mg/day for 12-18 months.
*[[Folic Acid|Folic acid]] supplementation is necessary during immunosuppressant therapy.
==Additional therapy==
===Rituximab===
*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>
===Interferon-alpha===
*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: Subcutaneous administration of 3 million I.U./3 times weekly
*Relapses are more frequent
===Anti-IgE therapy===
*[[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>
===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 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>
*Preferred regimen: 2 g/kg for 2–5-day cycles for every 3-4 weeks.
===Plasma exchange===
*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>
*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>


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Disease]]
[[Category:Pulmonology]]
[[Category:Autoimmune diseases]]
[[Category:Rheumatology]]


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

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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