Churg-Strauss syndrome medical therapy: Difference between revisions
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==Overview== | ==Overview== | ||
The mainstay of management for [[eosinophilic granulomatosis with polyangiitis]] is [[glucocorticoids]] and [[cyclophosphamide]]. | 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 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. | *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. | ||
* 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> | * 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== | ==Initiating pharmacotherapy== | ||
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*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> | *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> | ||
*Preferred regimen: | *Preferred regimen: | ||
**Oral dose of 0.5 to 1.0 mg/kg/day for 2-3 weeks | **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 | **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]]. | ||
*For life-threatening [[vasculitis]], intravenous [[glucocorticoids]] can be administered. | *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. | **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. | *Once remission is achieved, dose is gradually tapered over months. Patients who achieve remission will require long term low dose of [[Corticosteroid|corticosteroids]]. | ||
*Most common [[Adverse effect (medicine)|side effects]]: | *Most common [[Adverse effect (medicine)|side effects]]: | ||
**[[Water retention|Fluid retention]] | **[[Water retention|Fluid retention]] | ||
| Line 30: | Line 30: | ||
*[[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> | *[[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: | *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. | **[[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]]: | *Common [[Adverse effect (medicine)|side effects]]: | ||
**Severe drug induced [[neutropenia]] | **Severe drug induced [[neutropenia]] | ||
**[[Opportunistic infection]] | **[[Opportunistic infection]] | ||
**Gonadal toxicity | **Gonadal toxicity | ||
**Low renal clearance | **Low [[Clearance (medicine)|renal clearance]] | ||
==Maintenance therapy== | ==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> | *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> | ||
| Line 46: | Line 46: | ||
*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> | *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=== | ===Interferon-alpha=== | ||
*Second line therapy drug, may be useful for patients who are not responding to [[glucocorticoids]] or immunosuppressieve agents | *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 | *Preferred regimen: Subcutaneous administration of 3 million I.U./3 times weekly | ||
*Relapses are more frequent | *Relapses are more frequent | ||
===Anti-IgE therapy=== | ===Anti-IgE therapy=== | ||
*[[Omalizumab]] may be administered to control [[asthma]], [[Rhinosinusitis|sinusitis]] and [[eosinophilia]] refractory to systemic and 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> | *[[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=== | ===Anti-IL-5 antibodies=== | ||
*[[Mepolizumab]]: Preferred regimen: 300mg given every 4 weeks. | *[[Mepolizumab]]: Preferred regimen: 300mg given every 4 weeks. | ||
| Line 56: | Line 56: | ||
*They can be used to relieve symptoms of upper airway disease. | *They can be used to relieve symptoms of upper airway disease. | ||
===Intravenous immunoglobulins=== | ===Intravenous immunoglobulins=== | ||
* High doses of 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> | * 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. | *Preferred regimen: 2 g/kg for 2–5-day cycles for every 3-4 weeks. | ||
===Plasma exchange=== | ===Plasma exchange=== | ||
*The | *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> | *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> | ||
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
- 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]
Initiating pharmacotherapy
Systemic glucocorticosteroids
- 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:
Cyclophosphamide
- 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
Maintenance therapy
- 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.
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
- Omalizumab may be administered to control asthma, sinusitis and eosinophilia refractory to systemic and inhaled glucocorticoids.[11]
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
- 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]
References
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.