Churg-Strauss syndrome medical therapy: Difference between revisions
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**American College of Rheumatology (ACR) | **American College of Rheumatology (ACR) | ||
*Pharmacologic therapy for EGPA include systemic [[glucocorticoids]](eg, [[prednisone]]), immunosupressive agents(eg, [[cyclophosphamide]]), [[Chemotherapy|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]]), immunosupressive agents(eg, [[cyclophosphamide]]), [[Chemotherapy|cytotoxic drugs]](eg, [[azathioprine]], [[methotrexate]]), inhaled glucocorticoids, IVIG (intravenous immune globulin), anti-IgE(eg, [[omalizumab]]), anti-IL-5 antibodies(eg, [[mepolizumab]]), and plasma exchange. | ||
* Five factor score(FFS) and Birmingham vasculitis activity score (BVAS) can be used to assess the vasculitis 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> | ||
===Systemic glucocorticosteroids=== | ===Systemic glucocorticosteroids=== | ||
*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> | *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> | ||
*Preferred regimen: | *Preferred regimen: | ||
**Oral dose of 0.5 to 1.0 mg/kg/day | **Oral dose of 0.5 to 1.0 mg/kg/day | ||
**Higher doses up to 2mg/kg/day may be required in severe vasculitis involving heart, kidney, and GIT. | **Higher doses up to 2mg/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 | **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 corticosteroids. | ||
*Most common side effects: | *Most common [[Adverse effect (medicine)|side effects]]: | ||
**Fluid | **[[Water retention|Fluid retention]] | ||
**Bloating | **[[Bloating]] | ||
**Weight gain | **[[Weight gain]] | ||
**Osteoporosis | **[[Osteoporosis]] | ||
**Cataracts | **[[Cataract|Cataracts]] and [[glaucoma]] | ||
===Intravenous immunoglobulins=== | ===Intravenous immunoglobulins=== | ||
| Line 73: | Line 73: | ||
===Plasma exchange=== | ===Plasma exchange=== | ||
*The benifitial role of plasma exchange in addition to glucocortcoid or 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> | *The benifitial role of plasma exchange in addition to glucocortcoid or 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 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> | ||
==References== | ==References== | ||
Revision as of 17:37, 10 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
- Medical therapy for eosinophilic granulomatosis with polyangiitis is according to the guidelines proposed by:[1][2]
- EGPA Consensus Task Force recommendations by EULAR (European League Against Rheumatism)
- American College of Rheumatology (ACR)
- Pharmacologic therapy for EGPA include systemic glucocorticoids(eg, prednisone), immunosupressive agents(eg, cyclophosphamide), cytotoxic drugs(eg, azathioprine, methotrexate), inhaled glucocorticoids, IVIG (intravenous immune globulin), anti-IgE(eg, omalizumab), anti-IL-5 antibodies(eg, mepolizumab), and plasma exchange.
- Five factor score(FFS) and Birmingham vasculitis activity score (BVAS) can be used to assess the vasculitis severity and disease activity. These two scoring systems can be helpful in initiating therapy.[3][4]
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]
- Preferred regimen:
- 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:
Intravenous immunoglobulins
- High doses of intravenous immunoglobulins may be considered in patients whose flare are refractory to standard therapy and during pregnancy.[6]
- Preferred regimen: 2 g/kg for 2–5-day cycles for every 3-4 weeks.
Plasma exchange
- The benifitial role of plasma exchange in addition to glucocortcoid or immunosuppressieve therapy to improve survival rate in patients with eosinophilic granulomatosis with polyangiitis is unclear.[7]
- Plasma exchange may be considered for patients with severe and rapidly progressieve renal failure, and diffuse alveolar hemorrhage.[8]
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.
- ↑ 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.
- ↑ 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.