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{{Microscopic polyangiitis}}
{{Microscopic polyangiitis}}
{{CMG}}{{APM}}{{AE}}{{KW}}
{{CMG}} ; {{AE}}{{VKG}}
==Overview==
==Overview==
Microscopic polyangiitis responds well to treatment with glucocorticoids such as prednisone together with a immunosuppressant such as cyclophosphamide. The combination of these 2 drugs decreases the remission of Microscopic polyangiitis by about 90%.
[[Microscopic polyangiitis]] responds well to treatment with [[glucocorticoids]] such as [[prednisone]] together with an [[immunosuppressant]] such as [[cyclophosphamide]]. The combination of these 2 drugs decreases the remission of [[Microscopic polyangiitis]] by about 90%.


== Medical Therapy ==
== Medical Therapy ==
* Pharmacologic medical therapies for Microscopic polyangiitis include glucocorticoids and immunosuppressant.<ref name="pmid25992801">{{cite journal| author=Greco A, De Virgilio A, Rizzo MI, Gallo A, Magliulo G, Fusconi M et al.| title=Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches. | journal=Autoimmun Rev | year= 2015 | volume= 14 | issue= 9 | pages= 837-44 | pmid=25992801 | doi=10.1016/j.autrev.2015.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25992801  }}</ref>
* Pharmacologic medical therapies for [[Microscopic polyangiitis]] include [[glucocorticoids]] and [[immunosuppressant]].<ref name="pmid25992801">{{cite journal| author=Greco A, De Virgilio A, Rizzo MI, Gallo A, Magliulo G, Fusconi M et al.| title=Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches. | journal=Autoimmun Rev | year= 2015 | volume= 14 | issue= 9 | pages= 837-44 | pmid=25992801 | doi=10.1016/j.autrev.2015.05.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25992801  }}</ref><ref name="pmid23615499">{{cite journal |vauthors=Walsh M, Casian A, Flossmann O, Westman K, Höglund P, Pusey C, Jayne DR |title=Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear |journal=Kidney Int. |volume=84 |issue=2 |pages=397–402 |date=August 2013 |pmid=23615499 |doi=10.1038/ki.2013.131 |url=}}</ref>


=== '''Corticosteroids:''' ===
=== '''Corticosteroids:''' ===
* In more aggressive forms of the disease prednisone is interchanged with methylprednisolone which is given intravenously at a dose of 1 g/kg 3 times a day.   
* In more aggressive forms of the disease [[prednisone]] is interchanged with [[methylprednisolone]].<ref name="pmid20235186">{{cite journal |vauthors=Walsh M, Merkel PA, Mahr A, Jayne D |title=Effects of duration of glucocorticoid therapy on relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: A meta-analysis |journal=Arthritis Care Res (Hoboken) |volume=62 |issue=8 |pages=1166–73 |date=August 2010 |pmid=20235186 |pmc=2946200 |doi=10.1002/acr.20176 |url=}}</ref><ref name="pmid26031284">{{cite journal |vauthors=Hellmich B |title=[Treatment strategies for ANCA-associated vasculitides] |language=German |journal=Z Rheumatol |volume=74 |issue=5 |pages=388–97 |date=June 2015 |pmid=26031284 |doi=10.1007/s00393-014-1532-7 |url=}}</ref>  
* Both mild and severe forms of the disease are administered together with cyclophosphamide which is given in intravenous pulses every 2 weeks at a dose of 15 mg/kg for the first three infusions.   
* Both mild and severe forms of the disease are administered together with [[cyclophosphamide]] which is given in intravenous pulses every 2 weeks.   
* After the first 3 doses, cyclophosphamide is administered every 3 weeks at 15 mg/kg.   
* After the first 3 doses, [[cyclophosphamide]] is administered every 3 weeks.   
* Cyclophosphamide can also be given orally at a dose of 2 mg/kg/day, however, more side effects are seen with the oral dose, such as neutropenia.  
* [[Cyclophosphamide]] can also be given orally at a dose of 2 mg/kg/day, however, more side effects are seen with the oral dose, such as [[neutropenia]].  
* The dose of prednisone that is given is 1 mg/kg/day for less aggressive forms of the disease  
* The dose of [[prednisone]] that is given is 1 mg/kg/day for less aggressive forms of the disease.


* If Microscopic polyangiitis is severe, plasmapheresis may also be given in conjunction with an immunosuppressant and glucocorticoid.  
* If [[Microscopic polyangiitis]] is severe, [[plasmapheresis]] may also be given in conjunction with an [[immunosuppressant]] and [[glucocorticoid]].  
* Plasmapheresis has been shown to have benefit in patients with pulmonary and renal involvement.
* [[Plasmapheresis]] has been shown to have benefit in patients with [[pulmonary]] and [[renal]] involvement.
** Preferred regimen (1): [[Methylprednisolone]] 1g/kg 3 times a day.
** Preferred regimen (2): [[Cyclophosphamide]] 15 mg/kg.


=== '''Rituximab''' ===
=== '''Rituximab''' ===
* Induction therapy using rituximab and glucocorticoids in a recent study conducted by RITUXVAS compared rituximab and cyclophosphamide.  
* Induction therapy using [[rituximab]] and [[glucocorticoids]] in a recent study conducted by RITUXVAS compared [[rituximab]] and [[cyclophosphamide]].<ref name="pmid18281850">{{cite journal |vauthors=Jayne D |title=Challenges in the management of microscopic polyangiitis: past, present and future |journal=Curr Opin Rheumatol |volume=20 |issue=1 |pages=3–9 |date=January 2008 |pmid=18281850 |doi=10.1097/BOR.0b013e3282f370d1 |url=}}</ref><ref name="pmid25805743">{{cite journal |vauthors=McGregor JG, Hogan SL, Kotzen ES, Poulton CJ, Hu Y, Negrete-Lopez R, Kidd JM, Katsanos SL, Bunch DO, Nachman PH, Falk RJ |title=Rituximab as an immunosuppressant in antineutrophil cytoplasmic antibody-associated vasculitis |journal=Nephrol. Dial. Transplant. |volume=30 Suppl 1 |issue= |pages=i123–31 |date=April 2015 |pmid=25805743 |pmc=4447867 |doi=10.1093/ndt/gfv076 |url=}}</ref>
* The trial showed no superiority, in that both medications were effective at inducing remission. However, the safety and the long term use of rituximab needs to be further addressed.
* The trial showed no superiority, in that both medications were effective at inducing [[remission]]. However, the safety and the long term use of [[rituximab]] needs to be further addressed.


== Maintenance Therapy ==
== Maintenance Therapy ==
The maintenance therapy for Microscopic polyangiitis is with azathioprine, which is less toxic to that of cyclophosphamide. Azathioprine is administered for 18 months at a dose of 1 to 2 mg/kg/day.
* The maintenance therapy for [[Microscopic polyangiitis]] is with [[azathioprine]], which is less toxic to that of [[cyclophosphamide]]. [[Azathioprine]] is administered for 18 months.
** Preferred regimen (1):[[Azathioprine]] 1 to 2 mg/kg/day.


Other medications that may be used as maintenance are:
* Other medications that may be used as maintenance are:
* Myclophenolate mofetil: up to 1g twice a day
** Preferred regimen (1): [[Mycophenolate]] mofetil up to 1g twice a day.
* Methotrexate: 0.3 to 25 mg/kg/week
** Preferred regimen (2): [[Methotrexate]]: 0.3 to 25 mg/kg/week.


==References==
==References==

Latest revision as of 13:47, 30 April 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

Overview

Microscopic polyangiitis responds well to treatment with glucocorticoids such as prednisone together with an immunosuppressant such as cyclophosphamide. The combination of these 2 drugs decreases the remission of Microscopic polyangiitis by about 90%.

Medical Therapy

Corticosteroids:

  • In more aggressive forms of the disease prednisone is interchanged with methylprednisolone.[3][4]
  • Both mild and severe forms of the disease are administered together with cyclophosphamide which is given in intravenous pulses every 2 weeks.
  • After the first 3 doses, cyclophosphamide is administered every 3 weeks.
  • Cyclophosphamide can also be given orally at a dose of 2 mg/kg/day, however, more side effects are seen with the oral dose, such as neutropenia.
  • The dose of prednisone that is given is 1 mg/kg/day for less aggressive forms of the disease.

Rituximab

Maintenance Therapy

  • Other medications that may be used as maintenance are:
    • Preferred regimen (1): Mycophenolate mofetil up to 1g twice a day.
    • Preferred regimen (2): Methotrexate: 0.3 to 25 mg/kg/week.

References

  1. Greco A, De Virgilio A, Rizzo MI, Gallo A, Magliulo G, Fusconi M; et al. (2015). "Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches". Autoimmun Rev. 14 (9): 837–44. doi:10.1016/j.autrev.2015.05.005. PMID 25992801.
  2. Walsh M, Casian A, Flossmann O, Westman K, Höglund P, Pusey C, Jayne DR (August 2013). "Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear". Kidney Int. 84 (2): 397–402. doi:10.1038/ki.2013.131. PMID 23615499.
  3. Walsh M, Merkel PA, Mahr A, Jayne D (August 2010). "Effects of duration of glucocorticoid therapy on relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: A meta-analysis". Arthritis Care Res (Hoboken). 62 (8): 1166–73. doi:10.1002/acr.20176. PMC 2946200. PMID 20235186.
  4. Hellmich B (June 2015). "[Treatment strategies for ANCA-associated vasculitides]". Z Rheumatol (in German). 74 (5): 388–97. doi:10.1007/s00393-014-1532-7. PMID 26031284.
  5. Jayne D (January 2008). "Challenges in the management of microscopic polyangiitis: past, present and future". Curr Opin Rheumatol. 20 (1): 3–9. doi:10.1097/BOR.0b013e3282f370d1. PMID 18281850.
  6. McGregor JG, Hogan SL, Kotzen ES, Poulton CJ, Hu Y, Negrete-Lopez R, Kidd JM, Katsanos SL, Bunch DO, Nachman PH, Falk RJ (April 2015). "Rituximab as an immunosuppressant in antineutrophil cytoplasmic antibody-associated vasculitis". Nephrol. Dial. Transplant. 30 Suppl 1: i123–31. doi:10.1093/ndt/gfv076. PMC 4447867. PMID 25805743.

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