Microscopic polyangiitis medical therapy

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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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