Microscopic Polyangiitis Follow-up

  • Author: Mehran Farid-Moayer, MD; Chief Editor: Vecihi Batuman, MD, FACP, FASN   more...
 
Updated: Jan 24, 2012
 

Further Outpatient Care

  • Patients with microscopic polyangiitis (MPA) need to be monitored very closely at a rheumatology clinic.
    • Patients need to take immunosuppressive medications for a long time, at least for a year.
    • Clinical status and the ESR should be monitored.
    • The level of ANCA may be used to monitor the disease activity. However, ANCA levels do not correlate consistently with the disease activity. In one study, ANCA levels became undetectable in 83% of patients after treatment; however, ANCA levels increased in 57% of patients at a mean period of 7.8 weeks prior to relapse. In another study, anti-MPO antibodies yielded a very good positive predictive value for the detection of relapse.[15]
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Complications

  • Complications of vasculitis depend on the particular organ system involvement (see Physical).
  • One of the most significant complications of treatment is bladder cancer. Among people who are treated with cyclophosphamide, 5% develop bladder cancer after 10 years and 16% develop bladder cancer after 15 years.
  • One study reviewed cardiovascular outcomes during long-term follow-up of patients with Wegener's granulomatosis and microscopic polyangiitis in the first 4 European Vasculitis Study Group trials. A model was developed and validated to predict those at risk for a cardiovascular event. Older age, diastolic hypertension, and positive PR3-ANCA status were found to be independent determinants of cardiovascular outcomes. Within 5 years of diagnosis, 14% of patients experience cardiovascular event.[16]
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Prognosis

  • Remission: With treatment, 90% of patients improve and 75% achieve complete remission. The 5-year survival rate is approximately 75%. MPA carries a worse long-term survival rate than Wegener granulomatosis or Churg-Strauss syndrome, probably because of renal involvement at disease onset.
  • Relapse: Of patients with MPA, 30% relapse in 1-2 years.
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Patient Education

  • Patients with MPA should be aware of the severity of the disease and the risk of recurrence.
  • Compliance with medications and follow-up visits is critical.
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Contributor Information and Disclosures
Author

Mehran Farid-Moayer, MD  Adjunct Clinical Faculty, Department of Psychiatry, Sleep Disorders Clinic, Stanford Medical Center

Mehran Farid-Moayer, MD is a member of the following medical societies: American Academy of Sleep Medicine, American Medical Association, and American Thoracic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Spencer T Lowe, MD  Rheumatologist, Private Practice, Peninsula Medical Group, Burlingame, CA

Spencer T Lowe, MD is a member of the following medical societies: American College of Rheumatology, California Medical Association, and International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Specialty Editor Board

Bryan L Martin, DO  Associate Dean for Graduate Medical Education, Designated Institutional Official, Associate Medical Director, Director, Allergy Immunology Program, Professor of Medicine and Pediatrics, Ohio State University College of Medicine

Bryan L Martin, DO is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, and American Osteopathic Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Elliot Goldberg, MD  Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FACP, FASN  Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Medicine Service, Southeast Louisiana Veterans Health Care System

Vecihi Batuman, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, and International Society of Nephrology

Disclosure: Nothing to disclose.

References

  1. Fries JF, Hunder GG, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum. Aug 1990;33(8):1135-6. [Medline].

  2. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. Feb 1994;37(2):187-92. [Medline].

  3. Lightfoot RW, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. Aug 1990;33(8):1088-93. [Medline].

  4. Falk RJ, Hogan S, Carey TS, Jennette JC. Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis. The Glomerular Disease Collaborative Network. Ann Intern Med. Nov 1 1990;113(9):656-63. [Medline].

  5. Amezcua-Guerra LM, Prieto P, Bojalil R, Pineda C, Amigo MC. Microscopic polyangiitis associated with primary biliary cirrhosis: a causal or casual association?. J Rheumatol. Nov 2006;33(11):2351-3. [Medline].

  6. Seligman VA, Bolton PB, Sanchez HC, Fye KH. Propylthiouracil-induced microscopic polyangiitis. J Clin Rheumatol. Jun 2001;7(3):170-4. [Medline].

  7. Hervier B, Durant C, Masseau A, Ponge T, Hamidou M, Mussini JM. Use of muscle biopsies for diagnosis of systemic vasculitides. J Rheumatol. Mar 2011;38(3):470-4. [Medline].

  8. [Best Evidence] Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. Jul 15 2010;363(3):221-32. [Medline]. [Full Text].

  9. Roubaud-Baudron C, Pagnoux C, Méaux-Ruault N, et al. Rituximab Maintenance Therapy for Granulomatosis with Polyangiitis and Microscopic Polyangiitis. J Rheumatol. Jan 2012;39(1):125-130. [Medline].

  10. Reinhold-Keller E, De Groot K, Rudert H, Nölle B, Heller M, Gross WL. Response to trimethoprim/sulfamethoxazole in Wegener's granulomatosis depends on the phase of disease. QJM. Jan 1996;89(1):15-23. [Medline].

  11. Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CG. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med. Jul 4 1996;335(1):16-20. [Medline].

  12. Langford CA, Talar-Williams C, Sneller MC. Mycophenolate mofetil for remission maintenance in the treatment of Wegener's granulomatosis. Arthritis Rheum. Apr 15 2004;51(2):278-83. [Medline].

  13. Nowack R, Göbel U, Klooker P, Hergesell O, Andrassy K, van der Woude FJ. Mycophenolate mofetil for maintenance therapy of Wegener's granulomatosis and microscopic polyangiitis: a pilot study in 11 patients with renal involvement. J Am Soc Nephrol. Sep 1999;10(9):1965-71. [Medline].

  14. Iatrou C, Zerbala S, Revela I, Spanou E, Marinaki S, Nakopoulou L, et al. Mycophenolate mofetil as maintenance therapy in patients with vasculitis and renal involvement. Clin Nephrol. Jul 2009;72(1):31-7. [Medline].

  15. Terrier B, Saadoun D, Sène D, Ghillani P, Amoura Z, Deray G, et al. Antimyeloperoxidase antibodies are a useful marker of disease activity in antineutrophil cytoplasmic antibody-associated vasculitides. Ann Rheum Dis. Oct 2009;68(10):1564-71. [Medline].

  16. Suppiah R, Judge A, Batra R, Flossmann O, Harper L, Höglund P, et al. A model to predict cardiovascular events in patients with newly diagnosed wegener's granulomatosis and microscopic polyangiitis. Arthritis Care Res (Hoboken). Jan 14 2011;[Medline].

  17. Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. Mar 1999;42(3):421-30. [Medline].

  18. Haubitz M, Koch KM, Brunkhorst R. Cyclosporin for the prevention of disease reactivation in relapsing ANCA-associated vasculitis. Nephrol Dial Transplant. Aug 1998;13(8):2074-6. [Medline].

  19. Jayne D. Review article: Progress of treatment in ANCA-associated vasculitis. Nephrology (Carlton). Feb 2009;14(1):42-8. [Medline].

  20. Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. Jul 3 2003;349(1):36-44. [Medline].

  21. Jayne DR, Gaskin G, Pusey CD, Lockwood CM. ANCA and predicting relapse in systemic vasculitis. QJM. Feb 1995;88(2):127-33. [Medline].

  22. Jennette JC. Antineutrophil cytoplasmic autoantibody-associated diseases: a pathologist's perspective. Am J Kidney Dis. Aug 1991;18(2):164-70. [Medline].

  23. Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. Nov 20 1997;337(21):1512-23. [Medline].

  24. Kamesh L, Harper L, Savage CO. ANCA-positive vasculitis. J Am Soc Nephrol. Jul 2002;13(7):1953-60. [Medline].

  25. Lane SE, Watts RA, Shepstone L, Scott DG. Primary systemic vasculitis: clinical features and mortality. QJM. Feb 2005;98(2):97-111. [Medline].

  26. Lhote F, Cohen P, Genereau T, et al. Microscopic polyangiitis: clinical aspects and treatment. Ann Med Interne (Paris). 1996;147(3):165-77. [Medline].

  27. Liu LJ, Chen M, Yu F, Zhao MH, Wang HY. Evaluation of a new algorithm in classification of systemic vasculitis. Rheumatology (Oxford). May 2008;47(5):708-12. [Medline].

  28. Matteson EL. Small-vessel vasculitis. N Engl J Med. Apr 2 1998;338(14):994-5. [Medline].

  29. Ronco P, Verroust P, Mignon F, Kourilsky O, Vanhille P, Meyrier A, et al. Immunopathological studies of polyarteritis nodosa and Wegener's granulomatosis: a report of 43 patients with 51 renal biopsies. Q J Med. Spring 1983;52(206):212-23. [Medline].

  30. Savage CO, Harper L, Adu D. Primary systemic vasculitis. Lancet. Feb 22 1997;349(9051):553-8. [Medline].

  31. Sneller MC, Fauci AS. Pathogenesis of vasculitis syndromes. Med Clin North Am. Jan 1997;81(1):221-42. [Medline].

  32. Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. Feb 2007;66(2):222-7. [Medline].

  33. Watts RA, Jolliffe VA, Carruthers DM, et al. Effect of classification on the incidence of polyarteritis nodosa and microscopic polyangiitis. Arthritis Rheum. Jul 1996;39(7):1208-12. [Medline].

 
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