Microscopic Polyangiitis Treatment & Management
- Author: Mehran Farid-Moayer, MD; Chief Editor: Vecihi Batuman, MD, FACP, FASN more...
Medical Care
- Microscopic polyangiitis (MPA) can manifest as a mild systemic vasculitis with mild renal insufficiency, or it can manifest as a full blown acute disease with rapid deterioration of renal function and respiratory failure due to pulmonary capillaritis. Treatment depends on the extent of disease, the rate of progression, and the degree of inflammation (see Medication).
Consultations
- A rheumatologist would be the main consultant who helps with the diagnosis and immunosuppressive therapy.
- Base consultations on the specific organ system involvement, as follows:
- Consult a pulmonologist for the management of hemoptysis due to pulmonary alveolar capillaritis. Additionally, consult a pulmonologist to help with the management of respiratory failure associated with diffuse alveolar hemorrhage and with the diagnosis of pulmonary involvement.
- Consult a nephrologist for help with the diagnosis and management of renal involvement and possible need for dialysis.
- Consult a gastroenterologist, if necessary, for the management of gastrointestinal bleeding.
- Consult a surgeon in cases involving catastrophic events in gastrointestinal or other organ systems.
- Consult a hematologist if plasmapheresis is considered.
Diet
- The nutritional requirement depends on the particular clinical situation, such as renal failure, respiratory failure, or pancreatitis.
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].
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].
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].
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].
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].
Seligman VA, Bolton PB, Sanchez HC, Fye KH. Propylthiouracil-induced microscopic polyangiitis. J Clin Rheumatol. Jun 2001;7(3):170-4. [Medline].
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].
[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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Jayne D. Review article: Progress of treatment in ANCA-associated vasculitis. Nephrology (Carlton). Feb 2009;14(1):42-8. [Medline].
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].
Jayne DR, Gaskin G, Pusey CD, Lockwood CM. ANCA and predicting relapse in systemic vasculitis. QJM. Feb 1995;88(2):127-33. [Medline].
Jennette JC. Antineutrophil cytoplasmic autoantibody-associated diseases: a pathologist's perspective. Am J Kidney Dis. Aug 1991;18(2):164-70. [Medline].
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. Nov 20 1997;337(21):1512-23. [Medline].
Kamesh L, Harper L, Savage CO. ANCA-positive vasculitis. J Am Soc Nephrol. Jul 2002;13(7):1953-60. [Medline].
Lane SE, Watts RA, Shepstone L, Scott DG. Primary systemic vasculitis: clinical features and mortality. QJM. Feb 2005;98(2):97-111. [Medline].
Lhote F, Cohen P, Genereau T, et al. Microscopic polyangiitis: clinical aspects and treatment. Ann Med Interne (Paris). 1996;147(3):165-77. [Medline].
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].
Matteson EL. Small-vessel vasculitis. N Engl J Med. Apr 2 1998;338(14):994-5. [Medline].
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].
Savage CO, Harper L, Adu D. Primary systemic vasculitis. Lancet. Feb 22 1997;349(9051):553-8. [Medline].
Sneller MC, Fauci AS. Pathogenesis of vasculitis syndromes. Med Clin North Am. Jan 1997;81(1):221-42. [Medline].
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].
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].

