Microscopic Polyangiitis

  • Yeon Joo Jeong
  • Man Pyo Chung
  • Kyung Soo Lee
Part of the Medical Radiology book series (MEDRAD)


Microscopic polyangiitis is a systemic necrotizing vasculitis with mainly renal and pulmonary manifestations. It can be distinguished from other forms of small vessel vasculitis by the absence of granuloma formation, the relative lack of upper airway involvement, and by the predominance of myeloperoxidase for perinuclear antineutrophil cytoplasmic antibody staining. The radiologic features of microscopic polyangiitis reflect the presence of diffuse alveolar hemorrhage.


Ground Glass Opacity Diffuse Alveolar Hemorrhage Small Vessel Vasculitis Fibrinoid Necrosis Renal Manifestation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. Agard C, Mouthon L, Mahr A, Guillevin L (2003) Microscopic polyangiitis and polyarteritis nodosa: How and when do they start? Arthritis Rheum 49:709–715PubMedCrossRefGoogle Scholar
  2. Akikusa B, Sato T, Ogawa M et al (1997) Necrotizing alvelolar capillaritis in autopsy cases of microscopic polyangiitis. Incidence, histopathogenesis, and relationship with systemic vasculitis. Arch Pathol Lab Med 121:144–149PubMedGoogle Scholar
  3. Ando Y, Okada F, Matsumoto S, Mori H (2004) Thoracic manifestation of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)- related disease. CT findings in 51 patients. J Comput Assist Tomogr 28:710–716PubMedCrossRefGoogle Scholar
  4. Ara J, Mirapeix E, Rodriguez R et al (1999) Relationship between ANCA and disease activity in small vessel vasculitis patients with anti-MPO ANCA. Nephrol Dial Transplant 14:1667–1672PubMedCrossRefGoogle Scholar
  5. Birnbaum J, Danoff S, Askin FB, Stone JH (2007) Microscopic polyangiitis presenting as a “pulmonary-muscle” syndrome: is subclincal alveolar hemorrhage the mechanism of pulmonary fibrosis? Arthritis Rheum 56:2065–2071PubMedCrossRefGoogle Scholar
  6. Bosch X, Guilabert A, Espinosa G, Mirapeix E (2007) Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systemic review. JAMA 298:655–669PubMedCrossRefGoogle Scholar
  7. Chung SA, Seo P (2010) Microscopic polyangiitis. Rheum Dis Clin North Am 36:545–558PubMedCrossRefGoogle Scholar
  8. Chung MP, Yi CA, Lee HY et al (2010) Imaging of pulmonary vasculitis. Radiology 255:322–341PubMedCrossRefGoogle Scholar
  9. Davson J, Ball J, Platt R (1948) The kidney in periarteritis nodosa. Q J Med 17:175–202PubMedGoogle Scholar
  10. Eschun GM, Mink SN, Sharma S (2003) Pulmonary interstitial fibrosis as a presenting manifestation in perinuclear antineutrophilic cytoplasmic antibody microscopic polyangiitis. Chest 123:297–301PubMedCrossRefGoogle Scholar
  11. Gollins CE, Quismorio FP Jr (2005) Pulmonary involvement in microscopic polyangiitis. Curr Opin Pulm Med 11:447–451CrossRefGoogle Scholar
  12. Gomez-Puerta JA, Espinosa G, Morla R et al (2009) Interstitial lung disease as a presenting manifestation of microscopic polyangiitis successfully treated with mycophenolate mofetil. Clin Exp Rheumatol 27:166–167PubMedGoogle Scholar
  13. Guillevin L, Durand-Gasselin B, Cevallos R, Callard P et al (1999) Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 42:421–430PubMedCrossRefGoogle Scholar
  14. Jennette JC, Falk RJ (2008) New insight into the pathogenesis of vasculitis associated with antineutrophil cytoplasmic antibodies. Curr Opin Rheumatol 20:55–60PubMedCrossRefGoogle Scholar
  15. Jennette JC, Falk RJ, Andrassy K, Gross WL et al (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37:187–192PubMedCrossRefGoogle Scholar
  16. Jennette JC, Thomas DB, Falk RJ (2001) Microscopic polyangiitis (microscopic polyarteritis). Semi Diagn Pathol 18:3–13PubMedGoogle Scholar
  17. Kluger N, Pagnoux C, Guillevin L, Frances C (2008) Comparison of cutaneous manifestations in systemic polyarteritis nodosa and microscopic polyangiitis. Br J Dermatol 159:615–620PubMedCrossRefGoogle Scholar
  18. Lauque D, Cadranel J, Lazor R et al (2000) Microscopic polyangiitis with alveolar hemorrhage. A study of 29 cases and review of the literature. Groupe d’Etudes et de Recherche sur les Maladies ‘‘Orphelines’’ Pulmonaires (GERM‘‘O’’P). Medicine 79:222–233PubMedCrossRefGoogle Scholar
  19. Lhote F, Cohen P, Guillevin L (1998) Polyarteritis nodosa, microscopic polyangiitis and Churg–Strauss syndrome. Lupus 7:238–258PubMedCrossRefGoogle Scholar
  20. Pagnoux C, Mahr A, Cohen P, Guillevin L (2005) Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine 84:115–128PubMedCrossRefGoogle Scholar
  21. Savage CO, Winearls CG, Evans DJ et al (1985) Microscopic polyarteritis: presentation, pathology and prognosis. Q J Med 56:467–483PubMedGoogle Scholar
  22. Schwarz MI, Brown KK (2000) Small vessel vasculitis of the lung. Thorax 55:502–510PubMedCrossRefGoogle Scholar
  23. Stone JH, Merkel PA, Spiera R et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363:221–232PubMedCrossRefGoogle Scholar
  24. Ueda S, Matsumoto M, Ahn T et al (2001) Microscopic polyangiitis complicated with massive intestinal bleeding. J Gastroenterol 36:264–270PubMedCrossRefGoogle Scholar
  25. Westman KW, Bygren PG, Olsson H et al (1998) Relapse rate, renal survival, and cancer morbidity in patients with Wegener’s granulomatosis or microscopic polyangiitis with renal involvement. J Am Soc Nephrol 9:842–852PubMedGoogle Scholar
  26. Zhang W, Zhou G, Shi Q et al (2009) Clinical analysis of nervous system involvement in ANCA-associated systemic vasculitides. Clin Exp Rheumatol 27:S65–S69PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg  2011

Authors and Affiliations

  • Yeon Joo Jeong
    • 1
  • Man Pyo Chung
    • 2
  • Kyung Soo Lee
    • 3
  1. 1.Department of Diagnostic RadiologyPusan National University Hospital, Pusan National University School of MedicinePusanKorea
  2. 2.Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Samsung Medical CenterSungkyunkwan University School of MedicineSeoulKorea
  3. 3.Department of RadiologySamsung Medical Center, Sungkyunkwan University School of MedicineSeoulKorea

Personalised recommendations