Skip to main content
SpringerLink
Log in

Lung Involvement in ANCA-Associated Vasculitis

  • Chapter
  • First Online:
Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis

Part of the book series: Rare Diseases of the Immune System ((RDIS))

Abstract

The small vessel vasculitis syndromes, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic polyangiitis (EGPA) comprise the group referred to cumulatively as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The respiratory tract is commonly involved in each of the syndromes. The clinical presentation of pulmonary parenchymal and tracheobronchial inflammation of GPA and MPA is quite heterogeneous. Consequently, the differential diagnosis is broad and includes infection, malignancy, and other autoimmune diseases. Necrotizing granulomatous inflammation is the disease-defining feature of GPA causing lung nodules or mass lesions with or without cavitation, as well as tracheobronchial inflammation, often with subsequent stenosis. Capillaritis presenting clinically as diffuse alveolar hemorrhage is a severe disease manifestation often leading to respiratory failure which occurs in about 25% of patients with GPA or MPA, but is rare in patients with EGPA. MPA with ANCA targeting myeloperoxidase (MPO-ANCA) is sometimes associated with interstitial lung disease, a relationship that remains under investigation. The diagnostic approach for specific clinical presentations is described in this chapter. The disease manifestations caused by active inflammation in the respiratory tract usually responds well to immunosuppressive therapy following standard principles for AAV. However, damage caused by tracheobronchial involvement may require individualized interventions such as dilation procedures or stent placement. The management of diffuse alveolar hemorrhage caused by AAV is discussed in light of recent study results, and a practical approach to patients with interstitial lung disease and MPO-ANCA is proposed.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 99.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 129.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11.

    Google Scholar 

  2. Colby TV, Specks U. Wegener’s granulomatosis in the 1990s--a pulmonary pathologist's perspective. Monogr Pathol. 1993;36:195–218.

    Google Scholar 

  3. Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical pathology of the lung in Wegener’s granulomatosis. Review of 87 open lung biopsies from 67 patients. Am J Surg Pathol. 1991;15(4):315–33.

    Google Scholar 

  4. Travis WD. Common and uncommon manifestations of Wegener’s granulomatosis. Cardiovasc Pathol. 1994;3(3):217–25.

    Article  CAS  Google Scholar 

  5. Martinez F, Chung JH, Digumarthy SR, et al. Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation. Radiographics. 2012;32(1):51–69.

    Article  Google Scholar 

  6. Gibb W, Williams A. Nocardiosis mimicking Wegener’s granulomatosis. Scand J Infect Dis. 1986;18(6):583–5.

    Article  CAS  Google Scholar 

  7. Singh A, Chhina D, Soni RK, Kakkar C, Sidhu US. Clinical spectrum and outcome of pulmonary nocardiosis: 5-year experience. Lung India. 2016;33(4):398–403.

    Article  Google Scholar 

  8. Wechsler RJ, Steiner RM, Israel HL, Patchefsky AS. Chest radiograph in lymphomatoid granulomatosis: comparison with Wegener granulomatosis. AJR Am J Roentgenol. 1984;142(1):79–83.

    Article  CAS  Google Scholar 

  9. Roschewski M, Wilson WH. Lymphomatoid granulomatosis. Cancer J. 2012;18(5):469–74.

    Article  CAS  Google Scholar 

  10. Daum TE, Specks U, Colby TV, et al. Tracheobronchial involvement in Wegener’s granulomatosis. Am J Respir Crit Care Med. 1995;151(2 Pt 1):522–6.

    Article  CAS  Google Scholar 

  11. Yates M, Watts R, Bajema I, et al. Validation of the EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis by disease content experts. RMD Open. 2017;3(1):e000449.

    Article  Google Scholar 

  12. Seo P, Specks U, Keogh KA. Efficacy of rituximab in limited Wegener's granulomatosis with refractory granulomatous manifestations. J Rheumatol. 2008;35(10):2017–23.

    CAS  PubMed  Google Scholar 

  13. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221–32.

    Google Scholar 

  14. Polychronopoulos VS, Prakash UB, Golbin JM, Edell ES, Specks U. Airway involvement in Wegener’s granulomatosis. Rheum Dis Clin North Am. 2007;33(4):755–75. vi.

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  16. Watts R, Lane S, Hanslik T, 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. 2007;66(2):222–7.

    Article  Google Scholar 

  17. Lionaki S, Blyth ER, Hogan SL, et al. Classification of antineutrophil cytoplasmic autoantibody vasculitides: the role of antineutrophil cytoplasmic autoantibody specificity for myeloperoxidase or proteinase 3 in disease recognition and prognosis. Arthritis Rheum. 2012;64(10):3452–62.

    Article  CAS  Google Scholar 

  18. Ernst A, Rafeq S, Boiselle P, et al. Relapsing polychondritis and airway involvement. Chest. 2009;135(4):1024–30.

    Article  Google Scholar 

  19. Chiu K, Wright JL. Large and small airway disease related to inflammatory bowel disease. Arch Pathol Lab Med. 2017;141(3):470–3.

    Article  Google Scholar 

  20. Camus P, Colby TV. The lung in inflammatory bowel disease. Eur Respir J. 2000;15(1):5–10.

    Article  CAS  Google Scholar 

  21. Polychronopoulos VS, Prakash UBS. Airway involvement in sarcoidosis. Chest. 2009;136(5):1371–80.

    Article  Google Scholar 

  22. Summers RM, Aggarwal NR, Sneller MC, et al. CT virtual bronchoscopy of the central airways in patients with Wegener’s granulomatosis. Chest. 2002;121(1):242–50.

    Article  Google Scholar 

  23. Niles JL, Böttinger EP, Saurina GR, et al. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern Med. 1996;156(4):440–5.

    Article  CAS  Google Scholar 

  24. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488–98.

    Article  CAS  Google Scholar 

  25. Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. 2003;115(4):284–90.

    Article  CAS  Google Scholar 

  26. Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005;52(9):2926–35.

    Article  CAS  Google Scholar 

  27. Comarmond C, Pagnoux C, Khellaf M, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013;65(1):270–81.

    Article  Google Scholar 

  28. Cartin-Ceba R, Diaz-Caballero L, Al-Qadi MO, et al. Diffuse alveolar hemorrhage secondary to antineutrophil cytoplasmic antibody-associated vasculitis: predictors of respiratory failure and clinical outcomes. Arthritis Rheumatol. 2016;68(6):1467–76.

    Article  CAS  Google Scholar 

  29. Lara AR, Schwarz MI. Diffuse alveolar hemorrhage. Chest. 2010;137(5):1164–71.

    Article  Google Scholar 

  30. De Lassence A, Fleury-Feith J, Escudier E, Beaune J, Bernaudin JF, Cordonnier C. Alveolar hemorrhage. Diagnostic criteria and results in 194 immunocompromised hosts. Am J Respir Crit Care Med. 1995;151(1):157–63.

    Article  CAS  Google Scholar 

  31. Maldonado F, Parambil JG, Yi ES, Decker PA, Ryu JH. Haemosiderin-laden macrophages in the bronchoalveolar lavage fluid of patients with diffuse alveolar damage. Eur Respir J. 2009;33(6):1361–6.

    Article  CAS  Google Scholar 

  32. Epstein CE, Elidemir O, Colasurdo GN, Fan LL. Time course of hemosiderin production by alveolar macrophages in a murine model. Chest. 2001;120(6):2013–20.

    Article  CAS  Google Scholar 

  33. de Prost N, Parrot A, Cuquemelle E, Picard C, Cadranel J. Immune diffuse alveolar hemorrhage: a retrospective assessment of a diagnostic scale. Lung. 2013;191(5):559–63.

    Article  Google Scholar 

  34. Picard C, Cadranel J, Porcher R, et al. Alveolar haemorrhage in the immunocompetent host: a scale for early diagnosis of an immune cause. Respiration. 2010;80(4):313–20.

    Article  Google Scholar 

  35. Berden AE, Ferrario F, Hagen EC, et al. Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol. 2010;21(10):1628–36.

    Article  Google Scholar 

  36. Berti, A., Cornec-Le Gall E, Cornec D, et al., Incidence, prevalence, mortality and chronic renal damage of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in a 20-year population-based cohort. Nephrol Dial Transplant. 2018.

    Google Scholar 

  37. Sethi S, VD D’A, Nast CC, et al. A proposal for standardized grading of chronic changes in native kidney biopsy specimens. Kidney Int. 2017;91(4):787–9.

    Article  Google Scholar 

  38. de Prost N, Parrot A, Picard C, et al. Diffuse alveolar haemorrhage: factors associated with in-hospital and long-term mortality. Eur Respir J. 2010;35(6):1303–11.

    Google Scholar 

  39. Lauque D, Cadranel J, Lazor R, et al. 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 (Baltimore). 2000;79(4):222–33.

    Article  CAS  Google Scholar 

  40. Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis. 2003;42(6):1149–53.

    Article  Google Scholar 

  41. Walsh M, Merkel PA, Jayne D. The effects of plasma exchange and reduced-dose glucocorticoids during remission-induction for treatment of severe ANCA-associated vasculitis [abstract]. Arthritis Rheumatol. 2018;70(Suppl 10)

    Google Scholar 

  42. Katsumata Y, Kawaguchi Y, Yamanaka H. Interstitial lung disease with ANCA-associated vasculitis. Clin Med Insights Circ Respir Pulm Med. 2015;9(Suppl 1):51–6.

    PubMed  PubMed Central  Google Scholar 

  43. Alba MA, Flores-Suárez LF, Henderson AG, et al. Interstital lung disease in ANCA vasculitis. Autoimmun Rev. 2017;16(7):722–9.

    Article  Google Scholar 

  44. Borie R, Crestani B. Antineutrophil cytoplasmic antibody-associated lung fibrosis. Semin Respir Crit Care Med. 2018;39(4):465–70.

    Article  Google Scholar 

  45. Kagiyama N, Takayanagi N, Kanauchi T, Ishiguro T, Yanagisawa T, Sugita Y. Antineutrophil cytoplasmic antibody-positive conversion and microscopic polyangiitis development in patients with idiopathic pulmonary fibrosis. BMJ Open Respir Res. 2015;2(1):e000058.

    Article  Google Scholar 

  46. Hozumi H, Oyama Y, Yasui H, et al. Clinical significance of myeloperoxidase-anti-neutrophil cytoplasmic antibody in idiopathic interstitial pneumonias. PLoS One. 2018;13(6):e0199659.

    Article  Google Scholar 

  47. Idiopathic Pulmonary Fibrosis Clinical Research Network, et al. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012;366(21):1968–77.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Thoracic Disease Research Unit, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA

    Marta Casal Moura & Ulrich Specks

Authors
  1. Marta Casal Moura
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ulrich Specks
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ulrich Specks .

Editor information

Editors and Affiliations

  1. Department of Medicine and Surgery, University of Milano-Bicocca, Monza, Monza e Brianza, Italy

    Renato Alberto Sinico

  2. Department of Medicine, Paris Descartes University Hospital Cochin, Paris, France

    Loïc Guillevin

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Moura, M.C., Specks, U. (2020). Lung Involvement in ANCA-Associated Vasculitis. In: Sinico, R., Guillevin, L. (eds) Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-030-02239-6_10

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-02239-6_10

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-02238-9

  • Online ISBN: 978-3-030-02239-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation