Vasculitis and Rare Lung Diseases

  • Pasupathy SivasothyEmail author
  • Muhunthan Thillai


Common forms of vasculitides affecting the lung are granulomatosis with polyangiitis (Wegener’s granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg Strauss Syndrome). These two conditions, together with microscopic polyangiitis, are called antineutrophilic cytoplasmic antibodies-associated vasculitides (AAV). These disorders are multisystem diseases and are frequently associated with ENT, renal, neurological, and cutaneous manifestations and the clinical features of these syndromes may overlap. Autoimmune lung diseases—including anti-glomerular basement disease, pulmonary alveolar proteinosis, and IgG4 disease—are rarer lung diseases. Their management and treatment strategies (especially in refractory disease) are similar to AAV. Rare true cystic lung diseases include pulmonary Langerhans cell histiocytosis, lymphoid interstitial pneumonia, lymphangioleiomyomatosis, and Birt Hogg Dubé syndrome.


Antineutrophilic cytoplasmic antibody associated vasculitis Granulomatosis with polyangiitis Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis Behçet’s disease Takayasu’s arteritis Anti GBM disease Pulmonary alveolar proteinosis IgG4 disease Idiopathic pulmonary haemosiderosis Cystic lung diseases Langerhans cell histiocytosis Lymphangioleiomyomatosis 


  1. 1.
    Rare and orphan lung disease. In: Gibson GJ, Loddenkemper R, Lundbäck B, Sibille Y, editors. European lung white book. Sheffield, UK: European Respiratory Society; 2013.Google Scholar
  2. 2.
    Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11.CrossRefPubMedGoogle Scholar
  3. 3.
    Ntatsaki E, Carruthers D, Chakravarty K, D’Cruz D, Harper L, Jayne D, et al. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014;53(12):2306–9.CrossRefGoogle Scholar
  4. 4.
    Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68(3):310–7.CrossRefPubMedGoogle Scholar
  5. 5.
    Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2009;68(3):318–23.CrossRefPubMedGoogle Scholar
  6. 6.
    Chae EJ, Do KH, Seo JH, Park SH, Kang JW, Jang YM, et al. Radiologic and clinical findings of Behçet disease: comprehensive review of multisystemic involvement. Radiographics. 2008;28(5):e31.CrossRefPubMedGoogle Scholar
  7. 7.
    Kluth DC, Rees AJ. Anti-glomerular basement membrane disease. J Am Assoc Nephrol. 1999;10:2446–53.Google Scholar
  8. 8.
    Pedchenko V, Bondar O, Fogo AB, Vanacore R, Voziyan P, Kitching AR, et al. Molecular architecture of the Goodpasture autoantigen in anti-GBM nephritis. N Engl J Med. 2010;363(4):343–54.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Borie R, Danel C, Debray MP, Taille C, Dombret MC, Aubier M, et al. Pulmonary alveolar proteinosis. Eur Respir Rev. 2011;20(120):98–107.CrossRefPubMedGoogle Scholar
  10. 10.
    Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539–51.CrossRefPubMedGoogle Scholar
  11. 11.
    Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J. 2004;24:162–70.CrossRefPubMedGoogle Scholar
  12. 12.
    Beddy P, Babar J, Devaraj A. A practical approach to cystic lung disease on HRCT. Insights Imaging. 2011;2(1):1–7.CrossRefPubMedGoogle Scholar
  13. 13.
    Zinn DJ, Chakraborty R, Allen CE. Langerhans cell histiocytosis: emerging insights and clinical implications. Oncology (Williston Park). 2016;30(2):122–32. 139.Google Scholar
  14. 14.
    Harari S, Torre O, Cassandro R, Moss J. The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 2015;46(5):1471–85.CrossRefPubMedGoogle Scholar
  15. 15.
    Kokosi MA, Nicholson AG, Hansell DM, Wells AU. Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP. Respirology. 2016;21(4):600–14.CrossRefPubMedGoogle Scholar
  16. 16.
    Menko FH, van Steensel MA, Giraud S, Friis-Hansen L, Richard S, Ungari S, et al. Birt-Hogg-Dubé syndrome: diagnosis and management. Lancet Oncol. 2009;10(12):1199–206.CrossRefPubMedGoogle Scholar

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© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of MedicineCambridge University Hospitals Foundation TrustCambridgeUK
  2. 2.Cambridge Interstitial Lung Disease UnitPapworth HospitalCambridgeshireUK

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