Abstract
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.
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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.
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.
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.
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.
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.
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.
Kluth DC, Rees AJ. Anti-glomerular basement membrane disease. J Am Assoc Nephrol. 1999;10:2446–53.
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.
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.
Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539–51.
Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J. 2004;24:162–70.
Beddy P, Babar J, Devaraj A. A practical approach to cystic lung disease on HRCT. Insights Imaging. 2011;2(1):1–7.
Zinn DJ, Chakraborty R, Allen CE. Langerhans cell histiocytosis: emerging insights and clinical implications. Oncology (Williston Park). 2016;30(2):122–32. 139.
Harari S, Torre O, Cassandro R, Moss J. The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J. 2015;46(5):1471–85.
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.
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.
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Sivasothy, P., Thillai, M. (2018). Vasculitis and Rare Lung Diseases. In: Hart, S., Greenstone, M. (eds) Foundations of Respiratory Medicine. Springer, Cham. https://doi.org/10.1007/978-3-319-94127-1_17
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DOI: https://doi.org/10.1007/978-3-319-94127-1_17
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