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Pulmonary Imaging Findings of Vasculitis

  • Pulmonary Radiology (M Stephens and S Kapur, Section Editors)
  • Published:
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Abstract

Purpose of Review

The purpose of this article is to review the various pulmonary imaging findings that may be present in patients with various types of vasculitis. The review will also go over the potential clinical significance of the various findings.

Recent Findings

The majority of pulmonary findings related to vasculitis revolve around granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Additional etiologies are rare and mostly present as diffuse alveolar hemorrhage when small vessels are involved and aneurysms when medium and large vessels are involved.

Summary

Pulmonary imaging findings related to vasculitis vary widely across etiologies. While many of these manifestations are rare, it is important to be familiar with them as they can cause severe complications.

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References

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  1. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. - PubMed - NCBI. https://www.ncbi.nlm.nih.gov/pubmed/23045170. Accessed Mar 30, 2020.

  2. Frankel SK, Cosgrove GP, Fischer A, Meehan RT, Brown KK. Update in the diagnosis and management of pulmonary vasculitis. Chest. 2006;129(2):452–65.

    PubMed  Google Scholar 

  3. Gómez-Gómez A, Martínez-Martínez MU, Cuevas-Orta E, Bernal-Blanco JM, Cervantes-Ramírez D, Martínez-Martínez R, et al. Pulmonary manifestations of granulomatosis with polyangiitis. Reumatol Clin. 2014;10(5):288–93.

    PubMed  Google Scholar 

  4. Russell B, Mohan S, Chahal R, Carette S, Pagnoux C. Prognostic significance of cavitary lung nodules in granulomatosis with polyangiitis (Wegener’s): a clinical imaging study of 225 patients. Arthritis Care Res. 2018;70(7):1082–9.

    Google Scholar 

  5. Cordier JF, Valeyre D, Guillevin L, Loire R, Brechot JM. Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest. 1990;97(4):906–12.

    CAS  PubMed  Google Scholar 

  6. Bosch X. Microscopic polyangiitis (microscopic polyarteritis) with late emergence of generalised Wegener’s granulomatosis. Ann Rheum Dis. 1999;58(10):644–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  7. Néel A, Espitia-Thibault A, Arrigoni P-P, Volteau C, Rimbert M, Masseau A, et al. Bronchiectasis is highly prevalent in anti-MPO ANCA-associated vasculitis and is associated with a distinct disease presentation. Semin Arthritis Rheum. 2018;48(1):70–6.

    PubMed  Google Scholar 

  8. Shi X, Zhang Y, Lu Y. Risk factors and treatment of pneumothorax secondary to granulomatosis with polyangiitis: a clinical analysis of 25 cases. J Cardiothorac Surg. 2018;13(1):7.

    PubMed  PubMed Central  Google Scholar 

  9. Henes FO, Laudien M, Linsenhoff L, Bremer JP, Oqueka T, Adam G, et al. Accuracy of magnetic resonance imaging for grading of subglottic stenosis in patients with granulomatosis with polyangiitis: correlation with pulmonary function tests and laryngoscopy. Arthritis Care Res. 2018;70(5):777–84.

    Google Scholar 

  10. Kim YK, Lee KS, Chung MP, Han J, Chong S, Chung MJ, et al. Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings. Eur Radiol. 2007;17(12):3157–65.

    PubMed  Google Scholar 

  11. Silva CIS, Müller NL, Fujimoto K, Johkoh T, Ajzen SA, Churg A. Churg-Strauss syndrome: high resolution CT and pathologic findings. J Thorac Imaging. 2005;20(2):74–80.

    PubMed  Google Scholar 

  12. Nakamoto K, Saraya T, Ogawa Y, Ishii H, Takizawa H. Comparison of findings on thoracic computed tomography with the severity and duration of bronchial asthma in patients with eosinophilic granulomatosis with polyangiitis. Respir Med. 2018;139:101–5.

    PubMed  Google Scholar 

  13. 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.

    CAS  PubMed  Google Scholar 

  14. Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005;143(9):632–8.

    PubMed  Google Scholar 

  15. Ríos Blanco JJ, Gómez Cerezo J, Suárez I, Gutiérrez M, Vázquez JJ, Barbado FJ. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Comment on the article by Solans et al. Rheumatol Oxf Engl. 2002;41(8):946–7 author reply 947.

    Google Scholar 

  16. Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Microscopic polyarteritis: presentation, pathology and prognosis. Q J Med. 1985;56(220):467–83.

    CAS  PubMed  Google Scholar 

  17. Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999;42(3):421–30.

    CAS  PubMed  Google Scholar 

  18. Haworth SJ, Savage CO, Carr D, Hughes JM, Rees AJ. Pulmonary haemorrhage complicating Wegener’s granulomatosis and microscopic polyarteritis. Br Med J (Clin Res Ed). 1985;290(6484):1775–8.

    CAS  Google Scholar 

  19. Takahashi K, Hayashi S, Ushiyama O, Sueoka N, Fukuoka M, Nagasawa K. Development of microscopic polyangiitis in patients with chronic airway disease. Lung. 2005;183(4):273–81.

    CAS  PubMed  Google Scholar 

  20. Adu D, Howie AJ, Scott DG, Bacon PA, McGonigle RJ, Micheal J. Polyarteritis and the kidney. Q J Med. 1987;62(239):221–37.

    CAS  PubMed  Google Scholar 

  21. Lauque D, Cadranel J, Lazor R, Pourrat J, Ronco P, Guillevin L, 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.

    CAS  Google Scholar 

  22. Schwarz MI, Mortenson RL, Colby TV, Waldron JA, Lynch DA, Hutt MP, et al. Pulmonary capillaritis. The association with progressive irreversible airflow limitation and hyperinflation. Am Rev Respir Dis. 1993;148(2):507–11.

    CAS  PubMed  Google Scholar 

  23. Brugiere O, Raffy O, Sleiman C, Groussard O, Rothchild E, Mellot F, et al. Progressive obstructive lung disease associated with microscopic polyangiitis. Am J Respir Crit Care Med. 1997;155(2):739–42.

    CAS  PubMed  Google Scholar 

  24. Verburgh CA, Bruijn JA, Daha MR, van Es LA. Sequential development of anti-GBM nephritis and ANCA-associated Pauci-immune glomerulonephritis. Am J Kidney Dis Off J Natl Kidney Found. 1999;34(2):344–8.

    CAS  Google Scholar 

  25. Beechler CR, Enquist RW, Hunt KK, Ward GW, Knieser MR. Immunofluorescence of transbronchial biopsies in Goodpasture’s syndrome. Am Rev Respir Dis. 1980;121(5):869–72.

    CAS  PubMed  Google Scholar 

  26. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 52–1993. A 17-year-old girl with massive hemoptysis and acute oliguric renal failure. N Engl J Med. 1993;329(27):2019–26.

  27. García-Rostan y Pérez GM, García Bragado F, Puras Gil AM. Pulmonary hemorrhage and antiglomerular basement membrane antibody-mediated glomerulonephritis after exposure to smoked cocaine (crack): a case report and review of the literature. Pathol Int. 1997;47(10):692–7.

    PubMed  Google Scholar 

  28. Lazor R, Bigay-Gamé L, Cottin V, Cadranel J, Decaux O, Fellrath JM, et al. Alveolar hemorrhage in anti-basement membrane antibody disease: a series of 28 cases. Medicine (Baltimore). 2007;86(3):181–93.

    Google Scholar 

  29. Lombard CM, Colby TV, Elliott CG. Surgical pathology of the lung in anti-basement membrane antibody-associated Goodpasture’s syndrome. Hum Pathol. 1989;20(5):445–51.

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  31. Olson JC, Kelly KJ, Pan CG, Wortmann DW. Pulmonary disease with hemorrhage in Henoch-Schöenlein purpura. Pediatrics. 1992;89(6 Pt 2):1177–81.

    CAS  PubMed  Google Scholar 

  32. Vats KR, Vats A, Kim Y, Dassenko D, Sinaiko AR. Henoch-Schönlein purpura and pulmonary hemorrhage: a report and literature review. Pediatr Nephrol Berl Ger. 1999;13(6):530–4.

    CAS  Google Scholar 

  33. Markus HS, Clark JV. Pulmonary haemorrhage in Henoch-Schönlein purpura. Thorax. 1989;44(6):525–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  34. Shichiri M, Tsutsumi K, Yamamoto I, Ida T, Iwamoto H. Diffuse intrapulmonary hemorrhage and renal failure in adult Henoch-Schönlein purpura. Am J Nephrol. 1987;7(2):140–2.

    CAS  PubMed  Google Scholar 

  35. Kathuria S, Cheifec G. Fatal pulmonary Henöch-Schonlein syndrome. Chest. 1982;82(5):654–6.

    CAS  PubMed  Google Scholar 

  36. Yokose T, Aida J, Ito Y, Ogura M, Nakagawa S, Nagai T. A case of pulmonary hemorrhage in Henoch-Schönlein purpura accompanied by polyarteritis nodosa in an elderly man. Respir Int Rev Thorac Dis. 1993;60(5):307–10.

    CAS  Google Scholar 

  37. Erkan F, Gül A, Tasali E. Pulmonary manifestations of Behçet’s disease. Thorax. 2001;56(7):572–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  38. Hiller N, Lieberman S, Chajek-Shaul T, Bar-Ziv J, Shaham D. Thoracic manifestations of Behçet disease at CT. Radiogr Rev Publ Radiol Soc N Am Inc. 2004;24(3):801–8.

    Google Scholar 

  39. Tunaci M, Ozkorkmaz B, Tunaci A, Gül A, Engin G, Acunaş B. CT findings of pulmonary artery aneurysms during treatment for Behçet’s disease. AJR Am J Roentgenol. 1999;172(3):729–33.

    CAS  PubMed  Google Scholar 

  40. Zamora MR, Warner ML, Tuder R, Schwarz MI. Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome. Medicine (Baltimore). 1997;76(3):192–202.

    CAS  Google Scholar 

  41. Barile LA, Jara LJ, Medina-Rodriguez F, García-Figueroa JL, Miranda-Limón JM. Pulmonary hemorrhage in systemic lupus erythematosus. Lupus. 1997;6(5):445–8.

    CAS  PubMed  Google Scholar 

  42. Santos-Ocampo AS, Mandell BF, Fessler BJ. Alveolar hemorrhage in systemic lupus erythematosus: presentation and management. Chest. 2000;118(4):1083–90.

    CAS  PubMed  Google Scholar 

  43. Naniwa T, Banno S, Sugiura Y, Yokota K, Oosawa T, Maeda S, et al. Pulmonary-renal syndrome in systemic sclerosis: a report of three cases and review of the literature. Mod Rheumatol. 2007;17(1):37–44.

    PubMed  Google Scholar 

  44. Griffin MT, Robb JD, Martin JR. Diffuse alveolar haemorrhage associated with progressive systemic sclerosis. Thorax. 1990;45(11):903–4.

    CAS  PubMed  PubMed Central  Google Scholar 

  45. Bar J, Ehrenfeld M, Rozenman J, Perelman M, Sidi Y, Gur H. Pulmonary-renal syndrome in systemic sclerosis. Semin Arthritis Rheum. 2001;30(6):403–10.

    CAS  PubMed  Google Scholar 

  46. Vázquez-Del Mercado M, Mendoza-Topete A, Best-Aguilera CR, Garcia-De La Torre I. Diffuse alveolar hemorrhage in limited cutaneous systemic sclerosis with positive perinuclear antineutrophil cytoplasmic antibodies. J Rheumatol. 1996;23(10):1821–3.

    PubMed  Google Scholar 

  47. Endo H, Hosono T, Kondo H. Antineutrophil cytoplasmic autoantibodies in 6 patients with renal failure and systemic sclerosis. J Rheumatol. 1994;21(5):864–70.

    CAS  PubMed  Google Scholar 

  48. Casari S, Haeney M, Farrand S, Herrick A. Antineutrophil cytoplasmic antibodies a “red flag” in patients with systemic sclerosis. J Rheumatol. 2002;29(12):2666–7.

    PubMed  Google Scholar 

  49. Wutzl AL, Foley RN, O’Driscoll BR, Reeve RS, Chisholm R, Herrick AL. Microscopic polyangiitis presenting as pulmonary-renal syndrome in a patient with long-standing diffuse cutaneous systemic sclerosis and antibodies to myeloperoxidase. Arthritis Rheum. 2001;45(6):533–6.

    CAS  PubMed  Google Scholar 

  50. Schwarz MI, Zamora MR, Hodges TN, Chan ED, Bowler RP, Tuder RM. Isolated pulmonary capillaritis and diffuse alveolar hemorrhage in rheumatoid arthritis and mixed connective tissue disease. Chest. 1998;113(6):1609–15.

    CAS  PubMed  Google Scholar 

  51. Schwarz MI, Sutarik JM, Nick JA, Leff JA, Emlen JW, Tuder RM. Pulmonary capillaritis and diffuse alveolar hemorrhage. A primary manifestation of polymyositis. Am J Respir Crit Care Med. 1995;151(6):2037–40.

    CAS  PubMed  Google Scholar 

  52. Genta MS, Genta RM, Gabay C. Systemic rheumatoid vasculitis: a review. Semin Arthritis Rheum. 2006;36(2):88–98.

    PubMed  Google Scholar 

  53. Gómez-Tello V, Oñoro-Cañaveral JJ, de la Casa Monje RM, et al. Diffuse recidivant alveolar hemorrhage in a patient with hepatitis C virus-related mixed cryoglobulinemia. Intensive Care Med. 1999;25(3):319–22.

    PubMed  Google Scholar 

  54. Kramer J, Hennig H, Lensing C, Krüger S, Helmchen U, Steinhoff J, et al. Multi-organ affecting CMV-associated cryoglobulinemic vasculitis. Clin Nephrol. 2006;66(4):284–90.

    CAS  PubMed  Google Scholar 

  55. Silva F, Pinto C, Barbosa A, Borges T, Dias C, Almeida J. New insights in cryoglobulinemic vasculitis. J Autoimmun. 2019;105:102313.

    CAS  PubMed  Google Scholar 

  56. Choi HK, Merkel PA, Walker AM, Niles JL. Drug-associated antineutrophil cytoplasmic antibody-positive vasculitis: prevalence among patients with high titers of antimyeloperoxidase antibodies. Arthritis Rheum. 2000;43(2):405–13.

    CAS  PubMed  Google Scholar 

  57. Kudoh Y, Kuroda S, Shimamoto K, Iimura O. Propylthiouracil-induced rapidly progressive glomerulonephritis associated with antineutrophil cytoplasmic autoantibodies. Clin Nephrol. 1997;48(1):41–3.

    CAS  PubMed  Google Scholar 

  58. Short AK, Lockwood CM. Antigen specificity in hydralazine associated ANCA positive systemic vasculitis. QJM Mon J Assoc Phys. 1995;88(11):775–83.

    CAS  Google Scholar 

  59. Lee YJ, Park SS, Kim SY, Lee JY, Koo HK, Yoon HI. A case of systemic polyarteritis nodosa involving bronchial artery. Sarcoidosis Vasc Diffuse Lung Dis Off J WASOG. 2010;27(2):164–8.

    CAS  Google Scholar 

  60. Escalon JG, Wu X, Drexler IR, Lief L, Plataki M, Bender M, et al. Rare case of pulmonary involvement in an adult with Kawasaki disease. Clin Imaging. 2018;47:1–3.

    PubMed  Google Scholar 

  61. Singh S, Gupta A, Jindal AK, Gupta A, Suri D, Rawat A, et al. Pulmonary presentation of Kawasaki disease—a diagnostic challenge. Pediatr Pulmonol. 2018;53(1):103–7.

    PubMed  Google Scholar 

  62. Yang J, Peng M, Shi J, Zheng W, Yu X. Pulmonary artery involvement in Takayasu’s arteritis: diagnosis before pulmonary hypertension. BMC Pulm Med. 2019;19(1):225.

    PubMed  PubMed Central  Google Scholar 

  63. He Y, Lv N, Dang A, Cheng N. Pulmonary artery involvement in patients with Takayasu arteritis. J Rheumatol. 2020;47(2):264–72.

    CAS  PubMed  Google Scholar 

  64. Kramer MR, Melzer E, Nesher G, Sonnenblick M. Pulmonary manifestations of temporal arteritis. Eur J Respir Dis. 1987;71(5):430–3.

    CAS  PubMed  Google Scholar 

  65. Perruzza M, Fusha E, Cameli P, Capecchi PL, Selvi E, Gentili F, et al. Pleuroparenchymal fibroelastosis (PPFE) associated with giant cell arteritis: a coincidence or a novel phenotype? Respir Med Case Rep. 2019;27:100843.

    CAS  PubMed  PubMed Central  Google Scholar 

  66. Konishi C, Nakagawa K, Nakai E, et al. Interstitial lung disease as an initial manifestation of giant cell arteritis. Intern Med. 2017;56(19):2633–2637. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5658531/. Accessed Mar 30, 2020.

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Stephens, M.J., Aghayev, A. Pulmonary Imaging Findings of Vasculitis. Curr Pulmonol Rep 9, 143–150 (2020). https://doi.org/10.1007/s13665-020-00263-x

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