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Radiologic Approach for Pulmonary Vasculitis

폐혈관염의 영상의학적 접근

  • Chohee Kim (Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Yoon Kyung Kim (Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Joungho Han (Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 김초희 (성균관대학교 의과대학 삼성서울병원 영상의학과) ;
  • 김윤경 (성균관대학교 의과대학 삼성서울병원 영상의학과) ;
  • 한정호 (성균관대학교 의과대학 삼성서울병원 병리과)
  • Received : 2021.04.29
  • Accepted : 2021.05.25
  • Published : 2021.07.01

Abstract

Vasculitis is a systemic disease, characterized by inflammation of the vascular wall. Although rare, it is sometimes life-threatening due to diffuse pulmonary hemorrhage or acute glomerulonephritis. Besides primary vasculitis, whose cause is unknown, numerous conditions such as autoimmune diseases, drugs, infections, and tumors can cause secondary vasculitis. Vasculitis displays various non-specific symptoms, signs, and laboratory findings; hence, diagnosis of the disease requires integration of various results including clinical features, imaging findings, autoantibody tests, and pathological findings. In this review, we have discussed the clinical, radiologic, and pathological features of vasculitis. Further, we elaborated the imaging findings and differential diagnosis of typical vasculitis that frequently involves the lung and introduced a new international classification of vasculitis, the Diagnostic and Classification Criteria in Vasculitis.

혈관염은 혈관벽의 염증을 특징으로 하는 드문 전신 질환으로 간혹 미만성 폐출혈이나 급성사구체신염으로 생명을 위협하기도 한다. 원인을 알 수 없는 일차성 혈관염도 있지만 자가면역 질환이나 약물, 감염, 종양 등 수많은 원인들에 의해 발생하는 이차성 혈관염도 있으며 분류가 복잡하고 명확한 진단검사가 없어 진단에 어려움이 있다. 또한 다양하고 비특이적인 증상과 징후 및 검사 소견을 보여 혈관염의 진단에는 임상양상, 영상 검사, 자가항체 검사, 병리소견 등 여러 가지 결과를 종합하는 것이 필수적이다. 이 종설에서는 혈관염의 분류와 진단에 중요한 증상 및 징후, 특징적으로 폐를 침범하는 대표적인 폐혈관염의 영상 소견과 감별진단, 그리고 새로운 혈관염 분류를 위한 국제적 연구인 Diagnostic and Classification Criteria in Vasculitis에 대해 소개하고자 한다.

Keywords

References

  1. Fauci AS, Haynes B, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations. Ann Intern Med 1978;89:660-676 https://doi.org/10.7326/0003-4819-89-5-660
  2. O'Sullivan BP. Pulmonary complications of systemic vasculitides. Paediatr Respir Rev 2012;13:37-43 https://doi.org/10.1016/j.prrv.2011.04.002
  3. Casal A, Pereiro T, Valdes L. Pulmonary vasculitis: an update. Arch Bronconeumol (Engl Ed) 2018;54:407-408 https://doi.org/10.1016/j.arbr.2017.11.022
  4. Casal A, Diaz-Garel J, Pereiro T, Toubes ME, Ricoy J, Valdes L. Pulmonary vasculitis. J Thorac Dis 2018;10:5560-5575 https://doi.org/10.21037/jtd.2018.08.117
  5. Ahn JH. Pulmonary vasculitis. Tuberc Respir Dis 2000;48:825-836 https://doi.org/10.4046/trd.2000.48.6.825
  6. The Korean Journal of Medicine. Available at. http://www.ekjm.org/upload/42842889.pdf. Accessed Apr 29, 2021
  7. 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;65:1-11 https://doi.org/10.1002/art.37715
  8. Kim HW, Hur JW. Update on the classification criteria for vasculitis. Korean J Med 2014;87:401-414 https://doi.org/10.3904/kjm.2014.87.4.401
  9. Collard HR, Schwarz MI. Diffuse alveolar hemorrhage. Clin Chest Med 2004;25:583-592, vii https://doi.org/10.1016/j.ccm.2004.04.007
  10. Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;33:1129-1134 https://doi.org/10.1002/art.1780330811
  11. Calabrese LH, Michel BA, Bloch DA, Arend WP, Edworthy SM, Fauci AS, et al. The American College of Rheumatology 1990 criteria for the classification of hypersensitivity vasculitis. Arthritis Rheum 1990;33:1108-1113 https://doi.org/10.1002/art.1780330808
  12. Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990;33:1122-1128 https://doi.org/10.1002/art.1780330810
  13. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101-1107 https://doi.org/10.1002/art.1780330807
  14. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-1100 https://doi.org/10.1002/art.1780330806
  15. Mills JA, Michel BA, Bloch DA, Calabrese LH, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schonlein purpura. Arthritis Rheum 1990;33:1114-1121 https://doi.org/10.1002/art.1780330809
  16. Lightfoot RW Jr, Michel BA, Bloch DA, Hunder GG, Zvaifler NJ, McShane DJ, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum 1990;33:1088-1093 https://doi.org/10.1002/art.1780330805
  17. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187-192 https://doi.org/10.1002/art.1780370206
  18. DCVAS. Research study to develop classification and diagnostic criteria for primary systemic vasculitis. Available at. https://research.ndorms.ox.ac.uk/public/dcvas/index.php. Assessed Apr 29, 2021
  19. Robson J, Grayson P, Ponte C, Suppiah R, Craven A, Khalid S, et al. 110. Classification criteria for the Ancaassociated vasculitides. Rheumatology 2019;58:ii46-ii47 https://doi.org/10.1093/rheumatology/kez058.050
  20. Yoo J, Kim HJ, Ahn SS, Jung SM, Song JJ, Park YB, et al. The utility of the ACR/EULAR 2017 provisional classification criteria for granulomatosis with polyangiitis in Korean patients with antineutrophil cytoplasmic antibody-associated vasculitis. Clin Exp Rheumatol 2018;36 Suppl 111:85-87
  21. Martinez F, Chung JH, Digumarthy SR, Kanne JP, Abbott GF, Shepard JA, et al. Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation. Radiographics 2012;32:51-69 https://doi.org/10.1148/rg.321115060
  22. Hagen EC, Daha MR, Hermans J, Andrassy K, Csernok E, Gaskin G, et al. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR project for ANCA assay standardization. Kidney Int 1998;53:743-753 https://doi.org/10.1046/j.1523-1755.1998.00807.x
  23. Chung MP, Yi CA, Lee HY, Han J, Lee KS. Imaging of pulmonary vasculitis. Radiology 2010;255:322-341 https://doi.org/10.1148/radiol.10090105
  24. 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;15:315-333 https://doi.org/10.1097/00000478-199104000-00001
  25. Lee KS, Kim TS, Fujimoto K, Moriya H, Watanabe H, Tateishi U, et al. Thoracic manifestation of Wegener's granulomatosis: CT findings in 30 patients. Eur Radiol 2003;13:43-51 https://doi.org/10.1007/s00330-002-1422-2
  26. Castaner E, Alguersuari A, Gallardo X, Andreu M, Pallardo Y, Mata JM, et al. When to suspect pulmonary vasculitis: radiologic and clinical clues. Radiographics 2010;30:33-53 https://doi.org/10.1148/rg.301095103
  27. 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;17:3157-3165 https://doi.org/10.1007/s00330-007-0700-4
  28. Mahmoud S, Ghosh S, Farver C, Lempel J, Azok J, Renapurkar RD. Pulmonary vasculitis: spectrum of imaging appearances. Radiol Clin North Am 2016;54:1097-1118 https://doi.org/10.1016/j.rcl.2016.05.007
  29. Nada AK, Torres VE, Ryu JH, Lie JT, Holley KE. Pulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients. Mayo Clin Proc 1990;65:847-856 https://doi.org/10.1016/S0025-6196(12)62575-0
  30. Arulkumaran N, Periselneris N, Gaskin G, Strickland N, Ind PW, Pusey CD, et al. Interstitial lung disease and ANCA-associated vasculitis: a retrospective observational cohort study. Rheumatology (Oxford) 2011;50:2035-2043 https://doi.org/10.1093/rheumatology/ker236
  31. Katsumata Y, Kawaguchi Y, Yamanaka H. Interstitial lung disease with ANCA-associated vasculitis. Clin Med Insights Circ Respir Pulm Med 2015;9:51-56 https://doi.org/10.4137/CCRPM.S23314
  32. Mahr A, Specks U, Jayne D. Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum. Rheumatology (Oxford) 2019;58:1707-1709 https://doi.org/10.1093/rheumatology/kez148
  33. Mohammad AJ, Mortensen KH, Babar J, Smith R, Jones RB, Nakagomi D, et al. Pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: the influence of ANCA subtype. J Rheumatol 2017;44:1458-1467 https://doi.org/10.3899/jrheum.161224