Wiener klinische Wochenschrift

, Volume 128, Issue 21–22, pp 809–815 | Cite as

Imaging findings of pulmonary granulomatosis with polyangiitis (Wegener’s granulomatosis): lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy

  • Serkan Guneyli
  • Naim Ceylan
  • Selen Bayraktaroglu
  • Sercan Gucenmez
  • Kenan Aksu
  • Kenan Kocacelebi
  • Turker Acar
  • Recep Savas
  • Hudaver Alper
original article



Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis), in which pulmonary involvement often predominates, is a multisystem granulomatous, necrotizing vasculitis that affects small and medium-sized vessels. In this study we evaluated various radiological findings of pulmonary GPA and focused on spiculated pulmonary lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy.


This retrospective study included 48 patients, aged 28–73 (mean, 47.3) years, who showed either histopathological diagnosis of GPA (n = 39) or elevated levels of the cytoplasmic anti-neutrophilic cytoplasmic antibody serum marker (n = 9) between January 2003 and December 2013. All patients received a chest computed tomography (CT), and the types of pulmonary lesions were defined and evaluated.


Among the 48 patients, 33 had abnormal pulmonary findings on CT. The most commonly detected pulmonary lesion types were nodules and masses (n = 126) observed in 24 patients. Cavitation, necrosis, spiculation and invasion of the fissure, pleura or diaphragm were observed in 14, 9, 10 and 6 patients, respectively. Consolidation was found in 14 patients and thickening of bronchial wall in 8 patients.


Pulmonary lesion types of GPA have a wide spectrum, potentially mimicking a high number of diseases including malignancy, infection and noninfectious inflammatory diseases. A spiculated lung lesion invading the fissure, pleura or diaphragm is mostly present in malignancy, but it can be also seen in GPA.


Computed tomography Granulomatous Invasion Pulmonary Vasculitis 


Ethical standards statement

All procedures followed were in accordance with the ethical standards of the Turkish National Medical Ethics Committee and with the Helsinki Declaration of 1975, as revised in 2008.

Statement of informed consent

Informed consent was obtained from all patients for being included in the study.

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Rodrigues CE, Callado MR, Nobre CA, Moura FE, Vieira RM, de Albuquerque LA, et al. Wegener’s granulomatosis: prevalence of the initial clinical manifestations—report of six cases and review of the literature. Rev Bras Reumatol. 2010;50(2):150–64.CrossRefPubMedGoogle Scholar
  2. 2.
    Ananthakrishnan L, Sharma N, Kanne JP. Wegener’s granulomatosis in the chest: high resolution CT findings. AJR Am J Roentgenol. 2009;192:676–82. doi:10.2214.CrossRefPubMedGoogle Scholar
  3. 3.
    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.CrossRefPubMedGoogle Scholar
  4. 4.
    Mueller A, Holl-Ulrich K, Lamprecht P, Gross WL. Germinal centre-like structures in Wegener’s granuloma: the morphological basis for autoimmunity?. Rheumatology. 2008;47:1111–3. doi:10.1093.CrossRefPubMedGoogle Scholar
  5. 5.
    Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology. 2008;246:697–722. doi:10.1148.CrossRefPubMedGoogle Scholar
  6. 6.
    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. doi:10.1148.CrossRefPubMedGoogle Scholar
  7. 7.
    Campainha S, Gonçalves M, Tavares V, Castelões P, Marinho A, Neves S. Granulomatosis with polyangiitis initially misdiagnosed as lung cancer. Rev Port Pneumol. 2013;19:45–8. doi:10.1016.CrossRefPubMedGoogle Scholar
  8. 8.
    King TE. A lung biopsy is necessary in the management of ANCA-positive patients with chest roentgenographic abnormarmalities. Sarcoidosis Vasc Diffuse Lung Dis. 1996;13(3):238–40.PubMedGoogle Scholar
  9. 9.
    Schnabel A, HollUlrich K, Dalhoff K, Reuter M, Gross WL. Efficacy of transbronchial biopsy in pulmonary vasculitides. Eur Respir J. 1997;10(12):2738–43.CrossRefPubMedGoogle Scholar
  10. 10.
    Awasthi A, Malhotra P, Gupta N, Gupta D, Rajwanshi A. Pitfalls in the diagnosis of Wegener’s granulomatosis on fine needle aspiration cytology. Cytopathology. 2007;18(1):8–12.CrossRefPubMedGoogle Scholar
  11. 11.
    Miyahara N, Eda R, Umemori Y, Murakami T, Kunichika N, Makihata K, et al. Pulmonary lymphoma of large B-cell type mimicking Wegener’s granulomatosis. Intern Med. 2001;40(8):786–90.CrossRefPubMedGoogle Scholar
  12. 12.
    Cohen Y, Amir G, Schii G, Amariglio N, Polliack A. Rapidly progressive diffuse large B-cell lymphoma with initial clinical presentation mimicking seronegative Wegener’s granulomatosis. Eur J Haematol. 2004;73(2):134–8.CrossRefPubMedGoogle Scholar
  13. 13.
    Mark EJ, Matsubara O, TanLiu NS, Fienberg R. The pulmonary biopsy in the early diagnosis of Wegener’s (pathergic) granulomatosis: a study based on 35 open lung biopsies. Hum Pathol. 1998;19(9):1065–71.CrossRefGoogle Scholar
  14. 14.
    Lohrmann C, Uhl M, Kotter E, Burger D, Ghanem N, Langer M. Pulmonary manifestations of Wegener granulomatosis: CT findings in 57 patients and a review of the literature. Eur J Radiol. 2005;53(3):471–7.CrossRefPubMedGoogle Scholar
  15. 15.
    Rau AR, Kimi H, Pai RR, Shetty AB. Wegener’s granulomatosis mimicking paraneoplastic syndrome. Indian J Chest Dis Allied Sci. 2008;50(3):295–7.PubMedGoogle Scholar
  16. 16.
    Cesario A, Meacci E, Mulè A, Margaritora S. Wegener disease mimicking central lung cancer. Eur J Cardiothorac Surg. 2002;22(4):626.CrossRefPubMedGoogle Scholar
  17. 17.
    Langford CA, Sneller MC, Hallahan CW, Hoffman GS, Kammerer WA, Talar-Williams C, et al. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis. Arthritis Rheum. 1996;39(10):1754–60.CrossRefPubMedGoogle Scholar
  18. 18.
    Attali P, Begum R, Ban Romdhane H, Valeyre D, Guillevin L, Brauner MW. Pulmonary Wegener’s granulomatosis: changes at follow-up CT. Eur Radiol. 1998;8(6):1009–113.CrossRefPubMedGoogle Scholar
  19. 19.
    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 Rad. 2003;13(1):43–51.Google Scholar
  20. 20.
    Reuter M, Schnabel A, Wesner F, Tetzlaff K, Risheng Y, Gross WL, et al. Pulmonary Wegener’s granulomatosis: correlation between high-resolution CT findings and clinical scoring of disease activity. Chest. 1998;114(2):500–6.CrossRefPubMedGoogle Scholar
  21. 21.
    Komócsi A, Reuter M, Heller M, Muraközi H, Gross WL, Schnabel A. Active disease and residual damage in treated Wegener’s granulomatosis: an observational study using pulmonary high-resolution computed tomography. Eur Radiol. 2003;13(1):36–42.PubMedGoogle Scholar
  22. 22.
    Mark EJ, Flieder DB, Matsubara O. Treated Wegener’s granulomatosis: distinctive pathological findings in the lungs of 20 patients and what they tell us about the natural history of the disease. Hum Pathol. 1997;28(4):450–8.CrossRefPubMedGoogle Scholar
  23. 23.
    Sheehan RE, Flint JD, Müller NL. Computed tomography features of the thoracic manifestations of Wegener granulomatosis. J Thorac Imaging. 2003;18(1):34–41.CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Wien 2015

Authors and Affiliations

  • Serkan Guneyli
    • 1
  • Naim Ceylan
    • 2
  • Selen Bayraktaroglu
    • 2
  • Sercan Gucenmez
    • 3
  • Kenan Aksu
    • 3
  • Kenan Kocacelebi
    • 4
  • Turker Acar
    • 5
  • Recep Savas
    • 2
  • Hudaver Alper
    • 2
  1. 1.Department of RadiologyBulent Ecevit University School of MedicineZonguldakTurkey
  2. 2.Department of RadiologyEge University School of MedicineIzmirTurkey
  3. 3.Department of Internal MedicineEge University School of MedicineIzmirTurkey
  4. 4.Division of Nuclear MedicineEgerad Diagnostic Imaging CenterIzmirTurkey
  5. 5.Department of RadiologyMevlana University School of MedicineKonyaTurkey

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