Skip to main content
SpringerLink
Log in

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

  • original article
  • Published:
Wiener klinische Wochenschrift Aims and scope Submit manuscript

Summary

Background

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.

Methods

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.

Results

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.

Conclusions

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  16. Cesario A, Meacci E, Mulè A, Margaritora S. Wegener disease mimicking central lung cancer. Eur J Cardiothorac Surg. 2002;22(4):626.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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. 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.

    Article  CAS  PubMed  Google Scholar 

  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.

    PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

Download references

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.

Author information

Authors and Affiliations

  1. Department of Radiology, Bulent Ecevit University School of Medicine, 67100, Zonguldak, Turkey

    Serkan Guneyli MD

  2. Department of Radiology, Ege University School of Medicine, Izmir, Turkey

    Naim Ceylan MD,  Selen Bayraktaroglu MD,  Recep Savas MD &  Hudaver Alper MD

  3. Department of Internal Medicine, Ege University School of Medicine, Izmir, Turkey

    Sercan Gucenmez MD &  Kenan Aksu MD

  4. Division of Nuclear Medicine, Egerad Diagnostic Imaging Center, Izmir, Turkey

    Kenan Kocacelebi MD

  5. Department of Radiology, Mevlana University School of Medicine, Konya, Turkey

    Turker Acar MD

Authors
  1. Serkan Guneyli MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Naim Ceylan MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Selen Bayraktaroglu MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sercan Gucenmez MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Kenan Aksu MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Kenan Kocacelebi MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Turker Acar MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Recep Savas MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Hudaver Alper MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Serkan Guneyli MD.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Guneyli, S., Ceylan, N., Bayraktaroglu, S. et al. Imaging findings of pulmonary granulomatosis with polyangiitis (Wegener’s granulomatosis): lesions invading the pulmonary fissure, pleura or diaphragm mimicking malignancy. Wien Klin Wochenschr 128, 809–815 (2016). https://doi.org/10.1007/s00508-015-0747-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00508-015-0747-1

Keywords

Search

Navigation