Skip to main content

Advertisement

Log in

A case of primary Sjögren’s syndrome with pulmonary-limited Wegener’s granulomatosis

  • Case Report
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

A 60-year-old woman had a history of dyspnea for 5–6 weeks. The chest radiograph and computed tomography scans revealed bilateral patchy reticulonodular pattern. The patient had positive test results for antineutrophil cytoplasmic antibody against proteinase-3 (c-ANCA), antinuclear antibody and anti-Ro antibody. According to European Study Group on Classification Criteria for Sjögren’s Syndrome, the patient was diagnosed as primary Sjögren’s syndrome based on the presence of clinical features, positive findings on Schirmer’s test and parotis scintigraphy. Lung biopsy obtained by wedge resection showed granulomatous inflammation with extensive multinuclear giant cells involving the lung parenchyma and vascular structures. There was neither upper airway nor renal involvement. Thus, the patient was simultaneously diagnosed as pulmonary-limited Wegener’s granulomatosis. With this unique case, we would like to emphasize that the awareness of ANCA-associated vasculitis as a diagnostic possibility in primary Sjögren’s syndrome is important during the work-up of lung lesions.

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

Access this article

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

References

  1. Fox RI (2005) Sjögren’s syndrome. Lancet 366:321–331. doi:10.1016/S0140-6736(05)66990-5

    Article  CAS  PubMed  Google Scholar 

  2. Yazisiz V, Avci AB, Erbasan F et al (2009) Diagnostic performance of minor salivary gland biopsy, serological and clinical data in Sjögren’s syndrome: a retrospective analysis. Rheumatol Int 29:403–409. doi:10.1007/s00296-008-0698-1

    Article  PubMed  Google Scholar 

  3. Mellgren SI, Göransson LG, Omdal R (2007) Primary Sjögren’s syndrome associated neuropathy. Can J Neurol Sci 34:280–287

    PubMed  Google Scholar 

  4. Guillevin L, Pagnoux C (2008) Classification of systemic vasculitides. Rev Prat 58:480–486

    PubMed  Google Scholar 

  5. Karargyris G, Stamatopoulos K, Kittas C et al (2000) Sjögren’s syndrome co-existing with limited Wegener’s granulomatosis. Rheumatology (Oxford) 39:805–806. doi:10.1093/rheumatology/39.7.805

    Article  CAS  Google Scholar 

  6. Böttinger EP, Niles JL, Collins AB et al (1992) Antineutrophil cytoplasmic autoantibody-associated vasculitis presenting as Sjögren’s syndrome. Arthritis Rheum 35:1373–1376

    PubMed  Google Scholar 

  7. Vitali C, Bombardieri S, Jonsson R et al (2002) European Study Group on Classification Criteria for Sjögren’s Syndrome. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 61:554–558. doi:10.1136/ard.61.6.554

    Article  CAS  PubMed  Google Scholar 

  8. Merkel PA, Polisson RP, Chang Y et al (1997) Prevalence of antineutrophil cytoplasmic antibodies in a large inception cohort of patients with connective tissue disease. Ann Intern Med 126:866–873

    CAS  PubMed  Google Scholar 

  9. Font J, Ramos-Casals M, Cervera R et al (1998) Antineutrophil cytoplasmic antibodies in primary Sjögren’s syndrome: prevalence and clinical significance. Br J Rheumatol 37:1287–1291. doi:10.1093/rheumatology/37.12.1287

    Article  CAS  PubMed  Google Scholar 

  10. Sato Y, Sakamoto S, Kotajima Y et al (2005) A case of Sjögren’s syndrome with Wegener’s granulomatosis-like pulmonary involvement. Allergol Int 54:339–442. doi:10.2332/allergolint.54.339

    Article  Google Scholar 

  11. Parambil JG, Myers JL, Lindell RM et al (2006) Interstitial lung disease in primary Sjögren syndrome. Chest 130:1489–1495. doi:10.1378/chest.130.5.1489

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

This case report was supported by Akdeniz University Scientific Research Projects Unit.

Conflict of interest statement

The authors have declared no conflicts of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Veli Yazisiz.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Yazisiz, V., Ozbudak, I.H., Nizam, I. et al. A case of primary Sjögren’s syndrome with pulmonary-limited Wegener’s granulomatosis. Rheumatol Int 30, 1235–1238 (2010). https://doi.org/10.1007/s00296-009-1045-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-009-1045-x

Keywords

Navigation