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

Rheumatol Int. 2010 Jul;30(9):1235-8. doi: 10.1007/s00296-009-1045-x. Epub 2009 Jul 4.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Antinuclear
  • Female
  • Follow-Up Studies
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / immunology
  • Granulomatosis with Polyangiitis / pathology
  • Humans
  • Length of Stay
  • Lung / pathology*
  • Middle Aged
  • Myeloblastin
  • Patient Discharge
  • Sjogren's Syndrome
  • Time Factors
  • Treatment Outcome


  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Antinuclear
  • SS-A antibodies
  • Myeloblastin