Middle lobe syndrome as the pulmonary manifestation of primary Sjögren's syndrome

Horng-An Chen, Shinn-Liang Lai, Wei-Kang Kwang, Juhn-Cherng Liu, Chun-Hsiung Chen and De-Feng Huang
Med J Aust 2006; 184 (6): 294-295.

Middle lobe syndrome — recurrent atelectasis and/or bronchiectasis involving the right middle lobe and/or lingula — has, up to now, not been reported as the pulmonary manifestation of primary Sjögren’s syndrome. We describe a patient in whom lymphocytic bronchiolitis in the atelectatic lobes was proved histologically from two separate transbronchial biopsies. The atelectasis responded well to glucocorticoid treatment, suggesting that the peribronchiolar lymphocytic infiltrates may have played an important role in the development of middle lobe syndrome in this patient.

A 53-year-old woman was admitted to our hospital in 2003 with symptoms, for the past 3 days, of shortness of breath, cough, and blood-tinged sputum. Primary Sjögren’s syndrome had been diagnosed in 2002, based on the presence of xerostomia, keratoconjunctivitis sicca, anti-Ro/La antibody, and rheumatoid factor, as well as a positive Schirmer’s test and the results of sialoscintigraphy.

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  • Horng-An Chen1
  • Shinn-Liang Lai2
  • Wei-Kang Kwang3
  • Juhn-Cherng Liu4
  • Chun-Hsiung Chen5
  • De-Feng Huang6

  • Taipei Veterans General Hospital and National Yang-Ming University, Taipei, Taiwan.


Competing interests:

None identified.

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