Two cases of Sjögren's syndrome with multiple bullae

Intern Med. 2002 Feb;41(2):124-8. doi: 10.2169/internalmedicine.41.124.

Abstract

Here, we report two rare female cases of Sjögren's syndrome with multiple bullae, involving a 66- and a 51-year-old. Neither had any obvious pulmonary complaint. Chest radiographs and high-resolution CT (HRCT) scans showed interstitial linear and nodular opacities and multiple bullae. In the first case spirometry indicated an obstructive change judged by FEV1.0 and V50/V25. In both cases, histologic examination of the lung revealed thickening of alveolar septa and interstitial mononuclear cell infiltration. In the first case the bullae decreased in size with corticosteroid treatment. Airway narrowing due to peribronchiolar mononuclear cell infiltration causes a check-valve mechanism, which may lead to bullae formation. Although a rare occurrence, it is important to recognize that cystic or bullous lung disease can accompany Sjögren's syndrome.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Airway Obstruction / diagnostic imaging
  • Airway Obstruction / etiology
  • Airway Obstruction / pathology
  • Autoimmune Diseases / diagnostic imaging
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / pathology*
  • Blister / diagnostic imaging
  • Blister / etiology*
  • Blister / physiopathology
  • Female
  • Forced Expiratory Volume
  • Humans
  • Hypertrophy
  • Immunosuppressive Agents / therapeutic use
  • Lung Diseases / diagnostic imaging
  • Lung Diseases / etiology*
  • Lung Diseases / physiopathology
  • Middle Aged
  • Monocytes / pathology
  • Prednisolone / therapeutic use
  • Pulmonary Alveoli / pathology
  • Sjogren's Syndrome / diagnostic imaging
  • Sjogren's Syndrome / drug therapy
  • Sjogren's Syndrome / pathology*
  • Spirometry
  • Tomography, X-Ray Computed

Substances

  • Immunosuppressive Agents
  • Prednisolone