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IgG4-related lung disease with a desquamative interstitial pneumonia pattern radiologically and pathologically
  1. Shingo Tsuneyoshi1,
  2. Yoshiaki Zaizen1,
  3. Masaki Okamoto1,2 and
  4. Tomoaki Hoshino1
  1. 1Division of Respirology, Neurology, and Rheumatology, Department of Medicine, Kurume University School of Medicine, Kurume, Fukuoka, Japan
  2. 2Department of Respirology and Clinical Research Center, National Hospital Organisation Kyushu Medical Center, Fukuoka, Japan
  1. Correspondence to Dr Yoshiaki Zaizen; zaizen_yoshiaki{at}


A man in his 60s exhibited persistent dry cough and dyspnoea, which persisted even after smoking cessation. Chest high-resolution CT showed diffuse ground-glass opacities in the subpleural areas of both lungs. He underwent bronchoscopy, but no definitive diagnosis could be made. Histopathological analysis of the specimen obtained by surgical lung biopsy showed a desquamative interstitial pneumonia (DIP) pattern, with lymphocyte and plasma cell infiltrates in the alveolar septa; the ratio of IgG and IgG4-positive cells was more than 90%. He quit smoking, but the radiological findings worsened. Based on the pathological findings, we diagnosed the patient with DIP due to IgG4-related lung disease. Prednisolone was initiated, and the symptoms and radiological findings improved.

  • Respiratory medicine
  • Interstitial lung disease

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  • Contributors ST wrote the manuscript, cared for patients and performed bronchoscopy; YZ wrote the manuscript, cared for patients and performed a pathological diagnosis; MO wrote a part of the manuscript, treated and cared for patients; TH wrote a part of the manuscript and supervised patient care.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.