Cavitating Lung Disease: A Novel Presentation of IgG4-Related Disease

Am J Case Rep. 2015 Jul 21:16:478-82. doi: 10.12659/AJCR.894015.

Abstract

Background: Immunoglobulin (Ig) G4-related disease, previously referred to as IgG4-related sclerosing disease or hyper-IgG4 disease, may occur in the lung, involving alveolar parenchyma, airways, and pleura. Various pulmonary manifestations of IgG4-related disease have been reported, but to the best of our knowledge a cavitating lung disease has not been reported previously.

Case report: We describe a 60-year-old man who presented with hemoptysis and cavitating lung disease with clinical, laboratory, and histopathologic findings compatible with IgG4-related disease. Other potential causes of cavitation were excluded. Treatment was initiated with oral prednisone and subsequently mycophenolate mofetil was added. Follow-up 1 year later shows stable pulmonary function with complete resolution of the cavitary lesions.

Conclusions: We present a case of cavitating lung disease as a previously unreported manifestation of IgG4-related disease. Our patient had an excellent response to immunosuppression. An increased awareness of IgG4-related disease and its myriad of manifestations is very important for pulmonologists.

Publication types

  • Case Reports

MeSH terms

  • Humans
  • Immunoglobulin G / physiology*
  • Lung Diseases / diagnosis*
  • Lung Diseases / etiology*
  • Lung Diseases / therapy
  • Male
  • Middle Aged

Substances

  • Immunoglobulin G