Pathologic manifestations of Immunoglobulin(Ig)G4-related lung disease

Eunhee S. Yi, Hiroshi Sekiguchi, Tobias Peikert, Jay H. Ryu, Thomas V. Colby

Research output: Contribution to journalArticlepeer-review

29 Scopus citations


Immunoglobulin(Ig)G4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect virtually any organ and usually presents as tumefactive lesions involving multiple sites. Characteristic histopathology of IgG4-RD consists of dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis, accompanied by tissue infiltration of IgG4-positive plasma cells with or without elevation of serum IgG4 level. Despite a general similarity in the morphologic manifestations of IgG4-RD, site-specific unique morphologic features have been described in some organs including the lung. Compared with other sites, pulmonary involvement by IgG4-RD has been recognized more recently, and lung biopsy interpretation for this condition is often challenging, as both a relative paucity of pathognomonic features and a plethora of overlapping findings with other fibroinflammatory processes of the lung. This review is focused on the morphologic spectrum of IgG4-related lung disease documented in the current literature and on the pertinent issues in the differential diagnoses with other conditions encountered in the lung.

Original languageEnglish (US)
Pages (from-to)219-225
Number of pages7
JournalSeminars in Diagnostic Pathology
Issue number4
StatePublished - Nov 2012


  • Histopathology;
  • Immunoglobulin(Ig)G4;
  • Lung;
  • Lymphoma;
  • Rosai-Dorfman disease
  • Thorax;

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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