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.