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Case Reports
, 2019, 7572869
eCollection

Pleomorphic Pulmonary Manifestations of IgG4-Related Disease

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Case Reports

Pleomorphic Pulmonary Manifestations of IgG4-Related Disease

Srikanth Naramala et al. Case Rep Rheumatol.

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disorder which has been first reported in 2001 by Hamano and colleagues in a patient with autoimmune sclerosing pancreatitis. Almost every organ in the human body can be affected by IgG4-RD from infiltration with IgG4-positive plasma cells. Involvement of lungs with IgG4 is reported previously, but still, there is no clear picture of the pathophysiology behind lung involvement. Here, we are presenting a patient who has IgG4-RD presenting as pseudotumor of the lungs.

Conflict of interest statement

The authors declare that there are no conflicts of interest regarding the publication of this paper.

Figures

Figure 1
Figure 1
CT chest with contrast showing bilateral interlobular septal thickening, multiple ground-glass opacities, and cystic changes.
Figure 2
Figure 2
CT chest with contrast showing 1.4 cm right lower lobe lung nodule.
Figure 3
Figure 3
Immunostaining showing abundant IgG4 plasma cells.
Figure 4
Figure 4
High power field showing obliterative phlebitis with destruction of the vessel wall.
Figure 5
Figure 5
High power field showing fibrous bands.
Figure 6
Figure 6
High power field showing arteritis with minimal obliteration of the lumen. The orange arrow shows rich lymphoplasmacytic infiltrate.

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