Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2019 Aug 20:2019:7572869.
doi: 10.1155/2019/7572869. eCollection 2019.

Pleomorphic Pulmonary Manifestations of IgG4-Related Disease

Srikanth Naramala  1 , Sharmi Biswas  2 , Sreedhar Adapa  3 , Vijay Gayam  4 , Romeo C Castillo  5 , Srinadh Annangi  6 , Venu Madhav Konala  7
Affiliations
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.

PubMed Disclaimer

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.
See this image and copyright information in PMC

References

    1. Tashiro H., Takahashi K., Nakamura T., Komiya K., Kimura S., Sueoka-Aragane N. Coexistence of lung cancer and immunoglobulin G4-related lung disease in a nodule: a case report. Journal of Medical Case Reports. 2016;10(1):p. 113. doi: 10.1186/s13256-016-0898-3. - DOI - PMC - PubMed
    1. Nagashima K., Sano I., Kobayashi T., et al. IgG4-related lung pseudotumor and pleural inflammation with autoimmune hepatitis. Internal Medicine. 2018;57(1):43–48. doi: 10.2169/internalmedicine.9026-17. - DOI - PMC - PubMed
    1. Zaarour M., Weerasinghe C., Eter A., El-Sayegh S., El-Charabaty E. An overlapping case of lupus nephritis and IgG4-related kidney disease. Journal of Clinical Medicine Research. 2015;7(7):575–581. doi: 10.14740/jocmr2189w. - DOI - PMC - PubMed
    1. Kamisawa T., Zen Y., Pillai S., Stone J. H. IgG4-related disease. The Lancet. 2014;385(9976):1460–1471. doi: 10.1016/s0140-6736(14)60720-0. - DOI - PubMed
    1. Sarles H., Sarles J.-C., Muratore R., Guien C. Chronic inflammatory sclerosis of the pancreas—an autonomous pancreatic disease? The American Journal of Digestive Diseases. 1961;6(7):688–698. doi: 10.1007/bf02232341. - DOI - PubMed

Publication types

LinkOut - more resources