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
. 2024 Sep 20;11(9):e01481.
doi: 10.14309/crj.0000000000001481. eCollection 2024 Sep.

An Evasive Liver Mass in a Human Immunodeficiency Virus (HIV)-Positive Patient

Reanay Berezovskiy  1 , James M Crawford  2 , Arvind Rishi  2 , Sohil Khurana  2 , Joshua Kern  2 , Stefani Morscher  2 , Sanjaya K Satapathy  2
Affiliations
Case Reports

An Evasive Liver Mass in a Human Immunodeficiency Virus (HIV)-Positive Patient

Reanay Berezovskiy et al. ACG Case Rep J. .

Abstract

IgG4-related disease (IgG4-RD) is an autoimmune syndrome that is characterized by elevated levels of serum IgG4 and infiltration of various tissue types by IgG4 immunoreactive plasma cells. The IgG4-RD can result in systemic disease and the formation of inflammatory mass lesions, frequently addressed as pseudotumors. While IgG4-RD can manifest in various organs, liver involvement is rare, and because it is an immune-mediated inflammatory process, it is uncommon in patients who are immunocompromised. Furthermore, despite IgG4-RD responding well to immunosuppressive treatment, cases of spontaneous remission are exceedingly rare in the literature. In this report, we present the unique case of a self-resolving IgG4-RD lesion of the liver in a HIV positive patient.

Keywords: IgG4-related disease; immunosuppression; pseudotumor.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Arterial phase computed tomography demonstrated a large heterogenous mass in the right lobe of the liver measuring up to 11.2 cm (predominantly in segment 7 or 8). Areas of solid enhancement as well as a central necrosis are present. **IgG4-related pseudotumors usually present with variable enhancement patterns, most commonly exhibiting homogenous delayed enhancement.
Figure 2.
Figure 2.
Venous phase computed tomography demonstrated right posterior portal vein thrombosis; raising suspicion for a tumor in vein.
Figure 3.
Figure 3.
Densely inflamed liver biopsy tissue with storiform pattern of fibrosis (A 100× magnification H&E). Numerous plasma cells in a background of completely replaced hepatic parenchyma by the fibrosis (B 200× magnification H&E and (C) 400× magnification H&E). IgG4 immunohistochemistry highlight plasma cells within fibrotic stroma (400× magnification).
Figure 4.
Figure 4.
Magnetic resonance imaging at 18-month mark; enhancing scar at the site of previous lesion depicted by the arrow.
See this image and copyright information in PMC

References

    1. Perugino CA, Stone JH. IgG4-related disease: An update on pathophysiology and implications for clinical care. Nat Rev Rheumatol. 2020;16(12):702–14. - PubMed
    1. Liu C, Zhang P, Zhang W. Immunological mechanism of IgG4-related disease. J Transl Autoimmun. 2020;3:100047. - PMC - PubMed
    1. Karadeniz H, Vaglio A. IgG4-related disease: A contemporary review. Turk J Med Sci. 2020;50(SI-2):1616–31. - PMC - PubMed
    1. Umehara H, Okazaki K, Kawa S, et al. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Mod Rheumatol. 2021;31(3):529–33. - PubMed
    1. Huggett MT, Culver EL, Kumar M, et al. Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort. Am J Gastroenterol. 2014;109(10):1675–83. - PMC - PubMed

Publication types

LinkOut - more resources