IgG4-Related Systemic Disease Presenting as Secondary Sclerosing Cholangitis with Multiorgan Involvement: A Case Report

^1* Dr. Albert Shaji, ² Dr. Umashankar R, ³ Dr. Razook Fareedh, ⁴ Dr. Aishwarya Lakshmi M V, ⁵ Dr. Sheik Arshad Ali, ⁶ Dr. Shobana S

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ABSTRACT

IgG4-related systemic disease (IgG4-RD) is a systemic, immune-mediated fibroinflammatory disorder, that may involve a variety of organs. In this case report, the patient has an IgG4-related disease that can be characterized as secondary sclerosing cholangitis with a multifocal involvement including the pancreas, kidney, lymph node, and more. The patient presented abdominal pains, jaundice, and high liver enzymes. The imaging showed biliary strictures and organ enlargement. The IgG4 level in serum was significantly increased and histopathology revealed a dense lymphoplasmacytic infiltration with storiform fibrosis and IgG4-positive plasma cells. Imaging, biopsy and serological studies were used to rule out such differential diagnoses as primary sclerosing cholangitis and cholangiocarcinoma. The diagnosis of IgG4-related cholangitis was confirmed by the rapid improvement in the biochemical and clinical conditions of the patient under corticosteroid therapy. The main results of this report are that the early identification of IgG4-RD as a reversible cause of biliary obstruction is crucial, and has typical histological characteristics, and the possibility of relapse in case of premature withdrawal of treatment. In this paper, the diagnosis issue is highlighted because of the similarity with malignancies and autoimmune diseases. In the case, it becomes obvious that multidisciplinary assessment (imaging, serology, pathology) should be performed to make a correct diagnosis and prescribe immunosuppressive therapy. High awareness of IgG4-associated sclerosing cholangitis is critical to avoid invasive procedures that are not needed and enhance the long-term treatment outcomes in patients by providing early medical care and regularly checking them.

Keywords: IgG4-related disease, Sclerosing cholangitis, Multiorgan involvement, Autoimmune pancreatitis, Biliary obstruction, Corticosteroid therapy, Storiform fibrosis, Plasma cell infiltration, Differential diagnosis, Immunopathology.

How to cite this article: Shaji A, R U, Fareedh R, Lakshmi MAV, Ali SA, S S. IgG4-Related Systemic Disease Presenting as Secondary Sclerosing Cholangitis with Multiorgan Involvement: A Case Report. Int J Drug Deliv Technol. 2026;16(2): 98-106; DOI: 10.25258/ijddt.16.2.13

Source of support: Nil.

Conflict of interest: None