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IgG4-Related Disease: A Case Series Highlighting Diverse Clinical Manifestations and Treatment Outcomes

Thanda Aung1, Kaitlin Eblen1, Gregory A Fishbein2

  • 1Rheumatology, University of California Los Angeles David Geffen School of Medicine, Los Angeles, USA.

Cureus
|November 18, 2025
PubMed

IgG4-related disease (IgG4-RD) is a systemic immune-mediated fibroinflammatory condition characterized by tumor-like swelling of affected organs, lymphoplasmacytic infiltration enriched with IgG4-positive plasma cells, and variable degrees of fibrosis. We present three cases of IgG4-RD with diverse clinical presentations, highlighting the diagnostic challenges and management approaches. Our cases include a 45-year-old female with lacrimal and salivary gland involvement mimicking Sjögren's syndrome, a 77-year-old male with pancreatic mass, mesenteric vasculitis, and intra-abdominal lymphadenopathy initially suspected as pancreatic cancer, and a 75-year-old male with diffuse lymphadenopathy, initially thought to be lymphoma. All patients were successfully treated with rituximab with good clinical and radiological responses, emphasizing the importance of considering IgG4-RD in the differential diagnosis of conditions with multisystem involvement and atypical presentations.

Keywords:
igg4-related diseaselymphadenopathymikulicz's diseasesclerosing mesenteritissicca syndrome

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