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IgG4-Related Hepato-Pancreato-Biliary Disease

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Liver Disease in Clinical Practice

Part of the book series: In Clinical Practice ((ICP))

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IgG4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition characterised by histopathological findings of a lymphoplasmacytic infiltrate with abundant IgG4-positive plasma cells and storiform pattern of fibrosis in affected organs. It usually presents insidiously with mass-like lesions and/or fibrotic plaques/strictures. Given its systemic manifestations, patients will present to a range of different generalists and sub-specialists. It is often mis-diagnosed as malignancy or other infective/inflammatory conditions and can be left untreated for a number of years after initial presentation.

Diagnosis is based upon a combination of clinical presentation, serological findings, radiological evidence, histopathology where available and excellent response to corticosteroid therapy. Both systemic and organ-specific criteria have been developed to improve diagnostic accuracy. Early intervention is important to reduce morbidity and mortality associated with progressive and fibrotic disease. Treatment aims to provide symptomatic benefit, reduce inflammatory burden and prevent disease progression. IgG4-RD should be managed with a holistic and multidisciplinary approach to ensure accurate diagnosis, patient support and education, optimal choice and timing of therapy and effective management of complications.

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McNelis, E., Culver, E. (2022). IgG4-Related Hepato-Pancreato-Biliary Disease. In: Cross, T. (eds) Liver Disease in Clinical Practice. In Clinical Practice. Springer, Cham.

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-10011-6

  • Online ISBN: 978-3-031-10012-3

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