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IgG4-related sclerosing cholangitis


Key sources

The following summarized guidelines for the evaluation and management of IgG4-related sclerosing cholangitis are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2022), the British Society of Gastroenterology (BSG 2019), and the Japanese Society of Hepato-Biliary-Pancreatic Surgery (JSHBPS 2019). ...
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Screening and diagnosis

Clinical presentation: as per JSHBPS 2019 guidelines, recognize that 90% of patients with IgG4-SC have autoimmune pancreatitis. Recognize that IgG4-SC is also associated with dacryoadenitis, sialadenitis, retroperitoneal fibrosis, kidney lesions, pulmonary lesions, lymph node lesions, and vascular lesions (aorta and coronary arteries). Take into consideration these IgG4-related lesions.
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  • Diagnostic criteria

  • Differential diagnosis (cholangiocarcinoma)

  • Differential diagnosis (PSC)

Diagnostic investigations

IgG4: as per JSHBPS 2019 guidelines, obtain serum IgG4 levels for the diagnosis of IgG4-SC.

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  • Diagnostic imaging

  • Corticosteroid trial

Diagnostic procedures

Endoscopic retrograde cholangiography: as per JSHBPS 2019 guidelines, consider performing endoscopic retrograde cholangiography to detect diffuse or segmental stricture of the intrahepatic and/or extrahepatic bile ducts in patients with IgG4-SC.

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  • Bile duct biopsy

Medical management

Corticosteroids: as per EASL 2022 guidelines, initiate prednisone/prednisolone (0.5-0.6 mg/kg/day) as first-line therapy for untreated active IgG4-related cholangitis. Evaluate treatment response after 2-4 weeks before prednisone/prednisolone tapering by clinical, biochemical and/or radiological criteria.

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  • Immunosuppressants

Therapeutic procedures

Biliary drainage: as per JSHBPS 2019 guidelines, consider performing biliary drainage in patients with IgG4-SC having obstructive jaundice due to biliary stenosis.

Follow-up and surveillance

Indications for referral: as per BSG 2019 guidelines, refer patients with complex IgG4-SC or with suspected malignancy to a specialist multidisciplinary meeting for review.

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  • Follow-up

  • Management of relapse