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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 Japanese Society of Hepato-Biliary-Pancreatic Surgery (JSHBPS 2019), and the British Society of Gastroenterology (BSG 2019).
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Guidelines

1.Screening and diagnosis

Clinical presentation: 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)

2.Diagnostic investigations

Immunoglobulin G4: obtain serum IgG4 levels for the diagnosis of IgG4-SC.
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  • Diagnostic imaging

  • Corticosteroid trial

3.Diagnostic procedures

Endoscopic retrograde cholangiography: 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

4.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

5.Therapeutic procedures

Biliary drainage: consider performing biliary drainage in patients with IgG4-SC having obstructive jaundice due to biliary stenosis.
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6.Follow-up and surveillance

Indications for referral: 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