Igg4-associated cholangitisK83.0
Synonym(s)
DefinitionThis section has been translated automatically.
Autoimmunologically induced, chronic inflammation of the bile ducts, which belongs to the group of IgG4-associated infections.
EtiopathogenesisThis section has been translated automatically.
Pathogenetically, immunoglobulin IgG4 plays an important role in all IgG4-associated (autoimmune) diseases (Trampert DC et al. (2018). In several independent IAC cohorts a disproportionately high proportion of workers with high exposure to solvents, industrial dusts/oils and pesticides could be detected (Herta T et al. 2018)
ManifestationThis section has been translated automatically.
Patients with IgG4-associated cholangiopathy are usually > 60 years; m>w =8:1 (Herta T et al. 2018)
Clinical featuresThis section has been translated automatically.
In the early stages asymptomatic. Later icterus, itching, unclear upper abdominal pain, weight loss. 85.0% of patients with autoimmune pancreatitis have associated autoimmunological cholangitis (AIC). (Buechter M et al. 2017).
ImagingThis section has been translated automatically.
Imaging shows a symmetrical, circular wall thickening which may also affect non-stenosed bile duct areas. Criteria for IAC are a pathological cholangiogram (type 1-4):
- type 1: isolated distal ductus hepaticus communis (DHC) stenosis
- Type 2: diffuse stenoses
- Type 3: Hilary and distal DHC stenosis
- Type 4: Isolates hilary DHC stenosis.
LaboratoryThis section has been translated automatically.
IgG4 in serum increased (diagnostically relevant if the value is 4x higher than the normal value); IgG4-positive cells in bile duct cytology.
DiagnosisThis section has been translated automatically.
Coexistence with AIP, sialadenitis(Mikulicz syndrome) or retroperitoneal fibrosis; characteristic histological findings with infiltration of IgG4-positive plasma cells In case of uncertain attribution the diagnosis can be made ex juvantibus: evaluation of the healing success by therapy with glucocorticosteroids (good response to therapy with glucocorticosteroids).
Differential diagnosisThis section has been translated automatically.
In terms of differential diagnosis, all primary and secondary forms of sclerosing cholangitis (K83.0) (PSC: in 60% of cases detection of ANA and pANCA) as well as pancreatobiliary tumours (Roos E et al. 2018) must be considered. In about 50-80% of cases there is an association with autoimmune pancreatitis type 1 (K86.1).
TherapyThis section has been translated automatically.
Excellent response to systemic glucocorticosteroid therapy. Long-term therapy necessary. High recurrence rate. Possible combination with azathioprine.
LiteratureThis section has been translated automatically.
- Buechter M et al (2017) Outcome and Genetic Factors in IgG4-Associated Autoimmune Pancreatitis and Cholangitis: A Single Center Experience. Gastroenterol Res Pract 2017:6126707.
- Herta T et al (2018) IgG4-associated cholangitis - clinical presentation of an overlooked disease entity. Internist (Berl) 59:560-566.
- Roos E et al (2018) IgG4-associated cholangitis in Patients Resected for Presumed Perihilar Cholangiocarcinoma: a 30-Year Tertiary Care Experience. On J Gastroenterol 113:765-772.
- Trampert DC et al (2018) On the role of IgG4 in inflammatory conditions: lessons for IgG4-related disease. Biochim Biophys Acta 1864:1401-1409.