Screening and diagnosis

Classification and risk stratification

Perioperative care

Quality improvement

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Nonpharmacologic interventions

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Follow-up and surveillance

Specific circumstances

Preventative measures

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Inpatient care

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Diagnostic investigations

Insufficient evidence to make a recommendation.

Strongly favor this test or intervention.

The system determines the classification of IgG4-related sclerosing cholangitis (IgG4-SC) using specific clinical diagnostic criteria. The calculation is based on the presence or absence of a set of clinical, serological, and pathological findings indicative of IgG4-SC. These findings are:

1. Diffuse or segmental narrowing of the intrahepatic and/or extrahepatic bile duct associated with thickening of the bile duct wall on biliary tract imaging
2. Elevated serum IgG4 levels (>= 135 mg/dL)
3. Coexistence of autoimmune pancreatitis, IgG4-related dacryoadenitis/sialadenitis, or IgG4-related retroperitoneal fibrosis
4. Marked lymphocytic and plasma cell infiltration and fibrosis
5. Infiltration of IgG4-positive plasma cells (> 10 IgG4-positive plasma cells/hpf)
6. Storiform fibrosis
7. Obliterative phlebitis
8. Effectiveness of corticosteroid therapy

The system evaluates the combinations of these inputs to classify the diagnosis as definite, probable, or possible IgG4-SC, or as criteria not met. The decision is based on specific input combinations:

- Definite IgG4-SC:
  - Presence of criteria 1 and 3
  - Presence of criteria 1, 2, 4, and 5
  - Presence of criteria 4, 5, and 6
  - Presence of criteria 4, 5, and 7

- Probable IgG4-SC:
  - Presence of criteria 1, 2, and 8

- Possible IgG4-SC:
  - Presence of criteria 1 and 2

The clinical diagnostic criteria of IgG4-related sclerosing cholangitis (IgG4-SC) is a diagnostic tool designed to identify and classify patients with this specific form of cholangitis. The target patient population includes individuals presenting with symptoms of cholangitis, such as jaundice, abdominal pain, and pruritus, who may also have concurrent autoimmune conditions.

The primary components of this diagnostic criteria include both clinical and laboratory findings. Clinically, the patient may present with diffuse or segmental narrowing of the intrahepatic and/or extrahepatic bile duct associated with thickening of the bile duct wall on biliary tract imaging. Laboratory findings include elevated serum IgG4 levels (>= 135 mg/dL), marked lymphocytic and plasma cell infiltration and fibrosis, infiltration of IgG4-positive plasma cells (> 10 IgG4-positive plasma cells/hpf), and storiform fibrosis or obliterative phlebitis. The coexistence of autoimmune pancreatitis, IgG4-related dacryoadenitis/sialadenitis, or IgG4-related retroperitoneal fibrosis is also considered. The effectiveness of corticosteroid therapy is another component of the criteria.

The diagnosis of definite IgG4-SC is determined by the presence of specific combinations of these components. For instance, a patient meets the criteria for definite IgG4-SC if they have diffuse or segmental narrowing of the bile ducts on imaging and coexisting autoimmune conditions. Alternatively, a patient can be diagnosed with definite IgG4-SC if they have elevated serum IgG4 levels, marked lymphocytic and plasma cell infiltration and fibrosis, and infiltration of IgG4-positive plasma cells. Other combinations of these components can also lead to a diagnosis of definite IgG4-SC.

The criteria also allow for the classification of probable and possible IgG4-SC. For example, a patient meets the criteria for probable IgG4-SC if they have diffuse or segmental narrowing of the bile ducts on imaging, elevated serum IgG4 levels, and a positive response to corticosteroid therapy. A patient meets the criteria for possible IgG4-SC if they have diffuse or segmental narrowing of the bile ducts on imaging and elevated serum IgG4 levels. If the patient does not meet any of these combinations, the criteria for IgG4-SC are not met.

IgG4-related sclerosing cholangitis

Guidelines

Key sources