autoimmune cholangitis

Autoimmune cholangitis, also referred to as primary autoimmune cholangitis or autoimmune cholangiopathy, is a condition in which the bile ducts are inflamed and destroyed due to an autoimmune response [2]. This means that the body's immune system mistakenly attacks its own tissues, leading to inflammation and damage to the bile ducts.

Autoimmune cholangitis is considered to be in the spectrum of autoimmune liver disease, with a closer resemblance to primary biliary cirrhosis (PBC) than to autoimmune hepatitis [3]. However, it differs from PBC due to the absence of serum M2 antibody. The condition has been noted to have a limited response to prednisolone, with inflammation being affected but bile duct lesions and cholestasis generally remaining unaffected.

The symptoms and characteristics of autoimmune cholangitis are similar to those of primary biliary cirrhosis (PBC), including chronic inflammation in the bile ducts, damage to the liver, and potential progression to liver cirrhosis [4]. The condition is considered an autoimmune disease due to its hallmark serologic signature, antimitochondrial antibody (AMA), and specific bile duct pathology [13].

Autoimmune cholangitis predominantly affects women and is characterized by a chronic and destructive small bile duct granulomatous lymphocytic cholangitis with typical seroreactivity for antimitochondrial antibodies [15]. Patients have variable risks of progressive ductopenia, cholestasis, and biliary fibrosis.

References: [2] - The phrase “autoimmune cholangitis” was coined in 1997 to describe AMA-negative primary biliary cirrhosis. [3] - Autoimmune cholangitis is considered to be in the spectrum of autoimmune liver disease. [4] - Primary biliary cholangitis causes chronic inflammation in your bile ducts. Damage to your bile ducts can eventually affect your liver. [13] - Primary Biliary Cholangitis (PBC) is considered an autoimmune disease because of its hallmark serologic signature, antimitochondrial antibody (AMA), and specific bile duct pathology. [15] - Primary biliary cholangitis is an autoimmune liver disease that predominantly affects women.

Autoimmune cholangitis, also known as primary biliary cholangitis (PBC), is a chronic liver disease characterized by the progressive destruction of intrahepatic bile ducts. The signs and symptoms of autoimmune cholangitis can vary from person to person, but here are some common ones:

• Fatigue: Feeling extremely tired or exhausted is one of the most common initial symptoms of PBC, affecting about 65% of people [2].
• Itchy skin (pruritus): Itching of the skin, particularly on the arms and legs, is another common symptom, affecting around 55% of people [2].
• Abdominal pain: Pain or discomfort in the upper right side of the tummy can also occur.
• Jaundice: Yellowing of the eyes and skin (jaundice) can be a sign of liver dysfunction.
• Weight loss: Unintentional weight loss can occur due to poor appetite and malabsorption of nutrients.
• Dry eyes and mouth: Some people may experience dryness in their eyes and mouth.
• Bone and joint aches: Pain or discomfort in the bones and joints can also be experienced.

It's worth noting that many people with PBC have no symptoms in the early stages, and some are diagnosed through blood tests before any signs of illness appear [3]. If you're experiencing any of these symptoms, it's essential to consult a healthcare professional for proper evaluation and treatment.

Autoimmune cholangitis, also known as primary biliary cholangitis (PBC), is a chronic liver disease characterized by the progressive destruction of the bile ducts within the liver. Diagnosing PBC requires a combination of medical history, physical examination, and laboratory tests.

Medical History and Physical Examination Your healthcare provider will ask about your medical history and symptoms, such as fatigue, itching, and jaundice (yellowing of the skin and eyes). A physical exam may also be performed to check for signs of liver disease.

Laboratory Tests Several laboratory tests can help diagnose PBC:

• Autoimmune Liver Disease Panel: This group of tests checks for autoimmune liver disease, which is a condition where the body's immune system attacks its own tissues. The panel detects and quantifies antimitochondrial antibodies (AMAs) using an enzyme immunoassay and smooth muscle antibodies by indirect immunofluorescence [6][7].
• Liver Biochemistry Testing: This test measures liver enzymes, such as alkaline phosphatase (ALP), aspartate aminotransferase (AST), and bilirubin levels. Elevated levels of these enzymes can indicate liver damage [8].
• Imaging Tests: Imaging tests, such as magnetic resonance cholangiopancreatography (MRCP) or endoscopic ultrasonography (EUS), may be used to visualize the bile ducts and diagnose PBC [5].

Other Diagnostic Tests In addition to these tests, your healthcare provider may also use noninvasive tests, including bilirubin, ALP, AST, albumin, and platelet count tests, at baseline and for ongoing monitoring [3]. Endoscopic retrograde cholangiography (ERCP) or other imaging tests may be used to confirm the diagnosis.

References

• [1] Learn how doctors diagnose primary biliary cholangitis using your medical and family history, a physical exam, and the results of medical tests.
• [2] Your healthcare provider will ask about your medical history and symptoms and physically examine you. Then they'll test a sample of your blood for evidence of ...
• [3] Jun 13, 2024 — Noninvasive tests, including bilirubin, ALP, AST, albumin, and platelet count tests, can be used at baseline and for ongoing monitoring.
• [4] Feb 6, 2023 — An autoimmune liver disease panel is a group of tests that is done to check for autoimmune liver disease. An autoimmune liver disease means that ...
• [5] by AHM Alizadeh · 2017 · Cited by 56 — Endoscopic retrograde cholangiography, magnetic resonance cholangiopancreatography and endoscopic ultrasonography comprise three of the ...
• [6] Feb 6, 2023 — An autoimmune liver disease panel is a group of tests that is done to check for autoimmune liver disease. An autoimmune liver disease means ...
• [7] Autoimmune liver disease ... Our panel detects and quantifies antimitochondrial antibodies using an enzyme immunoassay and smooth muscle antibodies by indirect ...
• [8] Feb 2, 2024 — In addition to a clinical evaluation, a diagnosis of PBC is largely based on laboratory testing, including liver biochemistry testing, ...

Treatment Options for Autoimmune Cholangitis

Autoimmune cholangitis, also known as primary biliary cholangitis (PBC), is a rare liver disease characterized by the progressive destruction of bile ducts within the liver. While there is no cure for PBC, various drug treatments can help manage the condition and slow down its progression.

First-Line Treatment

The first-line treatment for PBC is ursodeoxycholic acid (UDCA), which has been shown to improve liver function tests and reduce the risk of disease progression in up to 40% of patients [1]. UDCA works by reducing the amount of bile acids produced in the liver, thereby alleviating some symptoms associated with PBC.

Alternative Treatment Options

For patients who do not respond adequately to UDCA or experience a relapse after treatment discontinuation, alternative options are available. Obeticholic acid (OCA) is another FDA-approved treatment for PBC that has been shown to improve liver function tests and reduce the risk of disease progression [2]. OCA works by activating the farnesoid X receptor, which helps regulate bile acid production in the liver.

Emerging Treatments

Recent clinical trials have investigated the efficacy of emerging treatments for PBC. Elafibranor, a dual peroxisome proliferator-activated receptor-alpha and gamma agonist, has shown promise in improving treatment outcomes for patients with PBC [3]. Additionally, Seladelpar, an investigational drug, has been found to be effective in reducing liver enzymes and improving symptoms associated with PBC [4].

Other Medications

In some cases, medications may be prescribed to suppress the immune system or reduce inflammation. These can include prednisone or azathioprine, which have been used to treat autoimmune cholangitis [5]. Colchicine, an antigout agent, has also been investigated as a potential treatment for PBC [6].

References

[1] by RB Bernal · 2023 · Cited by 7 — Currently, there are only 2 Food and Drug Administration (FDA)-approved treatments for PBC, ursodeoxycholic acid (UDCA) and obeticholic acid (OCA).

[2] by CH Liu · 2022 · Cited by 6 — The first-line treatment is ursodeoxycholic acid; up to 40% of patients do not have an adequate response and remain at risk of disease progression.

[3] Nov 16, 2023 — “Based on the clinical data, Elafibranor has the potential to improve treatment for our patients living with primary biliary cholangitis by both ...

[4] Mar 11, 2024 — A clinical trial involving UC Davis Health suggests the investigational drug Seladelpar is a potential novel treatment for patients with the ...

[5] Mar 22, 2024 — Medications to suppress the immune system may also be prescribed including prednisone or azathioprine in Primary Biliary Cholangitis (PBC) ...

[6] by C Levy · 2023 · Cited by 44 — First-line therapy is a non-specific anti-cholestatic agent, ursodeoxycholic acid. For those with residual cholestasis biochemically, obeticholic acid is ...

Autoimmune cholangitis, also known as autoimmune hepatitis, is a rare liver disease characterized by an immune-mediated destruction of the bile ducts. When diagnosing this condition, it's essential to consider several differential diagnoses that can mimic its symptoms and presentation.

Possible Differential Diagnoses:

• Primary Sclerosing Cholangitis (PSC): A rare cholestatic liver disease that can cause inflammation and scarring of the bile ducts. [3]
• Biliary Obstruction or Stricture: Conditions that can block or narrow the bile ducts, leading to similar symptoms as autoimmune cholangitis. [4]
• Cholangiocarcinoma: A type of cancer that affects the bile ducts and can cause similar symptoms, including jaundice and abdominal pain. Elevated CA 19-9 levels above 130 U/mL may suggest this diagnosis. [5]
• Autoimmune Hepatitis: Another autoimmune liver disease that can cause inflammation of the liver and bile ducts. [8]
• Alcoholic and Non-Alcoholic Steatohepatitis: Conditions characterized by inflammation and scarring of the liver, which can be confused with autoimmune cholangitis. [8]

Other Considerations:

• Portal Tract Inflammation: A condition that can cause inflammation in the portal tract area, leading to symptoms similar to autoimmune cholangitis. [6]
• Bile Duct Injury: Trauma or injury to the bile ducts can cause similar symptoms and may be considered as a differential diagnosis. [6]

Common Initial Symptoms:

• Fatigue
• Itching of the skin (pruritus)
• Abdominal pain
• Darkening of the skin (jaundice) [7]

It's essential to consider these differential diagnoses when evaluating patients with suspected autoimmune cholangitis, as accurate diagnosis and treatment can significantly impact patient outcomes.

References: [1] Sarcognato, S. (2021). Autoimmune cholestatic liver diseases. [3] Kunovsky, L. (2021). Primary sclerosing cholangitis. [4] (2024). Differential diagnoses of PBC. [5] (2019). Cholangiocarcinoma. [6] (2022). Autoimmune cholangitis. [7] (2024). Initial symptoms of primary sclerosing cholangitis. [8] (2024). Differential diagnoses of autoimmune hepatitis.