Effective Treatment is Need of the Hour in Management of Primary Sclerosing Cholangitis

 


Primary sclerosing cholangitis (PSC) is an uncommon chronic non-inflammatory disorder typically characterized by thickening, inflammation, and fibrosis of the bile tissues within the bile (septum) or the porcine bile duct (burgh's lobules). Both the apocrine and bile ducts are involved. The lining of the small intestine also becomes thickened. Patients generally have no symptoms. However, some symptoms may occur such as diarrhea, nausea, abdominal bloating, and vomiting. Other inflammatory diseases may co-occur with psoriasis.

Primary sclerosing cholangitis is also called systemic sclerosis. It is one of the most common causes of cirrhosis of the liver in the U.S. In approximately one third of patients with PSC, no identifiable disease or infection is present. In approximately twenty-five percent of patients with this disorder, the disorder is diagnosed based on history and symptoms. In approximately ten percent of individuals with this disease, specific tests for an adequate diagnosis are not performed. Approximately two-thirds of individuals with PSC do not have a history of cancer or other systemic autoimmune diseases. In approximately eight percent of cases, there are no abnormalities or redness in the skin.

One of the most commonly used techniques in diagnosing the disorder is the magnetic resonance cholangiopancreatography, MRCP, or abdominal ultrasound technique. The first step in the diagnosis process involves the use of a CT scan or an MRI to obtain detailed images of the abdominal cavity and the heart. After getting the details of the structure and the location of the obstruction, the doctor will be able to conclude if the patient has primary sclerosing cholangitis. During the evaluation of the patients, doctors will make a conclusion if there are also autoantibodies present. Autoantibodies are substances or cells that have the tendency to bind with the red blood cells or with the platelets of the patients' blood.

As of now, no pharmacologic treatment has been approved by the U.S. Food and Drug Administration for PSC and there is no curative treatment. In July 2020, ChemomAb, a clinical-stage biotech company focusing on discovery and development of innovative therapeutics for fibrosis-related diseases, announced that it has received all necessary regulatory approvals in the U.K and Israel, to commence a phase IIa clinical trial for CM-101 as treatment for PSC.

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