Chronic non-suppurative destructive cholangitis
What is it?
It is a chronic progressive liver disease that mainly affects women (90% of cases) from the fourth decade of life. It is characterized by inflammation and destruction of the bile canaliculi which are the ducts through which bile is secreted and which pass through the liver. This disease is also known as primary biliary cirrhosis, although this name is not correct since not all people who suffer from it have liver cirrhosis.
Causes of cholangitis
The disease is caused by the attack of the immune system (defenses) of the patient to the epithelium of the bile canaliculi (cells that form the wall of the canaliculi). The destruction of the bile canaliculi causes the accumulation of bile acids (components of bile) and other toxic substances in hepatocytes (liver cells) destroying them. There is evidence to indicate that there is a genetic predisposition to develop this disease and it has been suggested that infections by certain types of bacteria or viruses would be the trigger of the attack of the immune system in people who have genetic susceptibility.
Cholangitis occurs mainly in women from 40 years.
Diagnosis
In the initial phases, patients present no symptoms (60% of cases) and the disease is discovered when routine blood tests show elevated levels of liver enzymes (transaminases, alkaline phosphatase, gamma glutamyl transpeptidase). As the disease progresses, patients may have asthenia (weakness), pruritus (itching) and jaundice (yellowing of the skin and eyes) and 95% of them have anti-mitochondrial antibodies (AMA).
Treatment
The treatment of non-suppurative destructive chronic cholangitis consists of the administration of ursodeoxycholic acid (UDCA), which must be taken indefinitely. This drug manages to avoid inflammation and destruction of liver cells (normalization of liver parameters) in 70% of treated people. The UDCA presents an excellent tolerance, although some patients may notice bloating (gas), increased intestinal rhythm and digestive discomfort. If the patient does not respond to treatment with UDCA, immunosuppressive drugs (cortisone, etc.) can be added.
Chronic nonsuppurative destructive cholangitis is associated with the development of osteoporosis and, therefore, the use of vitamin D and calcium supplements is necessary.
As previously mentioned, patients with chronic non-suppurative destructive cholangitis may have pruritus (generalized itching) of great intensity requiring pharmacological treatment (resins, antihistamines, etc.).
Finally, if the disease continues to progress and ends in liver cirrhosis, it is necessary to resort to liver transplantation.
There is evidence that indicates that there is a genetic predisposition to develop this disease.
How can you know the aggressiveness of the disease
A liver biopsy not only confirms the clinical diagnosis of destructive non-suppurative chronic cholangitis, but also determines the degree of inflammation and destruction of the liver cells.
References
- Prince MI, Jones DE. Primary biliary cirrhosis: new perspectives in diagnosis and treatment. Postgrad Med J 2000;76:199-206.
- Corpechot C, et al. The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology 2005;128:297-303.
- Hohenester S, et al. Primary biliary cirrhosis. Semin Immunopathol 2009;31:283-307
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