Fibrosing Cholestatic Hepatitis - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Fibrosis cholestatic hepatitis (FCH) is also known as fibrosing cytolytic liver failure and fibroviral hepatitis. FCH is a rare variant of hepatitis. Fibrosing cholestatic hepatitis is an aggressive and fatal disease that usually occurs in patients with compromised immunity. Hepatitis is liver inflammation caused by hepatitis viruses A, B, C, D, and E. Cholestatic hepatitis is caused by hepatitis B and hepatitis C. It can also be seen in patients with liver transplants and immunosuppressed patients. The recurrence of hepatitis C can be very challenging to deal with in patients with liver transplants. Fibrosis cholestatic hepatitis is the leading cause of death in such patients. A high level of aminotransferase (liver enzyme) and histological changes in the liver cells signifies FCH. Various treatment modalities can prevent hepatitis C recurrence in patients with liver transplants.

What Is Fibrosis Cholestatic Hepatitis?

Fibrosis cholestatic hepatitis is an aggressive form of viral hepatitis (liver inflammation) which causes liver fibrosis (scarring of healthy liver cells). FCH is a fatal disease, and the prognosis is poor in patients. It is a common threat to liver transplantation. Hepatitis C is a common recurrence in liver transplantation patients. FCH is more common in immunosuppressant patients and is mostly seen in men. There is rapid progression to liver fibrosis which ultimately leads to death.

What Are the Causes of Fibrosis Cholestatic Hepatitis?

Hepatitis infection is the most common cause of FCH after liver or kidney transplants. Hepatitis B and C, in particular, cause fibrosing cholestatic hepatitis. The recurrence of hepatitis C is prevalent among allograft liver recipients (recipients who receive tissue from another person). Hepatitis C is caused by ribonucleic acid (RNA) flavivirus. Most individuals are not aware of hepatitis C despite being infected. Patients who are exposed to the virus become chronically infected eventually.

What Are the Symptoms of Fibrosis Cholestatic Hepatitis?

Many patients remain asymptomatic with acute infection of Hepatitis C. Hence, most affected individuals remain undiagnosed. The following are the symptoms of fibrosis cholestatic hepatitis:

• Headache.

• Myalgia (muscle pain).

• Arthralgia (joint pain).

• Nausea and vomiting.

• Irritability.

• Depression.

• Fatigue.

• Weight loss.

• Weakness.

• Anorexia (eating disorder).

• Jaundice.

• Diarrhea.

• Abdominal discomfort.

• Dark urine and pale stool.

• Hepatomegaly (liver enlargement).

• Mild splenomegaly (spleen enlargement).

• Cervical lymphadenopathy (lymph node enlargement).

• Liver fibrosis.

What Are the Risk Factors for Fibrosis Cholestatic Hepatitis?

The following are the risk factors for fibrosing cholestatic hepatitis:

• Hepatitis C infection.

• Hepatitis B infection.

• Immunocompromised people.

• Hepatitis A infection.

• Alcohol abuse.

• Drug abuse.

• Needle stick injury.

• Sharing razors or toothbrushes of infected Individuals.

• Liver transplantation.

What Are the Complications of Fibrosing Cholestatic Hepatitis?

If left untreated, fibrosis cholestatic hepatitis progresses to liver fibrosis and can lead to liver damage. Not all patients develop liver cirrhosis, but once liver cirrhosis occurs with hepatitis C, the survival rate is five to ten percent in most patients. Liver or kidney rejections in patients with liver transplantation are the most common threat in allograft recipients. It may result in multiple organ failure causing death. The following are the complications of fibrosis cholestatic hepatitis:

• Hepatocellular carcinoma (liver cancer).

• Liver damage.

• Liver cirrhosis.

• Multiple organ failure.

How Is Fibrosis Cholestatic Hepatitis Diagnosed?

Most patients remain unaware of exposure to hepatitis C. FCH is usually identified when the patients develop chronic liver disease. The following is the investigation by which fibrosis cholestatic hepatitis can be diagnosed:

• Routine Blood Tests - The increased number of white blood cells in the blood shows infection. Prolonged prothrombin time (clotting factors) is observed in FCH. Increased serum bilirubin level indicates jaundice, which occurs in the end-stage of FCH.

• Liver Function Test - Sometimes, the liver function test is normal, but a mild elevation in serum transaminases level is observed in FCH.

• Serology and Virology - The test helps identify several antibodies in infected patients against antigens. Anti-hepatitis antibodies persist in the serum of an infected person even after two or three weeks of infection, and hepatitis C RNA can be identified in the blood.

• Liver Histology - The degree of inflammation and fibrosis can be diagnosed by liver histology. Swelling in the liver cells, intracellular cholestasis (bile duct obstruction), collagen deposition in the cells, and prominent cholestasis are the common features of liver cell histopathological changes.

• Liver Biopsy - A liver biopsy helps detect histological changes occurring in liver cells, most common in liver cirrhosis. A liver biopsy should be done in case of FCH to diagnose the degree of liver damage.

• Ultrasound - This technique allows visualization of the abdomen. Ultrasound can measure the size of the liver. It can also be used to assess adjacent organs affected by hepatomegaly.

• Computed Tomography (CT Scan) - CT scan helps assess the liver's exact dimensions by providing higher-resolution images of the abdomen.

When Is It Necessary to See a Doctor?

Although FCH sometimes remains asymptomatic, immunocompromised patients should be monitored regularly. FCH can be fatal if left untreated. Patients should consult a doctor as soon as they observe the following symptoms:

• Black and tarry stools.

• Blood in stools.

• Blood in vomiting.

• Severe abdominal pain.

• Jaundice.

• High fever persists even after taking over-the-counter drugs.

How Is Fibrosis Cholestatic Hepatitis Treated?

The treatment aims to eradicate infection. Fibrosis cholestasis hepatitis leads to progressive fibrosis of the liver, resulting in liver cirrhosis. Immunocompromised patients can be treated by a combination of antiviral therapy with immunomodulators. According to studies, the outcomes of FCH in patients with a liver transplant are poor and have a minimum survival rate. Various treatment modalities can prevent the recurrence of infection. In addition, active vaccination against hepatitis can prevent the cycle of infection. Antiviral prophylaxis and immunoglobulins can reduce the reinfection rate in patients with liver transplants. This can improve the prognosis for liver transplantation. The following are the acceptable treatment for FCH:

• Interferon alpha is injected subcutaneously.

• Ribavirin can be given orally as an antiviral therapy.

• Direct-acting antivirals such as Telaprevir, Boceprevir, Sofosbuvir, Simeprevir, Daclatasvir, Ledipasvir, Paritaprevir, Ombitasvir, and Dasabuvir can be used.

Conclusion

Although FCH is a rare variant of hepatitis, it is seen in immunocompromised patients such as organ transplants. Therefore, patients with organ transplants should be prompt in seeking attention and have regular checkups and screening. There is no active or passive protection against the hepatitis C virus. Studies have shown no recovery report in patients with FCH. Various treatment modalities illustrated above can improve survival rates to one to two years. Despite the poor prognosis, regular checkups and appropriate medical interventions can improve life quality.