Liver Cirrhosis

Thank you Team 3 for presenting a case of a patient with cryptogenic liver cirrhosis.  Cirrhosis is a very common diagnosis and we frequently care for patients with this disease. However, it is less common that we see patients with cryptogenic cirrhosis. This combination of a common condition with a less common etiology makes the case a perfect one for Morning Report!

A healthy liver is pink, smooth and filled with happy hepatocytes. The word ‘cirrhosis’ comes from the Greek word kirrhos which means ‘yellowish’ – describing the colour of the diseased organ. In addition to becoming discoloured, cirrhotic livers are firm, nodular and composed of fibrous tissue. This description of abnormal structure gives you a sense of how impaired the liver function can become! Here are six things to know about cirrhosis…

There is a long list of causes of cirrhosis but there is a short list of common causes:

     1. Alcohol

     2. Viral Hepatitis (C & B)

     3. Non-Alcoholic Fatty Liver Disease (NAFLD)

     4. Hemochromatosis

The other less common causes to think about include: Autoimmune, Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis, Veno-occlusive disease, Wilson’s Disease, alpha-1 antitrypsin, medications (ie. methotrexate), right-sided heart failure and even celiac disease!  

Most patients remain asymptomatic until they develop decompensated cirrhosis which is characterized by the development of any of the following complications:

     - Variceal Bleed

     - Ascites

     - Encephalopathy

     - Spontaneous Bacterial Peritonitis

     - Jaundice

In addition to the acute complications of cirrhosis listed above, think about some of the other associated complications:

     - Hepatocellular carcinoma

     - Hepatorenal Syndrome

     - Osteoporosis

Bloodwork can provide clues that a patient has cirrhosis. Imaging and in some cases biopsy are used to confirm the diagnosis. According to the JAMA rational clinical exam, a platelet count of less than 160 increases the likelihood that the patient has cirrhosis. Remember, this is a sensitive finding – not specific! Other abnormal lab values in patients with cirrhosis include elevated bilirubin, elevated INR, reduced albumin and transaminitis. Abdominal ultrasound is the initial imaging modality of choice to assess for cirrhosis. Finally, a liver biopsy may be necessary to help determine an etiology.

Unfortunately, cirrhosis is irreversible so the management should focus on limiting or removing the precipitant and either preventing or dealing with the complications. This will commonly include managing ascites (diuretics, paracentesis, TIPS in select cases), SBP (antibiotics acutely and for prevention in certain cases), varices (non-selective beta-blockers, OGD with band ligation), encephalopathy (lactulose). Patients with decompensated cirrhosis should be considered for referral for liver transplant. There are many variables that go into deciding whether a patient is eligible for liver transplant including Model for End-stage Liver Disease (MELD) score, comorbidities, presence of complications and absence of contraindications.

A word about cryptogenic cirrhosis. This entity is not that uncommon, representing at least 5% of cases of cirrhosis. While the term cryptogenic implies that the cause is unclear, it is thought that many of these cases are due to unrecognized NASH, silent autoimmune hepatitis, non-B/C hepatitis or possibly from prior unreported alcohol use. Diagnosing cryptogenic cirrhosis requires a thorough history and extensive investigations. Biopsy can be used to help identify a cause of cirrhosis – particularly metabolic causes. The management is similar to that of other causes of cirrhosis. 

Finally, when diagnosing and/or managing cirrhosis - do not hesitate to refer to a gastroenterologist. UHN is home to world class hepatologists with interdisciplinary liver clinics that provide high quality care for these complex patients.

References:

1. Schuppan, Detlef, and Nezam H. Afdhal. "Liver cirrhosis." The Lancet 371.9615 (2008): 838-851.

2. Udell, Jacob A., et al. "Does this patient with liver disease have cirrhosis?."JAMA 307.8 (2012): 832-842.3

3. Caldwell, Stephen. "Cryptogenic cirrhosis: what are we missing?." Current gastroenterology reports 12.1 (2010): 40-48.