LUPUS and the Liver

Involvement of the liver in SLE is a frequently misunderstood complication of the disease. The liver can be affected as a result of lupus itself, as well as the medications used to treat inflammation caused by lupus. There is also a specific inflammatory disease of the liver, related to SLE, called lupoid hepatitis. This column attempts to reconcile our perceptions of what "lupus in the liver" really means.

Lupus can affect the liver in numerous ways that are delineated below:

• ENLARGEMENT OF THE LIVER, or hepatomegaly, is found in 10% of SLE patients. The liver is rarely tender unless the enlargement is so great that the capsule (or covering) of the organ is stretched. The most common cause of large livers in lupus include lupoid hepatitis, congestive heart failure, or cirrhosis.
• JAUNDICE, in which the person's skin has a yellowish hue, is seen in 1-4% of patients with SLE. Manifested by high levels of bilirubin which are responsible for this pigmentation, jaundice results from autoimmune hemolytic anemia, viral hepatitis, cirrhosis, or bile duct obstruction (from gallstones, tumor, or pancreatitis). Occasionally, certain medications including nonsteroidal anti- inflammatory drugs and azathioprine may produce jaundice.
• HEPATIC VASCULITIS, or inflammation of the small and medium sized arteries of the liver, is extremely rare and is noted in one lupus patient per thousand. It responds to corticosteroids.
• BUDD-CHIARI SYNDROME (which is very rare) results from a blood clot in the portal veins which drain materials from the liver. Lupus patients with the lupus anticoagulant, anticardiolipin antibody, or antiphospholipid syndrome appear to be uniquely at risk for developing these clots. Additionally, hepatic artery clots may occur. Untreated Budd-Chiari can lead to ascites, portal hypertension, and liver failure. The preferred treatment of Budd-Chiari syndrome is anticoagulation (blood thinning).
• ASCITES is a term which refers to fluid in the abdomen as a result of the manufacture of this liquid by the peritoneum, or lining of the abdominal cavity. Often associated with serositis (or similar fluid being made by the pleura or pericardium which line the lung and heart), ascites causes swelling of the abdomen and is noted in 10% of patients with SLE. Usually reflecting active disease, it may be painless or painful and can be mistaken for a "surgical abdomen" resulting in unnecessary surgery. If an infection is ruled out, ascites is treated with antiinflammatory medication, gentle diuresis, and occasionally periodic drainage. Ascites also may result from liver failure or nephrotic syndrome.
• ABNORMAL LIVER FUNCTION TESTS may be found in 30-60% of patients with SLE. Blood enzyme evaluations included in routine blood panels such as the AST (also called SGPT), ALT (also called SGOT), alkaline phosphates and GGT may be slightly elevated as a result of a variety of mechanisms. First of all, nearly all nonsteroidal antiinflammatory agents, as well as aspirin, can elevate these enzymes, and lupus patients appear to be particularly susceptible to this. These minor abnormalities are usually of little consequence and I ignore them unless they are greater than three times normal. Also, active lupus can elevate these enzymes. Most nonsteroidals can be stopped for a week or two and the enzymes rechecked. If they remain increased, the possibilities for this elevation include hepatitis, infection, biliary disease, alcoholism, or active lupus.

1. liver pathology consistent with chronic active hepatitis
2. absence of evidence for active hepatitis virus A, B or C infection
3. a positive ANA or LE cell prep

Untreated, autoimmune hepatitis is fatal within five years. It can respond to prednisone, steroids, alpha interferon, and azathioprine. Two of our patients have undergone successful liver transplants. We have come a long way in our understanding of liver disease in SLE and lupoid hepatitis, but it still takes a great of skill to sort out what is autoimmune versus viral versus medication related.