Lupus and the Liver
Daniel J. Wallace, MD, Associate Clinical Professor of Medicine, UCLA School of Medicine.
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, sometimes known as lupoid hepatitis. This article attempts to reconcile our differing 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 yellowish hue, is seen in l-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 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 anti-coagulation (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 anti-inflammatory medication, gentle diuresis and occasionally periodic drainage. Ascites also may result from liver failure or nephrotic syndrome.
Abnormal liver function testsmay 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 non-steroidal anti-inflammatory 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 non-steroidals 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.
This leaves us with the most perplexing problem to discuss: lupoid hepatitis. Described by Joske and King in 1955 and named by Mackay in 1956, lupoid hepatitis has undergone many changes in definitions. The overwhelming majority of patients who were told they had lupoid hepatitis between 1955 and 1975 would not fulfill current criteria for this disease. Initially thought of as the presence of chronic active hepatitis (hepatitis means inflammation of the liver) with LE cells, the term "auto-immune hepatitis" seemed more appropriate since few of these patients had typical clinical lupus. The development of diagnostic tests to detect hepatitis A, B and more recently C changed our concepts of lupoid hepatitis. The current working definition of lupoid hepatitis is:
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Even using these criteria, only 10% of antoimmune hepatitis patients at the Mayo Clinic fulfilled the American College of Rheumatology (ACR) criteria for SLE. Although fevers, joint aches, malaise and loss of appetite are common (as well as jaundice with itching), many of the physical findings we associate with SLE (rashes, other organ involvement) are usually absent. This is further complicated by the knowledge that lupus patients have compromised immune systems and can develop a viral hepatitis, take liver-toxic medications, and some abuse alcohol just as non-lupus patients do, which can lead to a chronic active hepatitis. Thirty to sixty percent of patients with autoimmune hepatitis, which is the term I prefer, also have antibodies to smooth muscle or mitochondria (AMA or SMA).
Untreated, autoimmune hepatitis is fatal within five years. It can respond to prednisone and other steroids, 6-mercaptopurine, and azathioprine. Several of our patients have undergone successful liver transplants. We have come a long way in our understanding of liver disease in SLE and "lupus hepatitis," but it still takes a great deal of skill to sort out what is autoimmune versus viral versus medication-related.
Reprinted from the Lupus Courier (Lupus Society of Alberta) with thanks - also with thanks to the original source LFA Lupus News (Lupus Foundation of America).
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