Connective Tissue Diseases and the Liver

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Journal of Clinical Gastroenterology 2002; 35(4):345-349

Connective Tissue Diseases and the Liver

Wael I. Youssef, M.D.; S. Tavill, M.D.

Abstract

Connective tissue diseases such as systemic lupus erythematosus (SLE),

rheumatoid arthritis, Sjögren's syndrome, and scleroderma are systemic

disorders that may have an autoimmune basis. The system manifestations vary,

and there is frequent overlap among the syndromes. Liver involvement in

patients with connective tissue diseases has been well documented but is

generally considered rare. Although advanced liver disease with cirrhosis

and liver failure is rare in patients with connective tissue diseases,

clinical and biochemical evidence of associated liver abnormalities is

common. Previous treatment with potentially hepatotoxic drugs or coincident

viral hepatitis has usually been implicated as the main causes of liver

disease in patients with connective tissue diseases. However, even after

careful exclusion of these etiologies, the question remains whether to

classify the patient as having a primary liver disease with associated

autoimmune, clinical, and laboratory features or as having liver disease as

a manifestation of generalized connective tissue disease. The main example

of this pathogenetic dilemma is autoimmune hepatitis and SLE-associated

hepatitis, which have been regarded as two different entities, although they

have features in common of autoimmune syndromes. Several clinical and

histopathologic features have been used to discriminate autoimmune hepatitis

from SLE, a relevant diagnostic exercise because complications and therapy

are quite different. Although hepatic steatosis and abnormal results on

biochemical liver function tests are the most common hepatic abnormalities

associated with connective tissue diseases, other less frequent

abnormalities have been noted, such as nodular regenerative hyperplasia,

portal vein obliteration and portal hypertension, features of primary

biliary cirrhosis, and rarely portal fibrosis with abnormal lobular

architecture. Vascular disorders of the liver also have been described, such

as Budd-Chiari syndrome. Histologic assessment may reveal a variety of

subclinical liver diseases. The aim of this contribution is to review the

current published data regarding liver involvement in connective tissue

diseases.