Systemic lupus erythematosus associated with multiple nodular hyperplasia of the liver

Three autopsy cases of systemic lupus erythematosus with unique association of multiple nodular hyperplasia (MNH) of the liver, portal hypertension, and hypertensive pulmonary vascular disease are reported. None of the patients had received oral contraceptive or androgenic steroid, but they were treated with glucocorticoids for 2 to 11 years. Raynaud's phenomenon, sclerodactyly, and mild impairment of the kidney were the common clinical features. Macroscopically, MNH is characterized by many nodules scattered throughout the non-cirrhotic liver, and histologically, each nodule is made up of normal-appearing hepatocytes and not encapsulated. Portal tracts are scanty in the nodules. MNH seems to be a regenerative-hyperplastic process, but its true nature still remains unclear. Relationships between MNH and portal hypertension, MNH and pulmonary hypertension, and collagen disease and pulmonary hypertension are discussed. A brief review of the literature concerning multiple benign hepatocellular tumors similar to MNH is also presented.