Clinical Images: Liver nodular regenerative hyperplasia in antisynthetase syndrome

Liver nodular regenerative hyperplasia (LNRH) is a rare hepatic widespread nodular‐like lesion in which generalized transformation of the hepatic parenchyma into multiple nonmalignant regenerative nodules occurs. LNRH develops as a result of an underlying persistent and systemic inflammatory status, such as autoimmune disease (1, 2, 3). The patient, a 56‐year‐old woman, had a medical history of antisynthetase syndrome with positive anti–glycyl–transfer RNA synthetase antibody, proximal girdle weakness with a high serum level of creatine phosphokinase, Raynaud phenomenon of fingers, and interstitial lung disease. She presented to the emergency department with progressive abdominal distention and bilateral lower extremities edematous change. A laboratory examination revealed elevated alanine aminotransferase (368 U/l) and aspartate aminotransferase (192 U/l) levels but a normal alpha‐fetoprotein level (1.04 ng/ml). Abdominal computed tomography (A: axial section, venous phase, slice thickness 5 mm; B: coronal reformatted section, venous phase, slice thickness 5 mm) showed numerous widespread hepatic enhancing nodules and massive ascites without any lymphadenopathy in the para‐aortic retroperitoneum or pelvic cavity. Initially, hepatocellular carcinoma or liver metastasis was suspected. However, a liver biopsy showed sinusoidal dilatation but no malignant cell, significant lobular or portal inflammation, or advanced fibrosis by Masson trichrome stain. Plasma cells were not overly represented by a Mum1 stain. Iron stain and Congo red stain results were also negative. A reticulin stain of the liver (C) demonstrated thickened hepatic cell plates (arrows) compressing adjacent hepatocytes (arrowheads) suggestive of LNRH. Thus, LNRH in antisynthetase syndrome was diagnosed. Systemic intravenous methylprednisolone (2 mg/kg/day) was administered, with the dose gradually tapered over a 2‐week period. Her antisynthetase syndrome–related LNRH was under control with improvements of liver enzymes and ascites after taking oral prednisolone and azathioprine.

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