Liver biopsy findings may include variable degrees of steatosis, inflammation, and fibrosis.
Dr. Lawrence S. Friedman
In one case we have seen, the patient presented with unexplained ascites and features suggestive of Budd-Chiari syndrome. The serum ascites albumin gradient was 2.3 with a total protein of 0.8 g/dL, and albumin 0.5 g/dL, with an ascitic WBC count of 88/mm3.
Dr. Paul Martin
Echocardiography showed an ejection fraction of 80%. Transjugular liver biopsy revealed a normal hepatic venous pressure gradient but marked sinusoidal dilatation and congestion with hepatocyte atrophy and focal necrosis suggestive of vascular outlet obstruction (Figure 1).
Courtesy Gastro Hep Advances/AGA
Hepatic venography, however, showed no evidence of Budd-Chiari syndrome. When seen in consultation, pertinent observations included Irish ancestry, a history of occasional diarrhea, short stature, osteoporosis, and an atrophic spleen on computed tomography. An IgA transglutaminase antibody was positive, and a small-bowel biopsy confirmed celiac disease (Figure 2).
Courtesy Gastro Hep Advances/AGA
On a gluten-free diet, the patient’s symptoms resolved, with clinical and laboratory abnormalities returning to normal. She lived another 20 years before dying of primary pulmonary hypertension. Recognition of an unusual hepatic manifestation of celiac disease led to effective management.
Dr. Friedman is the Anton R. Fried, MD, Chair of the department of medicine at Newton-Wellesley Hospital in Newton, Mass., and assistant chief of medicine at Massachusetts General Hospital, and a professor of medicine at Harvard Medical School and Tufts University School of Medicine, all in Boston. Dr. Martin is chief of the division of digestive health and liver diseases at the Miller School of Medicine, University of Miami, where he is the Mandel Chair of Gastroenterology. The authors disclose no conflicts.
Previously published in Gastro Hep Advances. 2023. doi: 10.1016/j.gastha.2023.03.018.
