Journal of the Louisiana State Medical Society
nuclear antibody levels were nega- tive, the anti-mitochondrial antibody level was elevated at 26.5 units (<20 units). C-ANCA, P-ANCA, alpha- 1-antitrypsin level, transferrin, ceru- loplasmin, and AFP were all normal. An outpatient liver biopsy was performed to further confirm the sus- pected diagnosis of PBC. The results revealed severe portal lymphocytic and granulomatous inflammation with small interlobular duct ductope- nia/destruction and extensive peri- portal necroinflammatory changes, consistent with early-stage primary biliary cirrhosis (Figures 3, 4). DISCUSSION Primary biliary cirrhosis (PBC) is a progressive autoimmune disease that primarily affects women. Of the estimated 9,232 cases of PBC in the United States in 1996, 88.7% occurred in females. 4 Autoimmune-mediated destruction of intrahepatic bile ducts eventually leads to bile flow obstruc- tion, hepatic fibrosis, cirrhosis, and eventual liver failure. The exact etiology continues to be a subject of investigation, but it is described by Gershwin and Mackay to be a combination of genetic predisposi- tion and underlying environmental factors. 5 Selmi et al. also suggest a genetic component by showing that the concordance of PBC in monozy- gotic twins is about 63%. 1 Based on a systematic review of epidemiologic studies published by Boonstra et al. in the Journal of Hepatology in 2012, worldwide incidence rates of PBC range from 0.33-5.8 per 100,000 and prevalence rates range from 1.91-40.2 per 100,000. 6 Due to improved awareness and diagnosis earlier in the clinical course, 50-60%of patients are asymp- tomatic at the time of diagnosis. 7 The most common symptoms seen in PBC are fatigue and pruritis. 8 Pruritis is estimated to occur in 25%-75% of pa- tients with PBC. It can often precede the actual diagnosis by several years, highlighting the need to consider PBC in any patient with pruritis that is not associated with a rash. 8 The
Figure 1: Axial contrast-enhanced CT demonstrates hepatosplenomegaly with an enlarged periportal lymph node (arrow), which measures greater than one centimeter in maximal short axis diameter.
Figure 2: Coronal contrast-enhanced images show hepatosplenomegaly with the liver and spleen measuring 28 and 23 centimeters in maximal craniocaudal dimensions, respectively.
130 J La State Med Soc VOL 166 May/June 2014
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