Contributed by Pal Kaposi-Novak, MD, PhD, Mordechai Rabinovitz, MD and Bruce Rabin MD, PhD
Contributed by Pal Kaposi-Novak, MD, PhD, Mordechai Rabinovitz, MD and Bruce Rabin MD, PhD
PATIENT HISTORY:
A 61-year-old female patient presented to UPMC Presbyterian Hospital with right upper quadrant pain and jaundice. She had non-specific complaints of feeling tired, and having dull pain in her shoulders for years. Recently, one of her co-workers noted that she was "yellow". The patient's medical history was significant for fibromyalgia, treated hypothyroidism and treated depression. Physical examination revealed a mildly enlarged, tender liver with a firm liver edge palpable 2 cm below the thoracic arch. Liver function tests showed AST of 360 U/mL, ALT 519 U/mL, ALP 94 U/mL GGT 327 U/mL and bilirubin 9.4 mg/mL. Alpha-1-antitrypsin, ceruloplasmin, and ferritin were within normal limits. No peripheral blood eosinophilia was present, but the patient had slight leukocytosis with a white blood cell count of 10,000/ml. There was no clinical sign suggestive of pancreatitis or cholecystitis. The patient's medications contained Synthroid, Celexa, Vitamin B12, Vitamin D, and Tramadol-hydrochloride tablets. A contrast enhanced abdominal CT scan was performed. It showed normal liver morphology with no focal lesions, no enhancement abnormalities, and no bile duct dilatation. The gallbladder and the pancreas were unremarkable.
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No hepatitis A, HBsAg or to HBe antibodies were detected by serology. Quantitative PCR tests for hepatitis C and hepatitis B were also negative. The serum IgG immunoglobulin level was elevated to 2130 mg/dL, while IgA and IgM globulins remained within the normal range. The autoantibody panel was positive for low titer (1:44) anti-smooth muscle (anti-SMA) antibody (FIGURE 1). Antibodies to endomysium, mitochondria, and to liver-kidney microsomal antigens were absent. Indirect immunofluorescence showed homogenous staining with antinuclear antibodies at a 1:40 titer (FIGURE 2). Antineutrophil antibodies were not detected.
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An ultrasound guided liver biopsy was performed. Histology showed severe portal/periportal and lobular hepatitis. The microscopic picture was characterized by marked hepatocellular swelling, hepatocanalicular cholestasis, as well as confluent perivenular hepatocyte dropout and confluent birding necrosis (FIGURE 3). The pattern of injury appeared to be relatively acute, although signs of transition towards chronicity such as focal periportal fibrosis and activated myofibroblasts were also present (FIGURE 4).
