Primary Billiary Cirrhosis (Antimitochondrial Antibody Negative) Leading to Secondary Amyloidosis

A 49 years old lady presented with low-grade fever (99-100°F) for 2 years. During this time she was extensively workedup for pyrexia of unknown origin but no diagnosis could be established. Her Initial blood work-up was all negative except high alkaline phosphatase and gamma GT (374 IU and 195 IU respectively). She later presented to our tertiary care centre with facial swelling, flushing and bilateral pedal swelling for 3 months. Along with generalized body swelling she had frothy urine. She was diagnosed as nephrotic syndrome on the basis of nephrotic range proteinuria. Her Renal biopsy done for workup of nephrotic was positive for AA amyloid. Also, her gastrointestinal biopsy was suggestive of amyloidosis. As a workup for secondary amyloidosis, her liver biopsy was done which revealed features of primary billiary cirrhosis (PBC).