The Natural History of Primary Biliary Cirrhosis

Pyoderma gangrenosum associated with primary biliary cirrhosis

“…Prednisone In the patient described herein, sodium iodide I 125 therapy had been administered during the most recent admission, but had not been given at the time of initial presentation of pyoderma gangrenosum. In the setting of (1) negative bacterial cultures of lesion and blood, (2) lack of response to antibiotics, (3) pathergy, (4) iodine exacerba¬ tion, (5) evolving clinical picture, (6) history of similar lesions with residual scarring, (7) biopsy findings, (8) rapid response to prednisone, (9) and a residual cribriform scar after healing, the diagnosis of pyoderma gangrenosum was firmly established in this patient.…”

Primary Biliary Cirrhosis

“…Elevated serum alkaline phos phatase level provided an indication for liver biopsy which showed features of non-suppurative destructive cholangitis. Since then, there has been increasing recognition of 'asymptomatic' or 'pre-symptomatic' disease [4][5][6][7][8][9][10][11][12][13] (fig. 1).…”

“…Gastrointestinal (GI) bleeding, the present ing symptom in only 6% at diagnosis, had occurred in 46% after 5 years. Bleeding is usually variceal; thus Kapelman [13] found variceal haemorrhage accounted for 57% of instances of bleeding, and gastric or duode nal ulceration for only 10%, although in 27% of cases the site of bleeding was un known. Ascites, present in 7% at diagnosis, had developed in 49% at 5 years although of the entire group.…”

Primary Biliary Cirrhosis

“…The natural history of PBC has been described in detail elsewhere (6,9,12,13). The disease is usually slowly progressive and ultimately fatal.…”