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Two cases with hepatic amyloidosis suspected of having primary sclerosing cholangitis
Author(s) -
Hirano Kenji,
Ikemura Masako,
Mizuno Suguru,
Tada Minoru,
Isayama Hiroyuki,
Watanabe Takeo,
Saito Tomotaka,
Kawahata Shuhei,
Uchino Rie,
Hamada Tsuyoshi,
Miyabayashi Koji,
Sasaki Takashi,
Kogure Hirofumi,
Yamamoto Natsuyo,
Sasahira Naoki,
Koike Kazuhiko,
Fukayama Masashi,
Sugawara Yasuhiko,
Kokudo Norihiro
Publication year - 2013
Publication title -
hepatology research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.123
H-Index - 75
eISSN - 1872-034X
pISSN - 1386-6346
DOI - 10.1111/hepr.12034
Subject(s) - medicine , amyloidosis , magnetic resonance cholangiopancreatography , liver transplantation , primary sclerosing cholangitis , differential diagnosis , jaundice , liver biopsy , pathology , biopsy , endoscopic retrograde cholangiopancreatography , radiology , gastroenterology , transplantation , disease , pancreatitis
Sclerogenic biliary changes in hepatic amyloidosis are seldom observed. Here, we report two recent cases initially suspected as primary sclerosing cholangitis ( PSC ), which were later diagnosed as hepatic amyloidosis ( AL type). Case 1: On the basis of magnetic resonance cholangiopancreatography ( MRCP ) findings, PSC was suspected in a 41‐year‐old woman with jaundice. Computed tomography ( CT ) showed nodular pulmonary lesions and swollen cervical, mediastinal and para‐aortic lymph nodes, the cause of which was unknown despite detailed examinations. Because of rapid deterioration in the patient's liver function, living donor liver transplantation was performed. She was then diagnosed with hepatic amyloidosis, but died of heart failure due to cardiac amyloidosis 74 days after surgery. Case 2: On the basis of MRCP findings, PSC was suspected in a 49‐year‐old woman with jaundice. CT showed multiple cystic pulmonary lesions, and hypogammaglobulinemia was also observed (immunoglobulin G , 481 mg/dL). After a biliary plastic stent was placed, liver and lung biopsy confirmed the presence of amyloid deposition. These two cases indicate that it is important to consider hepatic amyloidosis as a differential diagnosis of PSC . The presence of atypical extrahepatic lesions may be useful clues for confirming the diagnosis.