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Churg–Strauss syndrome manifesting as cholestasis and diagnosed by liver biopsy
Author(s) -
Harada Masaru,
Oe Shinji,
Shibata Michihiko,
Taguchi Masashi,
Matsuhashi Toru,
Hiura Masaaki,
Abe Shintaro,
Harada Riko,
Shimajiri Shohei
Publication year - 2012
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/j.1872-034x.2012.00993.x
Subject(s) - medicine , hypereosinophilia , cholestasis , liver biopsy , eosinophil , pathology , gastroenterology , endoscopic retrograde cholangiopancreatography , liver function tests , abdominal pain , biopsy , eosinophilia , asthma , pancreatitis
A 56‐year‐old woman was referred to our hospital due to fever and cholestatic liver dysfunction. Her eosinophil count was normal and she had no abdominal pain or neurological manifestations. We performed a liver biopsy and found fibrinoid necrosis of the hepatic artery with granulomatous reaction and eosinophilic infiltration in the portal area in the liver. Later, sensory abnormalities of the arms and legs appeared and the eosinophil count increased. Serum immunoglobulin E and immunoglobulin G4 were elevated and rheumatoid factor was strongly positive. Endoscopic retrograde cholangiopancreatography revealed no abnormality of the bile duct and pancreatic duct. We made a diagnosis of Churg–Strauss syndrome and began corticosteroid treatment. Fever and liver function immediately improved. In the present patient, Churg–Strauss syndrome manifested first in the liver, before hypereosinophilia and neural manifestations. We believe that Churg–Strauss syndrome is an autoimmune liver disease, and it is important to recognize that the liver may be involved in Churg–Strauss syndrome.