Concurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis.

Abstract

Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis, except epidemic schistosomiasis. Among them, primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues, giving rise to systemic amyloidosis, due to a progressive and unresolved inflammatory process, and its possible association with inflammatory bowel diseases. Nevertheless, only one such case has been reported in the literature to date. We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis, without any evidence of other inflammatory disorders. As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4, the presence of IgG4(+) plasma cells was examined systemically, resulting in unexpected documentation of Congo-red-positive amyloid deposits, but not IgG4(+) plasma cells, in the liver, stomach and salivary glands. Elevated serum IgG4 is the hallmark of IgG4-related disease, including IgG4-associated cholangitis, but it has also been demonstrated in certain patients with PSC. Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.

• Authors: Takehiro Kato, Atsumasa Komori, Sung-Kwan Bae, Kiyoshi Migita, Masahiro Ito, Yasuhide Motoyoshi, Seigo Abiru, Hiromi Ishibashi
• Journal: World journal of gastroenterology (World J Gastroenterol) Vol. 18 Issue 2 Pg. 192-6 (Jan 14 2012) ISSN: 2219-2840 [Electronic] United States
• PMID: 22253527 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Amyloid
• Immunoglobulin G

Topics

• Aged
• Amyloid (blood)
• Amyloidosis (pathology, physiopathology)
• Asian People
• Cholangiopancreatography, Endoscopic Retrograde
• Cholangitis (immunology, pathology, physiopathology)
• Cholangitis, Sclerosing (pathology, physiopathology)
• Diagnosis, Differential
• Female
• Humans
• Immunoglobulin G (blood)
• Immunoglobulin Light-chain Amyloidosis