Amyloid A gastrointestinal amyloidosis associated with idiopathic retroperitoneal fibrosis. Report of a rare autopsy case and review of the literature.
We report a rare autopsy case of secondary gastrointestinal amyloid A (AA) amyloidosis associated with idiopathic retroperitoneal fibrosis (IRF) in a 67-year-old woman. Masses were identified around the aorta and inferior vena cava in her abdomen. Histologically, plasma cell infiltration was observed within fibrotic areas. Because no specific cause for the inflammatory mass was apparent, we diagnosed it as IRF. Steroid therapy, which usually reduces IRF masses, proved ineffective, and malabsorption syndrome developed 4 years later. On autopsy, amyloid protein was present systemically in the vascular walls of several organs, and deposition was highest in the gastrointestinal mucosa. Amyloid protein was identified as AA type, strongly suggesting that the amyloidosis was secondarily induced by IRF. To our knowledge, only 2 other cases of IRF-associated amyloidosis have been reported. Two of the 3 patients of these cases were women who showed resistance to steroid therapy.
Department of Clinical Pathology, Toyooka Hospital, Toyooka City, Hyogo, Japan. hosakan@mxa.nkansai.ne.jp
Naoki
N
Hosaka
Masaaki
M
Ito
Yoji
Y
Taki
Hiroshi
H
Iwai
Junko
J
Toki
Susumu
S
Ikehara
D000368:Aged; D000686:Amyloidosis / Q000209:etiology* / Q000473:pathology; D001012:Aorta, Abdominal / Q000737:chemistry / Q000187:drug effects / Q000473:pathology; D001344:Autopsy; D004351:Drug Resistance; D017809:Fatal Outcome; D005260:Female; D005767:Gastrointestinal Diseases / Q000209:etiology* / Q000473:pathology; D006801:Humans; D012185:Retroperitoneal Fibrosis / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D000685:Serum Amyloid A Protein / Q000378:metabolism*; D014682:Vena Cava, Inferior / Q000737:chemistry / Q000187:drug effects / Q000473:pathology
D000685:Serum Amyloid A Protein