PubMed · 5947841
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A Lemaire, J Picard, E Housset, P Samair, J Dupuis. 1966. [Jirgl's test].. https://pubmed.ncbi.nlm.nih.gov/5947841/
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One 63-year-old woman, who presented with cholestatic jaundice due to common bile duct compression produced by primary retroperitoneal fibrosis, is studied. The patient was operated six years ago because of hydronephrosis, when the disease was first diagnosed. Magnetic resonance cholangiopancreatography (MRCP) revealed the presence of extrahepatic bile duct obstruction, which once was considered to be pathognomonic of pancreatic cancer. CT-scan demonstrated the change of retroperitoneal fibrosis around left kidney, atrophy of right kidney, and obstruction of extrahepatic bile duct (pancreatic head). An explorative laparotomy was performed, and the retroperitoneum and pancreas were grayish-white and hard, the fibrotic pancreatic head compressed the common bile duct. Bile duct stricture was managed by Rouxen-Y hepatocholangio-jejunostomy. To the best of our knowledge, few similar cases of retroperitoneal fibrosis have been reported.
The differential diagnosis of fever of unknown origin includes infectious diseases, malignancies and connective tissue diseases. Multiple myeloma is not usually included in the differential diagnosis, nor is it considered a frequent cause of cholestasis in the absence of amyloidosis. We report a case of multiple myeloma in a patient presenting with a long-standing fever and cholestasis. The patient underwent a long and unfruitful series of diagnostic procedures, including liver biopsy. The clue to the diagnosis, established with a marrow biopsy, was the presence of a paraprotein in the serum.
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