title: Renal transplantation in AA amyloidosis associated with Whipple's disease.
abstract: Whipple's disease (WD) is a chronic infection caused by Thropheryma whipplei that usually manifests with intestinal, articular, pulmonary, neurological and cardiac abnormalities. Rarely, WD has been associated with renal AA amyloidosis.We report a 50 year-old male with nephrotic syndrome and renal failure whose renal biopsy revealed extensive AA amyloidosis. Amyloid was not found in other organs, namely in gastrointestinal tract and bone marrow. There was no evidence of chronic inflammatory disease, and despite detailed investigation, the diagnosis of the underlying disease remained obscure. Eight months after referral he started peritoneal dialysis. Three years later he developed anorexia, weight loss, anemia, and recurrent attacks of non-bloody diarrhea. A biopsy of the small intestine showed typical histological findings of WD and PCR was positive for T. whipplei. He was treated with ceftriaxone followed by co-trimoxazole, with remission of complaints and histological features. Three years later the patient underwent successful cadaveric kidney transplantation. In this case, AA amyloidosis preceded the manifestations of WD. To the best of our knowledge, this is the first report of kidney transplantation in a patient with amyloidosis due to WD. Recurrence of amyloidosis in renal graft is not expected.
authors: Department of Nephrology, Hospital de Santo António, Porto, Centro Hospitalar do Porto, Portugal. sofiarocha81@gmail.com
authors: Sofia
authors: S
authors: Rocha
authors: Luísa
authors: L
authors: Lobato
authors: Maria João
authors: MJ
authors: Carvalho
authors: Jorge
authors: J
authors: Malheiro
authors: Ramón
authors: R
authors: Vizcaíno
authors: Anabela
authors: A
authors: Rodrigues
authors: António
authors: A
authors: Cabrita
mesh_terms: D000686:Amyloidosis / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002443:Ceftriaxone / Q000627:therapeutic use; D004386:Duodenum / Q000473:pathology; D006801:Humans; D007668:Kidney / Q000378:metabolism / Q000473:pathology; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000601:surgery; D000685:Serum Amyloid A Protein / Q000378:metabolism*; D015662:Trimethoprim, Sulfamethoxazole Drug Combination / Q000627:therapeutic use; D054851:Tropheryma; D008061:Whipple Disease / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000382:microbiology
chemical_list: D000900:Anti-Bacterial Agents; D000685:Serum Amyloid A Protein; D002443:Ceftriaxone; D015662:Trimethoprim, Sulfamethoxazole Drug Combination