Wild-type ATTR amyloidosis of the ureter in a 56-year-old woman with rheumatoid arthritis and Sjögren's syndrome.
We present a case of acute pyelonephritis with right hydronephrosis in a middle-aged woman, who had suffered from rheumatoid arthritis and Sjögren's syndrome. She had successfully treated with antibiotics, however, ureteral stenosis sustained. She underwent ureteroscopy and stenting of right ureter. Biopsy specimen revealed submucosal amyloid deposition in the interstitium overlying a benign urothelium. Amyloid protein was positive for transthyretin (TTR) by immunohistochemistry and amyloid deposition was not demonstrated in other organs. The patient's TTR genes were wild type and she was diagnosed with wild-type ATTR (ATTR wt) amyloidosis. This is the first report about symptomatic ATTR wt amyloidosis, which was also called 'systemic senile amyloidosis (SSA)' in the ureter. We should aware that SSA can occur at younger age and cause symptomatic ureteral stenosis. Further investigation is needed to clarify the association of autoimmune diseases to develop ATTR wt amyloidosis.
First Department of Internal Medicine, University of Toyama Japan.
Koichiro
K
Shinoda
First Department of Internal Medicine, University of Toyama Japan.
Hirofumi
H
Taki
Department of Morphological and Physiological Sciences, Graduate School of Life Sciences, Kumamoto University Japan.
Konen
K
Obayashi
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University Japan.
Yukio
Y
Ando
Department of Urology, University of Toyama Japan.
Akihiko
A
Watanabe
Department of Urology, University of Toyama Japan.
Akira
A
Komiya
Department of Urology, University of Toyama Japan.
Hideki
H
Fuse
Department of Diagnostic Pathology, Graduate School of Medical and Pharmaceutical Sciences, University of Toyama Japan.
Koichi
K
Tsuneyama
First Department of Internal Medicine, University of Toyama Japan.
Kazuyuki
K
Tobe
D028227:Amyloid Neuropathies, Familial / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D001172:Arthritis, Rheumatoid / Q000150:complications* / Q000175:diagnosis; D001706:Biopsy; D005260:Female; D005819:Genetic Markers; D006801:Humans; D006869:Hydronephrosis / Q000175:diagnosis / Q000209:etiology; D008875:Middle Aged; D011228:Prealbumin / Q000235:genetics*; D011704:Pyelonephritis / Q000175:diagnosis / Q000209:etiology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis; D015607:Stents; D014517:Ureteral Obstruction / Q000175:diagnosis / Q000209:etiology* / Q000235:genetics / Q000601:surgery; D018666:Ureteroscopy / Q000295:instrumentation
D005819:Genetic Markers; D011228:Prealbumin
Rheumatoid arthritis; Sjögren’s syndrome; senile systemic amyloidosis; ureteral amyloidosis; wild-type transthyretin amyloidosis