Masato Habuka: Division of Nephrology, Niigata Prefectural Shibata Hospital, Japan.
Mizusa Nishikiori: Division of Nephrology, Niigata Prefectural Shibata Hospital, Japan.
Chihiro Oikawa: Division of Nephrology, Niigata Prefectural Shibata Hospital, Japan.
Megumi Takahashi: Division of Nephrology, Niigata Prefectural Shibata Hospital, Japan.
Yuichi Sakamaki: Division of Nephrology, Niigata Prefectural Shibata Hospital, Japan.
Asa Ogawa: Division of Nephrology, Niigata Prefectural Shibata Hospital, Japan.
Norio Miyajima: Division of Urology, Niigata Prefectural Shibata Hospital, Japan.
Yasuhiko Tanabe: Division of Cardiology, Niigata Prefectural Shibata Hospital, Japan.
Keiichi Honma: Division of Pathology, Niigata Prefectural Shibata Hospital, Japan.
Kunihiko Wakaki: Division of Pathology, Niigata Prefectural Shibata Hospital, Japan.
Suguru Yamamoto: Division of Clinical Nephrology and Rheumatology, Kidney Research Center, Niigata University Graduate School of Medical and Dental Sciences, Japan.
Ichiei Narita: Division of Clinical Nephrology and Rheumatology, Kidney Research Center, Niigata University Graduate School of Medical and Dental Sciences, Japan.
The combination of systemic amyloid A (AA) amyloidosis and xanthogranulomatous pyelonephritis (XGP) resulting from a chronic urinary tract infection is extremely rare. We herein report a case of systemic AA amyloidosis secondary to XGP for which clinical remission developed after nephrectomy. To our knowledge, this is the first case report describing the clinical improvement of systemic AA amyloidosis secondary to XGP after nephrectomy in Japan. Clinicians should be aware of this uncommon combination and search for amyloid depositions in cases of XGP.