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Case Reports
. 2024 Feb 15;63(4):593-599.
doi: 10.2169/internalmedicine.1806-23. Epub 2023 Jul 5.

Systemic Amyloid A Amyloidosis Secondary to Xanthogranulomatous Pyelonephritis

Masato Habuka  1 , Mizusa Nishikiori  1 , Chihiro Oikawa  1 , Megumi Takahashi  1 , Yuichi Sakamaki  1 , Asa Ogawa  1 , Norio Miyajima  2 , Yasuhiko Tanabe  3 , Keiichi Honma  4 , Kunihiko Wakaki  4 , Suguru Yamamoto  5 , Ichiei Narita  5
Affiliations
Case Reports

Systemic Amyloid A Amyloidosis Secondary to Xanthogranulomatous Pyelonephritis

Masato Habuka et al. Intern Med. .

Abstract

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.

Keywords: serum amyloid A; systemic AA amyloidosis; xanthogranulomatous pyelonephritis.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
CT (A: left kidney tumor; B: right thyroid tumor). A left kidney tumor with a well-defined border and a mixture of high- and low-density areas (A, arrow) and a right thyroid tumor (B, arrow) with no marked changes in size since follow-up CT six months prior.
Figure 2.
Figure 2.
Histological findings in the lower gastrointestinal tract (A: Congo red staining; B: immunostaining with anti-AA antibody). Amyloid was deposited in the specimen obtained by the lower gastrointestinal tract and showed positive immunostaining with anti-AA antibody, indicating AA amyloidosis. Magnification: ×10. IHC: immunohistochemistry
Figure 3.
Figure 3.
Histological findings at left nephrectomy (A: macroscopic findings; B: Hematoxylin and Eosin staining; C: immunostaining with anti-CD68 antibody; D: immunostaining with anti-CD45 antibody). A: A yellowish nodule measuring 1.8×1.8×2.0 cm was present on the cut surface. B-D: This examination revealed xanthogranulomatous kidney inflammation with abundant lipid-laden foamy macrophages and lymphocytes. CD68 is a marker of macrophages, and CD45 is a marker of lymphocytes. Magnification: ×10.
Figure 4.
Figure 4.
Histological findings after the biopsy (A: the residual renal parenchyma; B: thyroid; C: myocardium; D: stomach). A: Amyloid deposited in the mesangium and vascular walls of the residual renal parenchyma. B-D: Amyloid deposited in the thyroid, myocardium, and stomach tissues.
Figure 5.
Figure 5.
Clinical course of the patient after the onset of diarrhea.
See this image and copyright information in PMC

References

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