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<p>Renal limited sarcoidosis is an exceptionally rare condition characterized by non-caseating granulomas confined solely to the kidneys, without involvement of other organs. Its diagnosis is challenging because of the absence of systemic manifestations. We present a case of a 74-year-old woman with a history of type 2 diabetes mellitus, papillary thyroid carcinoma, and osteoporosis, who showed progressive renal dysfunction. Laboratory findings revealed elevated serum creatinine and urinary β₂-microglobulin, indicating tubular injury. Kidney biopsy demonstrated granulomatous interstitial nephritis with non-caseating epithelioid cell granulomas. Other potential causes, including infections, drug-induced nephritis, and autoimmune diseases were excluded. Chest computed tomography and gallium-67 scintigraphy revealed no extrarenal involvement. Based on these findings, we diagnosed the patient as having renal limited sarcoidosis. The patient was treated with low dose of oral prednisolone, resulting in significant improvement in renal function and normalization of laboratory parameters. The present case underscores the importance of considering the possibility of renal limited sarcoidosis on differentiating the case with unexplained renal dysfunction, especially when non-caseating granulomas are identified on kidney biopsy and other causes have been excluded. Corticosteroids remain the cornerstone of treatment, with most patients responding favorably. However, in steroid-resistant cases, alternative immunosuppressive agents such as mycophenolate mofetil and infliximab have been employed, though their efficacy and safety require further investigation. In this article, we present this rare case in addition to providing comprehensive literature review of previously cases to summarize clinicopathological characters, treatment strategies, and renal outcomes of renal-limited sarcoidosis for future clinical management.</p>

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Renal limited sarcoidosis presenting with non-caseating granulomatous interstitial nephritis in the absence of extrarenal manifestations: a case report and literature review

  • 期刊论文

  • 发表日期：

    2026年1月7日

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  • Kiho Yanagisawa,
  • Kazuhiro Takeuchi,
  • Yukihiro Wada,
  • Genichi Saito,
  • Mayuko Hanyuda,
  • Hiroyuki Okawa,
  • Ryota Uchitsubo,
  • Sayumi Kawamura,
  • Shun Sakurabayashi,
  • Keiko Sano,
  • Tomomi Motohashi,
  • Shokichi Naito,
  • Togo Aoyama,
  • Yasuo Takeuchi

摘要

Renal limited sarcoidosis is an exceptionally rare condition characterized by non-caseating granulomas confined solely to the kidneys, without involvement of other organs. Its diagnosis is challenging because of the absence of systemic manifestations. We present a case of a 74-year-old woman with a history of type 2 diabetes mellitus, papillary thyroid carcinoma, and osteoporosis, who showed progressive renal dysfunction. Laboratory findings revealed elevated serum creatinine and urinary β₂-microglobulin, indicating tubular injury. Kidney biopsy demonstrated granulomatous interstitial nephritis with non-caseating epithelioid cell granulomas. Other potential causes, including infections, drug-induced nephritis, and autoimmune diseases were excluded. Chest computed tomography and gallium-67 scintigraphy revealed no extrarenal involvement. Based on these findings, we diagnosed the patient as having renal limited sarcoidosis. The patient was treated with low dose of oral prednisolone, resulting in significant improvement in renal function and normalization of laboratory parameters. The present case underscores the importance of considering the possibility of renal limited sarcoidosis on differentiating the case with unexplained renal dysfunction, especially when non-caseating granulomas are identified on kidney biopsy and other causes have been excluded. Corticosteroids remain the cornerstone of treatment, with most patients responding favorably. However, in steroid-resistant cases, alternative immunosuppressive agents such as mycophenolate mofetil and infliximab have been employed, though their efficacy and safety require further investigation. In this article, we present this rare case in addition to providing comprehensive literature review of previously cases to summarize clinicopathological characters, treatment strategies, and renal outcomes of renal-limited sarcoidosis for future clinical management.