IgG4-related disease manifesting as interstitial nephritis accompanied by hypophysitis

Ken Matsuda, Ayako Saito, Yoichi Takeuchi, Hirotaka Fukami, Hiroyuki Sato, Tasuku Nagasawa

Research output: Contribution to journalArticlepeer-review

5 Citations (Scopus)


Objective: Rare disease Background: IgG4-related disease is a systemic disease with marked infiltration of IgG4-positive plasma cells into affected organs and elevated serum IgG4. On clinical examination, swelling, nodules, and hypertrophic lesions might appear simultaneously or metachronously in different organs. Case Report: An 85-year-old man with sudden-onset polydipsia and polyuria insipidus was transported to our hospital because of hypothermia and general malaise. Laboratory tests revealed renal failure and central diabetes insipidus. According to his serum IgG4 level, the patient was diagnosed with possible IgG4-related kidney disease accompanied by IgG4-related hypophysitis. Abdominal contrast-enhanced computed tomography, hypophysis magnetic resonance imaging, and histological examination of the kidney were performed. Glucocorticoid therapy was administered and his renal function improved gradually. However, his central diabetes insipidus did not improve. Conclusions: Glucocorticoid therapy showed different therapeutic effects on the kidney and posterior lobe of the hypophysis. It is possible that glucocorticoid therapy needs to be supported by other immunomodulatory therapies to have an effect on all affected organs.

Original languageEnglish
Pages (from-to)593-598
Number of pages6
JournalAmerican Journal of Case Reports
Publication statusPublished - 2017 May 29


  • Diabetes insipidus
  • Immunoglobulin G
  • Kidney diseases
  • Neurogenic
  • Pituitary diseases


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