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IgG4-Related Disease Manifesting as Interstitial Nephritis Accompanied by Hypophysitis

Challenging differential diagnosis, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)

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

(Department of Nephrology, Japanese Red Cross Ishinomaki Hospital, Ishinomaki, Miyagi, Japan)

Am J Case Rep 2017; 18:593-598

DOI: 10.12659/AJCR.902187

Published: 2017-05-29

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.

Keywords: Diabetes Insipidus, Neurogenic, Immunoglobulin G, Kidney Diseases, Pituitary Diseases