Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
IgG4-related Tubulointerstitial Nephritis and Hepatic Inflammatory Pseudotumor without Hypocomplementemia
Fae KimKazunori YamadaDai InoueKenichi NakajimaIchiro MizushimaYasushi KakuchiHiroshi FujiiKenta NarumiMasami MatsumuraHisanori UmeharaMasakazu YamagishiMitsuhiro Kawano
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2011 Volume 50 Issue 11 Pages 1239-1244


Immunoglobulin G4 (IgG4)-related tubulointerstitial nephritis (TIN) is often accompanied by autoimmune pancreatitis (AIP) or chronic sclerosing dacryoadenitis and sialoadenitis. However, IgG4-related TIN without AIP or lacrimal and/or salivary gland lesions has not been well recognized. Here, we report a case of IgG4-related TIN associated with hepatic inflammatory pseudotumor without AIP or lacrimal and/or salivary gland lesions. A 58-year-old Japanese man with epigastralgia underwent contrast-enhanced computed tomography (CT), which revealed multiple low-density lesions in both kidneys and a low density hepatic mass. Laboratory tests showed an extremely high level of serum IgG4. Percutaneous renal and hepatic biopsies showed diffuse infiltration of lymphocytes and IgG4-positive plasma cells with fibrosis in both tissues. Two months after administration of oral prednisolone, both lesions decreased in size on follow-up CT, and the serum creatinine level also improved. No recurrence has been detected for two years with a maintenance dose of prednisolone.

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© 2011 by The Japanese Society of Internal Medicine
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