Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis

Saeki, Takako; Nishi, Shinichi; Imai, Naofumi; Ito, Tomoyuki; Yamazaki, Hajime; Kawano, Mitsuhiro; Yamamoto, Motohisa; Takahashi, Hiroki; Matsui, Shoko; Nakada, Shinji; Origuchi, Tomoki; Hirabayashi, Akira; Homma, Noriyuki; Tsubata, Yutaka; Takata, Takuma; Wada, Yoko; Saito, Akihiko; Fukase, Shigeru; Ishioka, Kunihiro; Miyazaki, Kana; Yasukawa, Masaki; Umehara, Hisanori; Sugai, Susumu; Narita, Ichiei

doi:10.1038/ki.2010.271

Cited by 371 publications

(471 citation statements)

References 43 publications

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“…Our case showed multiple poorly contrasted areas in the renal parenchyma and diffuse kidney enlargement. Also, about 30 % of the patients diagnosed with IgG4-related kidney disease show no abnormality on abdominal CT, with the percentage of patients showing abnormalities having been reported to be 72.1 % by Saeki et al [10] and 70.7 % by Kawano et al [5]. However, many patients do not undergo contrast-enhanced CT, and contrast-enhanced CT is considered necessary as long as the renal function tolerates the modality.…”

Section: Discussionmentioning

confidence: 99%

A case of IgG4-related kidney disease complicated by eosinophilic lung disease

et al. 2014

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IgG4-related disease is a systemic chronic inflammatory disorder characterized by a high blood level of IgG4 and the organ injuries by marked infiltration of IgG4-positive plasma cells and fibrosis. A 71-year-old male was hospitalized for a cough, malaise and anorexia. IgG4-related disease was suspected due to marked elevation of the serum IgG4 level. However, on lung biopsy, only eosinophil infiltration was demonstrated with no plasma cell infiltration. Otherwise abdominal contrastenhanced CT showed mild enlargement of the bilateral kidneys and many differed contrasted areas and FDG PET-CT. Moreover, renal biopsy specimens showed typical tubulointerstitial nephritis with a large number of IgG4-positive plasma cells infiltration (the IgG4/IgG-positive cell rate, 89 %) and fibrosis. We diagnosed this patient as typical IgG4-related kidney disease. He was treated by the moderate dose of prednisolone (0.8 mg/kg/day) alone, and showed prompt response in the clinical condition, and both the lung and kidney lesions. In this case, it was useful for diagnosis of IgG4-related diseases to evaluate an image such as abdominal contrast-enhanced CT and FDG PET-CT. Our case might be one of the possible patterns of IgG4-related lung diseases. In addition, we thought that there might be an association between hypereosinophilia and IgG4-related kidney disease.

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Section: Discussionmentioning

confidence: 99%

A case of IgG4-related kidney disease complicated by eosinophilic lung disease

et al. 2014

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“…There is a high probability of relapse if steroids are tapered off too quickly (31,32). For some critically ill patients, pulse corticosteroid therapy followed by oral steroid treatment is recommended (1,11,22). No reports have so far been published regarding the efficacy of combined therapy with steroids and other immunosuppressants.…”

Section: Discussionmentioning

confidence: 99%

“…Additionally, IgG4-related TIN may have concurrent glomerular diseases. Membranous nephropathy (MN), another IgG4-dominant disease (10,(23)(24)(25)) is most commonly observed in patients with IgG4-related TIN. In Case 1, the patient's daughter had been diagnosed with idiopathic membranous nephropathy.…”

Section: Discussionmentioning

confidence: 99%

“…In some cases, TIN is secondary to autoimmune diseases such as Sjögren's syndrome and systemic lupus erythematosus. A novel disease, named IgG4-related systemic disease (IgG4-RSD), was recently reported to be a cause of TIN (1,2). Although an accumulating number of cases of IgG4-related TIN have been reported in the published literature, few such cases have so far been reported in China.…”

Section: Introductionmentioning

confidence: 94%

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IgG4-Associated Tubulointerstitial Nephritis: Two Case Reports and a Literature Review

et al. 2012

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IgG4-related systemic disease (IgG4-RSD) is an autoimmune disease that includes a wide variety of lesions. IgG4-RSD is characterized by high levels of serum IgG4, abundant levels of IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. Tubulointerstitial nephritis (TIN) is a major finding when the kidneys are involved and is effectively treated with corticosteroid therapy. We herein describe two cases of IgG4-related TIN. Such cases have rarely been reported in China.

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“…IgG4-related disease represents a recently recognized group of multiorgan diseases characterized by hypergammaglobulinemia with elevated serum total IgG and/or IgG4 levels, a high level of serum IgG4, and dense infiltration of IgG4-positive cells into multiple organs (39,40). The term IgG4-related sclerosing disease is also used for this entity because it results in a sclerosing lesion of multiple organs, including the kidney, with formation of pseudotumors (41,42).…”

Section: Discussion Of Questionmentioning

confidence: 99%

American Society of Nephrology Quiz and Questionnaire 2012

Fervenza

Glassock

Bleyer

2013

Clinical Journal of the American Society of Nephrology

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SummaryPresentation of the Nephrology Quiz and Questionnaire (NQQ) has become an annual tradition at the meetings of the American Society of Nephrology. It is a very popular session, judged by consistently large attendance. Members of the audience test their knowledge and judgment on a series of case-oriented questions prepared and discussed by experts. They can also compare their answers in real time, using audience response devices, to those of program directors of nephrology training programs in the United States, acquired through an Internet-based questionnaire. The topic presented here is GN. Cases representing this category, along with single best answer questions, were prepared by a panel of experts (Drs. Fervenza, Glassock, and Bleyer). The correct and incorrect answers were then briefly discussed after the audience responses and the results of the questionnaire were displayed. This article recapitulates the session and reproduces its educational value for a larger audience-that of the readers of the Clinical Journal of the American Society of Nephrology. Have fun.Clin J Am Soc Nephrol 8: 1460-1465, 2013. doi: 10.2215/CJN.00440113 GN Case 1A 20-year-old woman was referred for evaluation of persistent hematuria and proteinuria. She had a history of fever and sore throat lasting 1 week that occurred 6 months earlier and were associated with abdominal pain and dark urine. She did not receive any antimicrobial agents. Significant findings on physical examination at that time included a BP of 140/90 mmHg and 2+ edema. Laboratory evaluation showed a serum creatinine value of 1.4 mg/dl, urinalysis with 2+ protein and 3+ blood, and a 24-hour urinary protein excretion of 680 mg. The C3 level was 46 mg/dl (reference range, 75-175 mg/dl) and the C4 level was 23 mg/dl (reference range, 14-40 mg/dl). A throat culture was negative for b-hemolytic streptococci, and an antistreptolysin O titer was 200 IU/ml (upper limit of normal, 200 IU/ml). The serum albumin concentration was 3.6 g/dl.A renal biopsy was performed and showed a pattern of membranoproliferative GN on light microscopy. No crescents were observed. Immunofluorescence microscopy showed bright (3+) mesangial and capillary wall C3 staining. Electron microscopy showed mesangial, intramembranous, and subendothelial deposits, as well as a few subepithelial hump-like deposits. Postinfectious GN and type III membranoproliferative GN were diagnosed. The patient was treated symptomatically, but proteinuria and hematuria persisted. Current evaluation shows a hemoglobin level of 11.8 g/dl, a serum creatinine concentration of 1.3 mg/dl, urinalysis with 3+ blood and 3+ protein, quantitative proteinuria of 2.2 g/24 hours, C3 concentration of 44 mg/dl, and C4 concentration of 22 mg/dl. Question 1 (see Figure 1 for responses of program directors and attendees)To further evaluate this patient, you would now order:A. Antinuclear antibodies B. Hepatitis B and C serology C. Anti-deoxyribonuclease (DNase) antibody titers D. Serum C1q level E. Antibodies to complement-regulating ...

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Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis

Cited by 371 publications

References 43 publications

A case of IgG4-related kidney disease complicated by eosinophilic lung disease

A case of IgG4-related kidney disease complicated by eosinophilic lung disease

IgG4-Associated Tubulointerstitial Nephritis: Two Case Reports and a Literature Review

American Society of Nephrology Quiz and Questionnaire 2012

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