Light-microscopic characteristics of IgG4-related tubulointerstitial nephritis: distinction from non-IgG4-related tubulointerstitial nephritis

Yoshita, Kazuhiro; Kawano, Mitsuhiro; Mizushima, Ichiro; Hara, Satoshi; Ito, Yumi; Imai, Naofumi; Ueno, Mitsuhiro; Nishi, Shinichi; Nomura, Hideki; Narita, Ichiei; Saeki, Takako

doi:10.1093/ndt/gfr761

Cited by 64 publications

(59 citation statements)

References 24 publications

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“…In IgG4-RKD, TIN is the most common feature, and certain common pathologic features are shared between IgG4-TIN and extrarenal organs affected by IgG4-RD. 5,26 However, urinary abnormalities were usually associated with glomerular lesions, and the relationship between glomerular lesions and IgG4-RD has not been elucidated. Membranous glomerulonephritis is the most commonly observed glomerular lesion in IgG4-RKD and is thought to be associated with IgG4-RD.…”

Section: Discussionmentioning

confidence: 99%

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The clinical course of patients with IgG4-related kidney disease

Saeki

Kawano

Mizushima

et al. 2013

Kidney International

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157

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Long-term follow-up for IgG4-related kidney disease, including relapse information, is sparse. To gather data on this we retrospectively examined the clinical course of 43 patients with IgG4-related kidney disease, in which most patients were treated with, and maintained on, corticosteroids. One month after the start of treatment, most of the abnormal serology and radiology parameters had improved. In 34 of the steroid-treated patients whose follow-up period was more than 12 months (median 34 months), excluding one hemodialysis patient, the estimated glomerular filtration rate (eGFR) before treatment was over 60 ml/min in 14 patients (group A) and under 60 ml/min in 20 patients (group B). In group A, there was no difference between the eGFR before therapy and at the last review. In group B, the mean eGFR before treatment (34.1 ml/min) was significantly improved after 1 month (45.0 ml/min), and renal function was maintained at a similar level through last follow-up. Among 24 evaluated patients at the last review, however, renal atrophy had developed in 2 of 9 in group A and in 9 of 15 in group B. Relapse of IgG4-related lesions occurred in 8 of 40 treated patients. Thus, the response of IgG4-related kidney disease to corticosteroids is rapid, not total, and the recovery of renal function persists for a relatively long time under low-dose maintenance. A large-scale prospective study to formulate more useful treatment strategies is necessary.

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Section: Discussionmentioning

confidence: 99%

“…Among the 43 patients, 39 patients had been included in our earlier study (nos. 4,9,15,17,19,22,26,28,29,31,34,38, 39, 41-43 were described in ref. 5).…”

Section: Materials and Methods Patientsmentioning

confidence: 99%

The clinical course of patients with IgG4-related kidney disease

Saeki

Kawano

Mizushima

et al. 2013

Kidney International

Self Cite

157

187

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“…9,29 Eosinophils, which are frequently observed in other aetiologies of TIN, are also characteristic of IgG4-related TIN (Figures 5a and 5b). 29 Whereas in some cases a dense cellular infiltrate with minimal fibrosis is observed, other cases are characterized by prominent expansile interstitial fibrosis. 9 One plausible hypothesis is that these different patterns are determined by the chronicity of the lesion (that is, the fibrotic lesions might result from more prolonged disease), but other undefined variables might also contribute.…”

Section: Laboratory Featuresmentioning

confidence: 99%

IgG4-related disease and the kidney

Cortazar

Stone

2015

Nat Rev Nephrol

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IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition that involves almost every organ system. In this Review, we summarize current knowledge of IgG4-RD and its most frequent manifestations in the kidney—IgG4-related tubulointerstitial nephritis (TIN) and membranous glomerulonephropathy (MGN). Diagnosis of IgG4-RD relies on histopathology: the typical features are a dense lymphoplasmacytic infiltrate and storiform fibrosis. A high percentage of plasma cells observed within lesions stain positively for IgG4. IgG4-related TIN bears the hallmark pathological findings of IgG4-RD; distinctive radiographic characteristics are also frequently observed with use of contrast-enhanced CT. MGN secondary to IgG4-RD seems to be distinct from idiopathic MGN. Humoral and cell-mediated immunity seem to have roles in the pathophysiology of IgG4-RD, but the details of these roles remain unclear. The IgG4 molecule itself is unlikely to be the primary driver of inflammation; rather, it probably downregulates the immune response. Fibrosis might be caused by activation of innate immune cells by polarized CD4(+) T cells. Glucocorticoids are the standard initial treatment for IgG4-RD, but their long-term adverse effects and the high frequency of relapse and renal damage associated with use of this treatment has prompted a search for more effective options. B-cell depletion and the targeting of plasmablasts are both promising approaches.

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“…Otra característica es el aumento del número de eosinofilos y el compromiso de la capsula renal 4 . La presencia en la inmunohistoquímica de numerosas células plasmáticas IgG4 es esencial para el diagnóstico 6 . La presencia de más de 10 células plasmáticas IgG4 por campo mayor y la relación IgG4/IgG > 40 son muy características, pero pueden encontrarse en otros tipos de NTI [6][7][8] .…”

Section: Discussionunclassified

“…La presencia en la inmunohistoquímica de numerosas células plasmáticas IgG4 es esencial para el diagnóstico 6 . La presencia de más de 10 células plasmáticas IgG4 por campo mayor y la relación IgG4/IgG > 40 son muy características, pero pueden encontrarse en otros tipos de NTI [6][7][8] . Para realizar el diagnóstico de la NTI-IgG4 se requiere la combinación de elementos clínicos, serológicos, imagenológicos e histopatológicos 3 .…”

Section: Discussionunclassified