Diagnosis of IgG4-related tubulointerstitial nephritis

Yassaman Raissian, Samih H. Nasr, Christopher P. Larsen, Robert B. Colvin, Thomas Christopher Smyrk, Naoki Takahashi, Ami Bhalodia, Aliyah R. Sohani, Lizhi Zhang, Suresh T Chari, Sanjeev M Sethi, Mary E. Fidler, Lynn D. Cornell

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Abstract

IgG4-related systemic disease is an autoimmune disease that was first recognized in the pancreas but also affects other organs. This disease may manifest as tubulointerstitial nephritis (IgG4-TIN), but its clinicopathologic features in the kidney are not well described. Of the 35 patients with IgG4-TIN whose renal tissue specimens we examined, 27 (77%) had acute or progressive chronic renal failure, 29 (83%) had involvement of other organ systems, and 18 of 23 (78%) had radiographic abnormalities. Elevated total IgG or IgG4 serum levels were present in 79%. All pathologic specimens featured plasma cell - rich TIN, with most showing diffuse, expansile interstitial fibrosis. Immune complexes along the tubular basement membranes were present in 25 of 30 (83%). All specimens had a moderate to marked increase in IgG4+ plasma cells by immunohistochemistry. We used a control group of 175 pathologic specimens with plasma cell - rich interstitial infiltrates that can mimic IgG4-TIN to examine the diagnostic utility of IgG4 immunostaining. Excluding pauci-immune necrotizing and crescentic glomerulonephritis, IgG4 immunohistochemistry had a sensitivity of 100% (95% CI 90-100%) and a specificity of 92% (95% CI 86-95%) for IgG4-TIN. Of the 19 patients with renal failure for whom treatment and follow-up data were available, 17 (89%) responded to prednisone. In summary, because no single test definitively diagnoses IgG4-related systemic disease, we rely on a combination of histologic, immunophenotypic, clinical, radiographic, and laboratory features. When the disease manifests in the kidney, our data support diagnostic criteria that can distinguish IgG4-TIN from other types of TIN.

Original languageEnglish (US)
Pages (from-to)1343-1352
Number of pages10
JournalJournal of the American Society of Nephrology
Volume22
Issue number7
DOIs
StatePublished - Jul 2011

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Interstitial Nephritis
Immunoglobulin G
Plasma Cells
Kidney
Immunohistochemistry
Glomerulonephritis
Prednisone
Antigen-Antibody Complex
Basement Membrane
Autoimmune Diseases
Chronic Kidney Failure
Renal Insufficiency
Pancreas
Fibrosis

ASJC Scopus subject areas

  • Nephrology

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Raissian, Y., Nasr, S. H., Larsen, C. P., Colvin, R. B., Smyrk, T. C., Takahashi, N., ... Cornell, L. D. (2011). Diagnosis of IgG4-related tubulointerstitial nephritis. Journal of the American Society of Nephrology, 22(7), 1343-1352. https://doi.org/10.1681/ASN.2011010062

Diagnosis of IgG4-related tubulointerstitial nephritis. / Raissian, Yassaman; Nasr, Samih H.; Larsen, Christopher P.; Colvin, Robert B.; Smyrk, Thomas Christopher; Takahashi, Naoki; Bhalodia, Ami; Sohani, Aliyah R.; Zhang, Lizhi; Chari, Suresh T; Sethi, Sanjeev M; Fidler, Mary E.; Cornell, Lynn D.

In: Journal of the American Society of Nephrology, Vol. 22, No. 7, 07.2011, p. 1343-1352.

Research output: Contribution to journalArticle

Raissian, Y, Nasr, SH, Larsen, CP, Colvin, RB, Smyrk, TC, Takahashi, N, Bhalodia, A, Sohani, AR, Zhang, L, Chari, ST, Sethi, SM, Fidler, ME & Cornell, LD 2011, 'Diagnosis of IgG4-related tubulointerstitial nephritis', Journal of the American Society of Nephrology, vol. 22, no. 7, pp. 1343-1352. https://doi.org/10.1681/ASN.2011010062
Raissian Y, Nasr SH, Larsen CP, Colvin RB, Smyrk TC, Takahashi N et al. Diagnosis of IgG4-related tubulointerstitial nephritis. Journal of the American Society of Nephrology. 2011 Jul;22(7):1343-1352. https://doi.org/10.1681/ASN.2011010062
Raissian, Yassaman ; Nasr, Samih H. ; Larsen, Christopher P. ; Colvin, Robert B. ; Smyrk, Thomas Christopher ; Takahashi, Naoki ; Bhalodia, Ami ; Sohani, Aliyah R. ; Zhang, Lizhi ; Chari, Suresh T ; Sethi, Sanjeev M ; Fidler, Mary E. ; Cornell, Lynn D. / Diagnosis of IgG4-related tubulointerstitial nephritis. In: Journal of the American Society of Nephrology. 2011 ; Vol. 22, No. 7. pp. 1343-1352.
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abstract = "IgG4-related systemic disease is an autoimmune disease that was first recognized in the pancreas but also affects other organs. This disease may manifest as tubulointerstitial nephritis (IgG4-TIN), but its clinicopathologic features in the kidney are not well described. Of the 35 patients with IgG4-TIN whose renal tissue specimens we examined, 27 (77{\%}) had acute or progressive chronic renal failure, 29 (83{\%}) had involvement of other organ systems, and 18 of 23 (78{\%}) had radiographic abnormalities. Elevated total IgG or IgG4 serum levels were present in 79{\%}. All pathologic specimens featured plasma cell - rich TIN, with most showing diffuse, expansile interstitial fibrosis. Immune complexes along the tubular basement membranes were present in 25 of 30 (83{\%}). All specimens had a moderate to marked increase in IgG4+ plasma cells by immunohistochemistry. We used a control group of 175 pathologic specimens with plasma cell - rich interstitial infiltrates that can mimic IgG4-TIN to examine the diagnostic utility of IgG4 immunostaining. Excluding pauci-immune necrotizing and crescentic glomerulonephritis, IgG4 immunohistochemistry had a sensitivity of 100{\%} (95{\%} CI 90-100{\%}) and a specificity of 92{\%} (95{\%} CI 86-95{\%}) for IgG4-TIN. Of the 19 patients with renal failure for whom treatment and follow-up data were available, 17 (89{\%}) responded to prednisone. In summary, because no single test definitively diagnoses IgG4-related systemic disease, we rely on a combination of histologic, immunophenotypic, clinical, radiographic, and laboratory features. When the disease manifests in the kidney, our data support diagnostic criteria that can distinguish IgG4-TIN from other types of TIN.",
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AU - Takahashi, Naoki

AU - Bhalodia, Ami

AU - Sohani, Aliyah R.

AU - Zhang, Lizhi

AU - Chari, Suresh T

AU - Sethi, Sanjeev M

AU - Fidler, Mary E.

AU - Cornell, Lynn D.

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