Kidney Biopsy Teaching Case
IgG4-Related Tubulointerstitial Nephritis With Plasma Cell–Rich Renal Arteritis

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Immunoglobulin G4 (IgG4)-related tubulointerstitial nephritis is a newly recognized clinicopathologic entity that may occur as an isolated renal lesion or as part of a multisystem disorder. It is characterized by plasma cell–rich interstitial nephritis with abundant IgG4-positive plasma cells and IgG-dominant tubulointerstitial immune deposits. We report the first case of IgG4-related tubulointerstitial nephritis with multifocal plasma cell–rich renal arteritis presenting as acute kidney injury in a 72-year-old man. Seven weeks of prednisone therapy led to nearly complete recovery of kidney function. This case enlarges the morphologic spectrum of this disorder and emphasizes the need to distinguish it from other causes of renal vasculitis.

Introduction

Immunoglobulin G4 (IgG4)-related tubulointerstitial nephritis was first recognized as a distinct clinicopathologic entity in 2004.1, 2 Kidney involvement can occur in isolation or as part of a multisystem disorder known as IgG4-related systemic disease. Approximately 80%-95% of patients have extrarenal involvement.3, 4

IgG4-related disease can involve the pancreas, bile duct, gall bladder, gastrointestinal tract, liver, kidney, ureter, prostate, orbit, pituitary gland, lacrimal gland, salivary gland, thyroid, lung, lymph nodes, and breast.3, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15 Presenting clinical features vary depending on the severity and distribution of organ involvement. IgG4-related disease is more common in men and the elderly.

Kidney involvement usually manifests as acute, subacute, or chronic glomerular filtration rate (GFR) loss and minimal proteinuria (protein excretion <0.5 g/d).3, 15 Rarely, nephrotic syndrome may occur in cases with associated glomerulopathy such as membranous nephropathy.3, 15 Total serum IgG and IgG4 levels usually are increased. Hypocomplementemia has been reported in 17%-36% of cases.15 Antinuclear antibodies, rheumatoid factor, elevated IgE levels, and peripheral eosinophilia may occur.15 On radiologic examination, kidney involvement varies from unilateral to bilateral, with unifocal, multifocal, or diffuse distributions. Localized kidney masses often are diagnosed by surgical resection for pseudotumor; however, greater clinical awareness of this entity has led to increasing use of kidney biopsy.3, 15

The histopathology of IgG4-related disease in various organs is remarkably similar, including lymphoplasmacytic infiltrates, increased numbers of IgG4-positive plasma cells, storiform fibrosis, and variable eosinophils. Vascular involvement is a rare feature and usually occurs as obliterative phlebitis.16 Here, we describe the first case of obliterative renal arteritis in IgG4-related tubulointerstitial nephritis mimicking vasculitis.

Section snippets

Clinical History

A 72-year-old white man was admitted to the hospital with a 4-week history of fever, anorexia, diarrhea, and 25-lb weight loss. Medical history included type 2 diabetes mellitus for 19 years, hypertension, and chronic atrial fibrillation. He had a history of chronic kidney disease with baseline serum creatinine level of 1.5 mg/dL (estimated GFR [eGFR], 46 mL/min/1.73 m2 using the IDMS traceable 4-variable MDRD [Modification of Diet in Renal Disease] Study equation and microalbuminuria with

Discussion

In the recent report of 35 patients with IgG4-related tubulointerstitial nephritis by Raissian et al,3 average participant age was 65 (range, 20-81) years, 86% were men, and 71% were white. Thus, the 72-year-old white man in our case fits the typical demographic profile.3 The proposed diagnostic criteria3 require more than 10 IgG4-positive plasma cells per 40× magnification high-power field in the most concentrated area, on a background of plasma cell–rich interstitial nephritis. A supporting

Acknowledgements

Support: None.

Financial Disclosure: The authors declare that they have no relevant financial interests.

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    Originally published online December 3, 2012.

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