Kidney Biopsy Teaching CaseIgG4-Related Tubulointerstitial Nephritis With Plasma Cell–Rich Renal Arteritis
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.
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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.
References (33)
- et al.
IgG4-related periureteral fibrosis presenting as a unilateral ureteral mass
Pathol Res Pract
(2011) - et al.
IgG4-related sclerosing cholangitis
Ann Hepatol
(2011) - et al.
Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis
Kidney Int
(2010) - et al.
Idiopathic hypocomplementemic interstitial nephritis with extensive tubulointerstitial deposits
Am J Kidney Dis
(2001) - et al.
Membranous nephropathy associated with IgG4-related disease
Am J Kidney Dis
(2011) - et al.
IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung
Hum Pathol
(2005) - et al.
Renal sarcoidosis presenting as acute kidney injury with granulomatous interstitial nephritis and vasculitis
Am J Kidney Dis
(2012) - et al.
Acute tubulointerstitial nephritis associated with autoimmune-related pancreatitis
Am J Kidney Dis
(2004) - et al.
IgG4-associated idiopathic tubulointerstitial nephritis complicating autoimmune pancreatitis
Nephrol Dial Transplant
(2004) - et al.
Diagnosis of IgG4-related tubulointerstitial nephritis
J Am Soc Nephrol
(2011)
Clinicopathological findings of immunoglobulin G4-related kidney disease
Clin Exp Nephrol
Inflammatory pseudotumors mimicking intrahepatic cholangiocarcinoma of the liver; IgG4-positivity and its clinical significance
J Hepatobiliary Pancreat Sci
A unique case of IgG4 sclerosing dacryocystitis
Ophthal Plast Reconstr Surg
Comparison of plasma cell type of Castleman's disease and IgG4-related sclerosing disease: a histopathological and immunohistochemical study
Pathobiology
Mass-forming autoimmune pancreatitis caused after resection of an IgG4-related renal pelvic lesion
Nihon Shokakibyo Gakkai Zasshi
Diagnostic criteria for IgG4-related sclerosing cholangitis based on cholangiographic classification
J Gastroenterol
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Originally published online December 3, 2012.