Kidney non-tumor - IgA nephropathy (Berger’s disease)

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Kidney non-tumor

Primary glomerular diseases

IgA nephropathy (Berger’s disease)

Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 1 July 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● A type of diffuse mesangioproliferative glomerulonephritis (others: Henoch-Schonlein purpura, SLE, IgM nephropathy, resolving stage of postinfectious glomerulonephritis)
● IgA present in mesangium and elevated serum IgA
● Note: thromboangiitis obliterans is also known as Buerger's disease

Pathophysiology
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● Excess amounts of poorly galactosylated serum immunoglobulin IgA1 trigger the generation of glycan-specific IgG and IgA autoantibodies, resulting in circulating IgA immune complexes (Kidney Int 2012;81:833)
● IgA immune complexes are deposited in mesangium and activate alternative complement pathway and possibly the lectin pathway (J Biomed Biotechnol 2012;2012:476739)
● Podocyte injury, due to epithelial to mesenchymal transition, plays an important role in glomerulosclerosis in IgA nephropathy (Cell Physiol Biochem 2012;29:743)

Clinical features
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● Most common form of primary glomerulonephritis worldwide; causes 10% of cases of end stage renal failure in many countries (eMedicine)
● Common at ages 10-29 years, usually males who present with gross or microscopic hematuria after respiratory infection, but no systemic disease (Pediatr Nephrol 2012;27:1293)
● More common in southern Europe, Asia and Native Americans, less common in individuals of African lineage
● Slowly progressive: 25%-50% have renal failure at 20 years; recurs in 20-60% of allografts
● Rarely causes death due to pulmonary hemorrhage (Arch Pathol Lab Med 1994;118:542)
● Note: IgA deposits are also present in Henoch-Schonlein purpura; diseases may be related
● Secondary disease associated with gluten enteropathy (celiac disease), liver disease and dermatitis herpetiformis (Arch Pathol Lab Med 1983;107:324)
● Poor prognosis: prominent arteriolar hyalinization, older age, heavy proteinuria and hypertension

Treatment
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● Controlling proteinuria and blood pressure reduces loss of kidney function (Am J Kidney Dis 2012;59:865)
● Tonsillectomy improved clinicopathologic features in relatively severe pediatric IgA nephropathy, especially with early-surgery (Nephrology (Carlton) 2012 May 23 [Epub ahead of print])
● Steroids (Clin Exp Nephrol 2012 May 23 [Epub ahead of print]); tacrolimus if refractory (Am J Nephrol 2012;35:312)

Case reports
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● 8 year old boy with Wiskott-Aldrich syndrome (Int Urol Nephrol 2012 May 1 [Epub ahead of print])
● 42 year old man with bilateral nodular scleritis (J Ophthalmic Inflamm Infect 2012 Mar 13 [Epub ahead of print])

Micro description
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● Diffuse proliferation of mesangial cells and matrix without significant involvement of capillary walls or lumina
● Mesangial involvement is often uneven and resembles focal and segmental glomerulosclerosis
● Normal or hypercellular glomeruli with diffuse necrotizing crescentic glomerulonephritis

Micro images
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Various images including EM

Mild mesangial expansion

Granular mesangial staining for IgA

Immunofluorescence
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● IgA, often granular IgG and C3 in mesangium
● IgA also in capillaries of dermis, lung, liver and intestine
● IgA more intense or equally intense compared to IgG and IgM
● Staining for C1q should raise the suspicion for lupus nephritis

Electron microscopy description
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● Electron dense deposits in mesangium of all glomeruli

Electron microscopy images
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Electron dense deposits in mesangium

Differential diagnosis
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● Lupus nephritis
● Mesangial IgA deposition associated with obstructive jaundice (Hum Pathol 1987;18:1149)

End of Kidney non-tumor > Primary glomerular diseases > IgA nephropathy (Berger’s disease)

Ref Updated: 5/25/12

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