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Kidney non-tumor

Primary glomerular diseases

IgA nephropathy (Bergerís disease)


Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 20 February 2014, 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


Mesangial expansion and loop adhesion on Jones silver stain


Mesangial expansion and global sclerosis on PAS stain


Mesangial expansion and loop adhesion on PAS stain


Mesangial 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


Mesangial deposits

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 (Bergerís disease)

Ref Updated: 5/25/12


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