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Kidney non-tumor

Associated with systemic conditions

Henoch-Schonlein purpura


Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 20 September 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.
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General
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● Purpuric skin lesions on extensor arms and legs and buttocks
● Also abdominal pain, vomiting, GI bleeding, arthralgias, hematuria, proteinuria and nephrotic syndrome
● Due to systemic small vessel leukocytoclastic vasculitis
● Most common systemic vasculitis in children
● Also called anaphylactoid purpura

Clinical features
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● Significant clinicopathological differences with IgA nephropathy (Zhongguo Dang Dai Er Ke Za Zhi 2012;14:506
● Renal symptoms in 30-70%; some adults develop rapidly progressive glomerulonephritis
● 70% are ages 2-11 years; rare in adults or infants 1 year or less
● Higher rate of renal involvement in children ages 10-18 (Iran J Kidney Dis 2012;6:269)
● Associated with atopy in 1/3; may follow respiratory infection
● Related to IgA nephropathy, due to elevated serum IgA, circulating immune complexes with IgA, similar kidney lesions (Arch Pathol Lab Med 1982;106:192), high serum galactose-deficient immunoglobulin A1 levels (Kidney Int 2011;80:79)
● Hypertension, serum creatinine, proteinuria, cellular crescents, glomerular necrotizing lesions and chronic renal lesions are associated with renal failure (Mod Pathol 2001;14:635)
● Variable recurrence rates (12-69%) after renal transplant, but usually not clinically important (Transplantation 2011;92:907, Clin J Am Soc Nephrol 2011;6:1768, Clin J Am Soc Nephrol 2011;6:2034)
Prognosis: excellent in children (50% have spontaneous remission); poorer in adults (Clin Nephrol 2011;76:49) or with nephrotic syndrome; often difficult to predict (Clin J Am Soc Nephrol 2011;6:679)

Case reports
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● 15 year old girl with prior onset of IgA nephropathy (Fukushima J Med Sci 2010;56:157)
● 72 year old man with coexisting IgG4-related tubulointerstitial nephritis (Allergy Asthma Clin Immunol 2011;7:5)
● 75 year old man with rectal bleeding and acute renal injury (J Med Case Rep 2011;5:364)

Treatment
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● Recommended to administer intensive therapy initially (Indian J Pediatr 2012;79:207)
● Corticosteroids, cytotoxic drugs
● Mycophenolate mofetil for children with nephrotic-range proteinuria (Pediatr Nephrol 2012;27:765)

Micro description
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Acute:
● Leukocytoclastic vasculitis of small vessels due to deposition of IgA immune complexes
● Diffuse proliferation of mesangial cells and matrix without significant involvement of capillary walls or lumina
● Also segmental necrotizing lesions (50%), endocapillary proliferation (13%), cellular crescents, glomerular acute and chronic inflammatory infiltrate

Chronic:
● Glomerular sclerosis, tubular loss, interstitial fibrosis and hyaline arteriolosclerosis

Skin:
● Hemorrhage and necrotizing vasculitis in dermal small vessels, which contain IgA
● Vasculitis is present in other organs but usually NOT kidney

Micro images
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Various images

               
Silver stain

Immunofluorescence
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● IgA deposition in mesangium, resembling IgA nephropathy
● Variable IgG, IgM, C3 and properdin

Immunofluorescence images
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Henoch-Schönlein purpura nephritis

Electron microscopy description
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● Mesangial deposits, may extend into subendothelial areas
● May have subepithelial deposits

End of Kidney non-tumor > Associated with systemic conditions > Henoch-Schonlein purpura

Ref Updated: 7/26/12


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