Kidney non-tumor - Systemic sclerosis

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Kidney non-tumor

Associated with systemic conditions

Systemic sclerosis

Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 21 December 2012, last major update December 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Connective tissue disorder of unknown etiology with multiple organ system involvement, symptoms due to excessive collagen deposition and vascular disease
● Also called scleroderma

Clinical features
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● 75% women, usually ages 30+, rare in children
● Symptoms: digital pulp atrophy, esophageal dysmotility, malabsorption, musculoskeletal involvement, Raynaud’s syndrome, skin thickening, telangiectasia
● Death due to pulmonary hypertension, interstitial fibrosis and cardiomyopathy

Sclerodermal renal disease
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● 60% have renal involvement, either (a) renal crisis / acute and rapidly progressive form of renal failure associated with malignant hypertension, systemic vasoconstriction, microangiopathic hemolytic anemia; or (b) slowly progressive chronic form with proteinuria, hypertension and azotemia
● Scleroderma renal crisis: malignant hypertension and oligo-anuric acute renal failure; occurs in 2-5%, particularly those with diffuse cutaneous systemic sclerosis in initial years of disease evolution (Nephrol Ther 2011;7:192)
● Risk factors for renal crisis: increased serum sCD147 levels (Clin Rheumatol 2012;31:835), RNA polymerase III antibodies (Scand J Rheumatol 2012;41:39), use of glucocorticoids (Clin Rev Allergy Immunol 2011;40:84)
● Increased risk of acute kidney injury with hematopoietic cell transplantation (Biol Blood Marrow Transplant 2011;17:674)

Gross images
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Finely granular, petechial hemorrhages beneath the capsule

Cortical hemorrhages

Micro description
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● Intimal thickening by loose myxoid fibrous tissue in interlobular arteries
● Subendothelial fibrin deposition and intimal hemorrhage
● Fibrinoid necrosis and thrombosis in arterioles
● Acute ischemic changes in glomeruli

Micro images
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Various images

Sclerodermal renal crisis

Medium sized renal cortical artery in patient with diffuse systemic sclerosis and renal crisis; note severe concentric, edematous myxoid intimal proliferation with almost total obliteration of lumen


Fig 16.25-mucoid intimal hyperplasia with prominent luminal narrowing; fig 16.26- small interlobular artery shows luminal narrowing, little collagen deposition


Fig 16.27-extensive (circumferential) fibrin insudation; fig 16.29-subendothelial fibrin deposition

Fig 16.30-significant chronicity, with extensive tubulointerstitial fibrosis, ischemic glomeruli and intimal fibroplasia of an interlobular size artery

Fibrinoid necrosis

Immunofluorescence
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● Fibrinogen, variable IgM and C3

Electron microscopy images
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Wide electron-lucent zone on the endothelial side of the capillary basement membrane is seen, with mesangial cell interposition

Differential diagnosis
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● Malignant hypertension
● HUS-TTP: different clinically, but similar renal lesions

End of Kidney non-tumor > Associated with systemic conditions > Systemic sclerosis

Ref Updated: 8/10/12

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