Kidney nontumor
Associated with systemic conditions
Systemic sclerosis

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 6 February 2018, last major update December 2012

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Systemic sclerosis [title] kidney

Cite this page: Sangle, N. Systemic sclerosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneysystemicsclerosis.html. Accessed February 25th, 2018.
Definition / general
  • 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
  • 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
  • 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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Cortical hemorrhages

Microscopic (histologic) description
  • 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
Microscopic (histologic) images

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Sclerodermal renal crisis

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Fibrinoid necrosis


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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
Immunofluorescence
  • Fibrinogen, variable IgM and C3
Differential diagnosis