It is speculated that it may be an autoimmune disorder whereby immunologic mechanisms lead to fibrosis, possibly by cytokines that activate fibroblasts or inflict damage to small blood vessels.,
Scleroderma is usually diagnosed between ages 30 and 50.
Female to male ratio of 3:1.
|General Gross Description|
The acutely affected kidney may not show much in the way of gross pathologic features. An occasional infarct may be discerned.
The healed or chronically affected kidney shows reduction of cortical mass and fine cortical surface scarring similar to that of nephrosclerosis.
|General Microscopic Description|
The histology of scleroderma in the acute phase is that of a thrombotic microangiopathy.
The structures that are most involved are the interlobular arteries.
Interlobular arteries show intimal thickening that is loose, myxoid and edematous. Fibroblasts and red cell fragments may be present in the thickened intima. Fibrin thrombi may occlude lumens.
Chronic or healed arterial lesions show concentric intimal fibroelastosis.
Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 210-213.
Renal Disease, 2nd edition, Churg et al. (eds). New York: Igaku-Shoin, 1985, pp.293-294.
This link will directly take you to the relevant new literature
||Synopsis by: Harold Yamase M.D. (T71000M52000)