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Currently filtering by tag: scleroderma renal crisis

Art of Medicine: Thrombotic Microangiopathy

Thrombotic Microangiopathy
The above painting shows a glomerulus containing capillary loop fibrin thrombi, an arteriole with onion-skinning, and acute tubular injury, findings that can be seen in thrombotic microangiopathy.  Morphologic findings of thrombotic microangiopathy that can be seen on a renal biopsy include arteriolar or capillary loop fibrin or platelet thrombi, red blood cell fragmentation within glomerular capillary loops or within arteries, mesangiolysis, endothelial cell swelling,  glomerular basement membrane duplication, mucoid intimal edema of arteries, and a myointimal proliferation surrounding arterioles (onion-skinning like reaction). Thrombotic microangiopathy (TMA) has a wide clinical differential diagnosis, which can have substantial morphologic overlap on a kidney...


Acute kidney injury, anti-centromere antibodies, ischemia, schistoctes, scleroderma renal crisis, thrombotic microangiopathy, scleroderma
The patient is a 32-year-old African American female who presents with hypertensive urgency, positive ANA, positive anti-RNA polymerase III antibody, schistocytes on the peripheral smear, forgetfulness, and a creatinine of 2. On physical examination, the skin is tight and thickened along the face ("masked face"), back, and proximal arms. Urinalysis shows 250 mg of proteinuria and no active urine sediment. Serologies for c-ANCA, p-ANCA, dsDNA, and anti-GBM are negative. The patient has scleroderma renal crisis. Figure 1 shows an ischemic glomerulus. Figure 2 shows moderate interstitial fibrosis. From low power, the intimal edema and intimal proliferation within the arteries is...

Diagnose This (August 27, 2018)

What is this finding and what is the likely etiology?   ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​    ...