Abstract
BackgroundRenal thrombotic microangiopathy (TMA) is occasionally seen in biopsies with pauci-immune necrotizing crescentic glomerulonephritis (PCGN). Recent study indicated that the complement activation is more prominent in the ANCA-negative glomerulonephritis.Case presentationWe report a case of concurrent TMA and PCGN without ANCA positivity. Interestingly, our patient also had biopsy features supportive of Alport syndrome (AS). Genetic studies identified variants and polymorphisms in alternative complement pathway genes that confer substantial risk of developing atypical hemolytic uremic syndrome (aHUS).ConclusionsAbnormal activation in complement pathway may represent a common pathogenic link between these three distinct entities.
Highlights
Renal thrombotic microangiopathy (TMA) is occasionally seen in biopsies with pauci-immune necrotizing crescentic glomerulonephritis (PCGN)
We present a patient with biopsy evidence of thrombotic microangiopathy (TMA), Alport syndrome (AS) and glomerular crescents and discuss potential pathogenic link
The clinical, laboratory and genetic findings indicate a diagnosis of atypical hemolytic uremic syndrome (aHUS) coexistent with glomerular crescents seen on biopsy
Summary
Abnormal activation in complement pathway may represent a common pathogenic link between these three distinct entities.
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