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A case of primary antiphospholipid antibody syndrome with acute renal failure showing thrombotic microangiopathy.

Abstract：

:An 18-year-old woman complained of fever and edema and was admitted to Showa University Hospital for treatment of thrombocytopenia and deteriorating renal function. Laboratory studies demonstrated the presence of lupus anticoagulant (LA), prolongation of prothrombin time, hemolytic anemia, a negative Coombs' test, the absence of antinuclear antibodies, and a normal fibrinogen level. Renal biopsy revealed mesangial hypercellularity, severe endocapillary cell damage, and double contour of the basement membrane walls. Immunofluorescence studies demonstrated focal, peripheral, and finely granular deposits for IgG, IgM, and IgA but were negative for fibrinogen. Electron microscopy showed glomerular capillary loops with subendothelial widening and subendothelial deposits, mesangiolysis, mesangial interposition, and marked luminal narrowing. Biopsy findings were consistent with thrombotic microangiopathy. The patient was treated with hemodialysis, methylprednisolone pulse therapy, and dipyridamole. After treatment, LA disappeared, the prothrombin time became normal, and renal function improved. The renal lesions in this patient were caused by primary antiphospholipid antibody syndrome. This case strongly suggests an important causal relationship between LA and renal lesions in thrombotic microangiopathy. We present this case to promote understanding of the pathogenesis of primary antiphospholipid antibody syndrome.

journal_name

Am J Nephrol

journal_title

American journal of nephrology

authors

Sakaguchi S,Kitazawa K,Watanabe M,Mukai K,Totsuka D,Shibata T,Sugisaki T

doi

10.1159/000013526

subject

Has Abstract

pub_date

1999-01-01 00:00:00

pages

594-8

issue

5

eissn

0250-8095

issn

1421-9670

pii

13526

journal_volume

19

pub_type

杂志文章

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