4.4 Article

Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy

Journal

PEDIATRIC NEPHROLOGY
Volume 26, Issue 6, Pages 979-985

Publisher

SPRINGER
DOI: 10.1007/s00467-011-1787-z

Keywords

Antiphospholipid syndrome; Nephrotic syndrome; Membranous nephropathy; Deep venous thrombosis

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Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by thrombotic events and the presence of circulating antiphospholipid antibodies. Large vessel involvement in the form of thrombosis/stenosis and thrombotic microangiopathy is a commonly described renal finding. However, non-thrombotic glomerulopathies are increasingly being recognized in patients with antiphospholipid syndrome. We report a rare occurrence of both renal vein thrombosis and membranous nephropathy in a previously healthy adolescent male. Investigations revealed persistently positive antiphospholipid antibodies in the absence of an underlying systemic autoimmune disorder or malignancy. Our patient responded favourably to anti-proteinuric therapy and anticoagulation with complete resolution of proteinuria and a nearly occlusive thrombus.

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