Sneddon's syndrome, anti-cardiolipin antibody and glomerular thrombosis.
Sneddon's syndrome, cerebrovascular thrombosis and livedo reticularis, is often a variant of the "primary" anti-phospholipid syndrome (PAPS). We report a woman with PAPS, presenting as Sneddon's syndrome, with renal impairment and glomerular thrombosis on renal biopsy. An IgG anti-cardiolipin antibody (aCL) was identified. The aCL was purified by affinity chromatography, gel filtration chromatography and ion-exchange chromatography, assayed in a modified ELISA and found to be of the type that requires the plasma protein beta 2-GPI to bind aCL. As beta 2-GPI has anticoagulant properties it is postulated that its interaction with aCL has a pathogenic role in the thrombotic lesions associated with aCL.
Stevens
J M
JM
Renal Unit, Queen Elizabeth Hospital, Woodville, South Australia.
Hunt
J E
JE
Seymour
A E
AE
Krilis
S A
SA
Pugsley
D J
DJ
D000328:Adult; D017153:Antibodies, Anticardiolipin / Q000032:analysis*; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D002542:Intracranial Embolism and Thrombosis / Q000209:etiology* / Q000276:immunology* / Q000473:pathology; D007674:Kidney Diseases / Q000209:etiology*; D007678:Kidney Glomerulus; D010859:Pigmentation Disorders / Q000209:etiology; D017445:Skin Diseases, Vascular / Q000209:etiology* / Q000276:immunology* / Q000473:pathology; D013577:Syndrome; D013927:Thrombosis / Q000209:etiology*
D017153:Antibodies, Anticardiolipin; D007074:Immunoglobulin G