Key Points
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Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent arterial or venous thrombosis and/or pregnancy morbidity and persistently elevated levels of antiphospholipid antibodies
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Renal involvement can be caused by thrombosis at any level within the vasculature of the kidney
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Clinical features include renal artery stenosis, renal infarction, renal vein thrombosis, thrombotic microangiopathy and hypertension
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Treatment of renal involvement in APS is centred on anticoagulation with long-term warfarin
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Patients who are refractory to standard anticoagulation therapy or who have catastrophic APS might benefit from targeted therapy, including the use of intravenous immunoglobulins, rituximab or eculizumab
Abstract
Antiphospholipid syndrome (APS) is an autoimmune disease defined by the presence of arterial or venous thrombotic events and/or pregnancy morbidity in patients who test positive for antiphospholipid antibodies (aPLs). APS can be isolated (known as primary APS) or associated with other autoimmune diseases, such as systemic lupus erythematosus (SLE; known as secondary APS). The kidney is a major target organ in APS and renal thrombosis can occur at any level within the vasculature of the kidney (renal arteries, intrarenal arteries, glomerular capillaries and renal veins); events reflect the site and size of the involved vessels. Histological findings vary widely, including ischaemic glomeruli and thrombotic lesions without glomerular or arterial immune deposits on immunofluorescence. Renal prognosis is affected by the presence of aPLs in patients with lupus nephritis and can be poor. In patients with SLE and aPLs, biopsy should be performed because inflammatory and thrombotic lesions require different therapeutic approaches. Renal involvement in patients with definite APS is treated by anticoagulation with long-term warfarin. The range of renal manifestations associated with APS is broadening and, therefore, aPLs have increasing relevance in end-stage renal disease, transplantation and pregnancy.
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S.S. and D.R. researched data for the article. S.S. and M.J.C. wrote the article and made substantial contribution to discussion of the content. M.K. made substantial contribution to discussion of the content. S.S., M.K. and D.R. reviewed and edited the manuscript before submission.
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Sciascia, S., Cuadrado, M., Khamashta, M. et al. Renal involvement in antiphospholipid syndrome. Nat Rev Nephrol 10, 279–289 (2014). https://doi.org/10.1038/nrneph.2014.38
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