Renal involvement in antiphospholipid syndrome

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 (aPL). 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 aPL in patients with lupus nephritis and can be poor. In patients with SLE and aPL, 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, aPL has increasing relevance in end-stage renal disease, transplantation and pregnancy.

Obstetric antiphospholipid syndrome: early variations of angiogenic factors are associated with adverse outcomes

“…The range of renal manifestations associated with APS has been broadened. 21 Renal function and the sFlt1 renal loss may thus be a strong modulating factor impacting on the negative prognostic value of circulating sFlt1. On the other hand, PlGF is a much smaller protein (≈30 kDa) which is readily filtered; however, decreased urinary PlGF at mid gestation is strongly associated with subsequent early development of preeclampsia.…”

The antiphospholipid syndrome: from pathophysiology to treatment

“…Furthermore, in patients with APS and SLE, APS renal disease may coexist with immune complex-mediated lupus nephritis. In these patients, biopsy should be performed in order to distinguish inflammatory and thrombotic lesions since the two conditions require different treatments, i.e., anticoagulation versus immunosuppression, respectively [92,93]. From a clinical point of view, the most frequent manifestations of renal involvement in APS are hypertension (often severe), proteinuria (from mild levels to nephrotic range), hematuria, and renal insufficiency [90,91].…”

Acute Renal Infarction Pathogenesis and Atrial Fibrillation: Case Report and Literature Review

“…APS can cause both arterial and venous thrombotic events and while these events mostly involve lower limb deep veins and cerebral arteries, the renal circulation can also be targeted. Renal infarction can be a sequela of the above as well as renal artery thrombosis or emboli from cardiac or other arterial sources [19]. The rarity of antithrombin III deficiency as a causative factor of ARI is demonstrated by the fact that there is only a single reported case in the literature of a 47-year-old male with antithrombin III deficiency and heterozygosity for prothrombin (G20210A) gene mutation who presented with bilateral renal infarcts [20].…”