Abstract
Kidney damage is a well-recognized complication of the antiphospholipid syndrome (APS), either primary or systemic lupus erythematosus (SLE)-associated APS. Kidney involvement in APS involves a variety of manifestations, such as renal artery thrombosis or stenosis, renal vein thrombosis, allograft loss due to thrombosis after kidney transplantation, and injury to the renal microvasculature, also known as APS nephropathy. Biopsy in patients with APS nephropathy includes acute thrombotic microangiopathy lesions and chronic intrarenal vascular lesions such as interlobular fibrous intimal hyperplasia, arterial and arteriolar recanalizing thrombosis, fibrous arterial occlusion, and focal cortical atrophy. The most frequent clinical features are hypertension, microscopic hematuria, proteinuria (ranging from mild to nephritic levels), and renal insufficiency. It is uncertain whether antiphospholipid antibodies or other factors are implicated in the development of APS nephropathy, and whether it is driven mainly by thrombotic or by inflammatory processes. Experimental models and clinical studies of thrombotic microangiopathy lesions implicate activation of the complement cascade, tissue factor, and the mTORC pathway. Currently, the management of APS nephropathy relies on expert opinion, and consensus is lacking. There is limited evidence about the effect of anticoagulants, and their use remains controversial. Treatment approaches in patients with APS nephropathy lesions may include the use of heparin based on its role on complement activation pathway inhibition or the use of intravenous immunoglobulin and/or plasma exchange. Targeted therapies may also be considered based on potential APS nephropathy pathogenetic mechanisms such as B-cell directed therapies, complement inhibition, tissue factor inhibition, mTOR pathway inhibition, or anti-interferon antibodies, but prospective multicenter studies are needed to address their role.
Highlights
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by thrombotic episodes in the arterial or venous circulation, in the presence of antiphospholipid antibodies, namely lupus anticoagulant (LA), anticardiolipin antibodies, and anti-β2glycoprotein-I antibodies [1]
Natejumnong et al showed that patients with systemic lupus erythematosus (SLE) nephritis and LA positivity had higher systolic blood pressure (133.7 versus 121.9 mmHg, p = 0.005), serum creatinine (233.0 versus 94.9 μmol/L), and 24-h urine protein excretion (2.6 versus 1.4 g, p = 0.02), features associated with worse renal prognosis [6]
A very recent study of 446 kidney transplant recipients showed that the risk of GFR decline within the first year post-transplant was elevated in patients with positive aPL, even without thrombotic events prior to transplantation [20]
Summary
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by thrombotic episodes in the arterial or venous circulation, in the presence of antiphospholipid antibodies (aPL), namely lupus anticoagulant (LA), anticardiolipin antibodies, and anti-β2glycoprotein-I antibodies (anti-β2GPI) [1]. APS can be either primary or secondary when it occurs in the context of other underlying autoimmune disorder, mainly systemic lupus erythematosus (SLE). APL positivity may occur in 0–5% of healthy individuals and in approximately 30–40% of patients with SLE; one third of SLE patients with positive aPL develop thrombosis during their follow-up APS can be either primary or secondary when it occurs in the context of other underlying autoimmune disorder, mainly systemic lupus erythematosus (SLE). aPL positivity may occur in 0–5% of healthy individuals and in approximately 30–40% of patients with SLE; one third of SLE patients with positive aPL develop thrombosis during their follow-up
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