Kidney Disease in Antiphospholipid Syndrome
Section snippets
Systemic hypertension
Hypertension (HT) is a common feature of the APS both in the primary and the secondary forms [1], [2], [3], [4]. It appears to be a marker of nephropathy as it has been consistently present in the majority of patients with APS and renal involvement. It is important to keep in mind that both microvasculopathy or occlusion of the renal artery can contribute to severe HT in APS [1], [5]. The prevalence of HT in 600 patients with primary APS (n = 68), secondary APS (n = 522), or positive for aPL (n
Renal artery lesions
Renal artery occlusion/stenosis has been reported in patients with aPL, both in the context of rheumatic conditions, mainly SLE, or in the primary APS. Early reports described unilateral or bilateral renal artery stenosis (RAS) or thrombosis with renal cortical ischemia or renal infarction [7], [8], [9], [10]. Of particular interest are the communications by Rossi and colleagues [11] and Mandreoli and colleagues [12], [13] regarding cases of renovascular HT with renal artery stenosis, which
Intrarenal vascular lesions—The nephropathy of antiphospholipid syndrome
The most commonly reported intrarenal vascular lesion in patients with aPL is thrombotic microangiopathy (TMA) (Fig. 1, Fig. 2, Fig. 3). This condition was initially described in patients with SLE [19], [20], [21] and in patients with lupus anticoagulant (LA) and pregnancy-related renal failure [22]. Subsequently, isolated cases of TMA in patients with primary APS were reported [23], [24]. In our original series, published in 1992, we described five patients who had renal disease and HT among
Renal vein thrombosis
Thrombosis of one or both main renal veins occurs in a variety of settings including trauma, extrinsic compression, renal cell carcinoma, nephrotic syndrome, pregnancy, and oral contraceptives. Renal vein thrombosis (RVT) has been described in aPL positive patients with SLE [37], in a fatal case of renal transplant [38], and in patients with primary APS including a patient with bilateral RVT in the postpartum period [39], [40]. Asherson [41] was the first to describe the association between aPL
The significance of antiphospholipid antibodies in patients with systemic lupus erythematosus nephropathy
The prevalence and significance of glomerular capillary thrombosis in patients with SLE have been studied by Kant and colleagues [42] and Glueck and colleagues [37]. These authors found capillary thrombosis in near 50% of the cases with proliferative glomerulonephritis including 78% in patients with LA and 38% in those without. Notably, the presence of glomerular thrombi in the initial biopsy was a strong predictor of glomerular sclerosis. Other studies have not shown an association between aPL
Summary
Renal involvement is a frequent finding in patients with APS. All vascular structures of the kidney may be affected, leading to diverse clinical consequences including severe hypertension, proteinuria, hematuria, nephrotic syndrome, and renal failure. In some instances ESRD may occur. Unfortunately, APS patients are at high risk of posttransplant renal thrombosis. The nephropathy of APS is characterized by TMA, FIH, and FCA. The nephropathy of APS should be included in the APS classification
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2009, Handbook of Systemic Autoimmune DiseasesCitation Excerpt :In fact, besides thrombosis in medium/large arterial and/or venous renal vessels, aPL have been also associated with kidney lesions characterized by the risk of developing an end-stage insufficiency (APS nephropathy). There are no studies that specifically address the pathogenic mechanisms by which aPL can induce such damage; however, the ability of aPL to perturb endothelium at the level of the kidney microcirculation is likely to play a major role (Amigo, 2006). Central nervous system (CNS) involvement is another APS condition in which clear ischemic events cannot be identifiable in all the cases (Miyakis et al., 2006).