Recurrent Thrombosis and Renal Vascular Disease in Patients with a Lupus Anticoagulant

Abstract

In five patients suffering from recurrent thrombosis and/or fetal death, a lupus anticoagulant was associated with a renal vasculopathy. Ischaemic episodes also involved the skin, heart, eyes and/or central nervous system. All patients were hypertensive. Two had renal insufficiency, two had non-nephrotic proteinuria, and in the last patient renal cortical ischaemia was detected by a tomographic scan in the absence of proteinuria. Renal biopsy showed thrombosis and/or intimal fibrosis of intrarenal vessels, and normal or ischaemic glomeruli without proliferative lesions. High-titres of anticardiolipin antibodies were found in 3 of 3 cases, and persisted after steroid therapy even if the circulating anticoagulant factor disappeared. All patients received corticosteroid therapy, alone or in combination with immunosuppressive drugs; two patients had prolonged oral anticoagulation, but thrombotic episodes recurred after stopping the drug. One patient died; the remaining four survived 18 months to 11 years after diagnosis, with stable chronic renal insufficiency in one of them. These results show that a lupus anticoagulant may be associated with prominent renal vascular disease, in the absence of proliferative glomerular lesions, and suggest that continuous anticoagulation may be beneficial in these patients.

Antiphospholipid Antibodies and APS Nephropathy

“…In a study of APS nephropathy in systemic lupus erythematosus (SLE) patients, hypertension was a presenting feature in 77.1% of SLE patients with APS nephropathy and was the only clinical feature that remained significantly associated with APS nephropathy after multivariate regression analysis [50]. Other studies of renal disease in SLE patients with aPL report prevalence rates of hypertension approaching 100% [51]. The typical thrombotic microangiopathic intrarenal lesions that characterize APS nephropathy generate systemic hypertension, which in turn may worsen these lesions leading to progressive damage.…”

“…The typical thrombotic microangiopathic intrarenal lesions that characterize APS nephropathy generate systemic hypertension, which in turn may worsen these lesions leading to progressive damage. The appearance of hypertension in a patient with primary or secondary APS could indicate the development of nephropathy associated with this disorder or stenosis or occlusion of the renal artery [46,[50][51][52]. As such, APS patients who develop hypertension, even in the absence of proteinuria, hematuria or decreased glomerular filtration rate, must be extensively investigated, including renal biopsy [53].…”

“…Hematuria occurred in 56% of patients, while microangiopathic hemolytic anemia occurred rarely. Other studies of APS nephropathy in primary APS patients also report the high prevalence of hypertension, mild insufficiency, mild proteinuria and intermittent hematuria, with the rare occurrence of nephrotic range proteinuria and end-stage renal disease [47,[51][52][53][54][55].…”

“…Interestingly, APS nephropathy occurs with significantly higher frequency in primary and secondary APS patients, compared to non-APS lupus patients with aPL positive for aPL [7,50]. The prevalence of APS nephropathy in primary APS patients with renal disease was almost always 100% across several studies [46,47,55,[69][70][71][72], while the prevalence in secondary APS patients with SLE and biopsy-proven renal involvement ranged from 67% to 100% across studies [48,50,51,70,[73][74][75]. The association of APS nephropathy with aPL, seems to be strongest for LA, but has been reported for anticardiolipin antibodies as well.…”

The Kidney in Antiphospholipid Syndrome

“…Hypertension from mild to malignant, hematuria, proteinuria from mild to nephrotic range, acute or chronic renal failure are the main clinical and laboratory findings in patients with SLE and aPL [Kleinknecht et al, 1989]. But all these findings may be the consequence of antiphospholipid syndrome nephropathy without any lesion of lupus nephropathy, or lupus nephropathy alone, or lupus nephropathy associated with antiphospholipid nephropathy.…”