Antiphospholipid syndrome and kidney disease

Abstract

The antiphospholipid syndrome is a common autoimmune disease caused by pathogenic antiphospholipid antibodies, leading to recurrent thrombosis and/or obstetrical complications. Importantly for nephrologists, antiphospholipid antibodies are associated with various renal manifestations including large renal vessel thrombosis, renal artery stenosis, and a constellation of intrarenal lesions that has been termed antiphospholipid nephropathy. This last condition associates various degrees of acute thrombotic microangiopathy, proliferative and fibrotic lesions of the intrarenal vessels, and ischemic modifications of the renal parenchyma. The course of the disease can range from indolent nephropathy to devastating acute renal failure. The pejorative impact of antiphospholipid antibody-related renal complication is well established in the context of systemic lupus erythematous or after renal transplantation. In contrast, the exact significance of isolated antiphospholipid nephropathy remains uncertain. The evidence to guide management of the renal complications of antiphospholipid syndrome is limited. However, the recent recognition of the heterogeneous molecular mechanisms underlying the progression of intrarenal vascular lesions in antiphospholipid syndrome have opened promising tracks for patient monitoring and targeted therapeutic intervention.

Case of Severe Hypertension and Nephrotic Range Proteinuria

“…Our case also reaffirms the role of the antiphospholipid syndrome in renovascular hypertension as has been previously described (9)(10)(11)15,16). Figure 1.…”

“…Bursztyn: Absolutely. There are reviews which note a wide range of renal manifestations of antiphospholipid syndrome including both renal artery stenosis and thrombosis (9)(10)(11). Some of those could have been relevant, but I think there is no question what was the pathophysiology in our case.…”

Antiphospholipid Syndrome: Nephrologists’ Perspective

“…Control of arterial hypertension and anticoagulation when necessary are thought to prevent progression to end-stage renal disease and future thrombosis [13].…”

“…Renal infarction might be associated with APS. In situ thrombosis, emboli from an upstream arterial lesion or a heart valve lesion can result in thrombotic events occurring in small diameter intraparenchymal vessels leading to renal infarction[12] [13].Symptoms at presentation of renal infarction include pain, hypertension (often severe) and impairment of renal function, and it might be one of the first features of APS. Multiple, often serious, thrombotic episodes have been described in some patients, mostly localized in the renal cortex [11] [12] [13].…”

Stellenwert der Nierenbiopsie bei Lupusnephritis

“…Histologisch zeigen sich Zeichen einer thrombotischen Mikroangiopathie (TMA) (▶ abb. 2f), proliferative und fibrotische intrarenale Gefäßveränderungen bis hin zur arteriellen Okklusion und ischämischen Veränderungen im Nierenparenchym [20]. Klinisch sind die betroffenen Patienten meist hypertensiv, können ein akutes Nierenversagen entwickeln [20].…”