Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: Prevalence, clinical associations, and long‐term outcome

Abstract

Objective. To evaluate the prevalence, clinical associations, and outcome of antiphospholipid syndrome (APS) nephropathy in patients with systemic lupus erythematosus (SLE) and antiphospholipid antibodies (aPL) and in SLE patients without aPL.Methods. Kidney biopsy specimens obtained from 81 patients with aPL (18 of whom had APS) and 70 patients without aPL were retrospectively examined for the presence of APS nephropathy. Clinical and serologic data obtained at the time of kidney biopsy and during a mean followup of 7 years were recorded. In cases for which serial kidney biopsy specimens were available, the evolution of APS nephropathy was examined.Results. APS nephropathy existed in 39.5% of patients with aPL, compared with only 4.3% of patients without aPL. APS nephropathy was associated with both lupus anticoagulant and anticardiolipin antibodies. Among aPL-positive SLE patients, APS nephropathy was found in two-thirds of those with APS and in one-third of those without APS. A strong association between APS nephropathy and the presence of arterial thrombosis and livedo reticularis was noted. Patients with APS nephropathy had a higher frequency of hypertension and elevated serum creatinine levels at the time of kidney biopsy but did not have a higher frequency of renal insufficiency, end-stage renal disease, or death at the end of followup. Serial kidney biopsy specimens were available from 11 patients and showed progression of APS nephropathy lesions. During followup, manifestations of APS (especially arterial thromboses) developed more frequently in the SLE/non-APS patients with APS nephropathy than in those without APS nephropathy.Conclusion. Among patients with SLE, APS nephropathy occurs almost exclusively in those with aPL, suggesting an important role of aPL in the pathogenesis of APS nephropathy. Patients with APS nephropathy develop hypertension, raised serum creatinine levels, and progression of histologic lesions, all of which are associated with a poor renal outcome. Manifestations of APS also tend to develop in these patients. APS nephropathy should be included in the APS classification criteria, and the use of appropriate anticoagulant therapy should be tested.

The clinical significance of antiphospholipid antibodies in systemic lupus erythematosus

“…The prevalence of "aPL-nephropathy" in SLE patients without aPL varies between 4%-16 %, whereas 25%-39% of the aPL-positive SLE patients have "aPL-nephropathy" (92,94 Catastrophic antiphospholipid syndrome Encountered in less than 1% of APS patients, catastrophic APS is characterized by accelerated widespread small/medium vessel thromboses with unusual organ involvement and has a mortality of 30%-50% despite aggressive multimodal intensive treatment (102)(103)(104).…”

Successful Graft Recovery from Thrombotic Acute Kidney Injury in a Kidney Transplant Patient with Antiphospholipid Syndrome

“…The improved blood flow of the grafted kidney after 3 months with continuous anticoagulation therapy also suggested the APSN. APSN is usually associated with a catastrophic functional outcome in the first posttransplant year (5). However in our case, renal function was improved dramatically with the appropriate anticoagulation therapy after the early detection of allograft APSN.…”

“…Antibodies should be demonstrable on at least two occasions separated by 12 weeks(1). These antibodies may be associated with connective tissue diseases such as systemic lupus erythematosus (secondary APS) or be found in isolation (primary APS) (5). However, the most recent international consensus statement on classification criteria for APS advises against this terminology because the clin-ical consequences in both groups appear to be the same (5).…”

Renal involvement in antiphospholipid syndrome

“…The association between aPL and membranous glomerulonephritis (class V) seems unclear, as it has been reported in some studies and anecdotal case reports,24-26 but not confirmed in others. 17,27 In 2002, Daugas et al14 performed a retrospective study of 114 patients with lupus who had undergone renal biopsy. APS nephropathy was observed in 32% of patients with SLE, irrespective of lupus nephritis.…”

“…Indeed, APS nephropathy was associated with positive tests for lupus anticoagulant and antibodies against cardiolipin. 17 The same study evaluated the progression of APS nephropathy through analysis of serial kidney biopsy findings. Evolution from acute thrombotic features to chronic thrombotic disease characterized by proliferative, obstructive and fibrotic lesions was found.…”