Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (I): catastrophic APS, APS nephropathy and heart valve lesions

Abstract

The objectives of the 'Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations' were to assess the clinical utility of the international consensus statement on classification criteria and treatment guidelines for the catastrophic APS, to identify and grade the studies that analyse the relationship between the antiphospholipid antibodies and the non-criteria APS manifestations and to present the current evidence regarding the accuracy of these non-criteria APS manifestations for the detection of patients with APS. This article summarizes the studies analysed on the catastrophic APS, APS nephropathy and heart valve lesions, and presents the recommendations elaborated by the Task Force after this analysis.

Catastrophic antiphospholipid syndrome (CAPS)

“…An update of the diagnostic algorithm for CAPS was recently proposed [9] and discussed in 'Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations' developed on the occasion of the 13th International Congress on Antiphospholipid, held in Galveston (Texas) in 2011 [10]. This approach represents a step forward in diagnosis of CAPS but it needs to be validated versus other thrombotic microangiopathy.…”

Renal involvement in antiphospholipid syndrome

“…Thus, aPL-related nephropathy warrants inclusion in the diagnosis of APS. 8 In this Review, we describe the role of aPL antibodies in inducing renal disease and the clinical and histological features of APS involving the kidney. We also review the management of patients with renal involvement, including discussions of available and upcoming therapies.…”

Antiphospholipid Antibodies and APS Nephropathy

“…This indicates that the characteristic lesions seen in APS nephropathy occur as a result of the pathological action of aPL. 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].…”

“…They concluded that there was level II-III evidence that aPL correlates with both acute and chronic lesions of APS nephropathy [7]. This Task Force also evaluated the accuracy of APS nephropathy for the detection of patients with APS, comparing primary and secondary APS patients with patients negative for aPL.…”

“…Other causes of renal microangiopathy should be ruled out, including diabetes mellitus (DM), thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), cyclosporine use and systemic sclerosis. In recent years, the typical morphological lesions of APS nephropathy have been well characterized and several studies have established a link between these features and the presence of various aPL [7]. In this review, we outline the proposed pathophysiological mechanisms of aPLmediated thrombosis, the characteristic clinical and morphological features of APS nephropathy and the evidence linking aPL action to the occurrence of APS nephropathy.…”