Renal and vascular outcomes in patients with isolated antiphospholipid syndrome nephropathy. Issue 132 (October 2022)

• Record Type: Journal Article
• Authors: Rousselin, Clémentine; Amoura, Zahir; Faguer, Stanislas; Bataille, Pierre; Boffa, Jean-Jacques; Canaud, Guillaume; Guerrot, Dominique; Jourde-Chiche for the GCLR, Noémie; Karras, Alexandre; Auxenfants, Eric; Chapelet, Agnès; Lambert, Marc; Behal, Hélène; Nochy, Dominique; Jean-Paul, Duong Van Huyen; Brocheriou for the CFPR, Isabelle; Gnemmi, Viviane; Quemeneur, Thomas
• Is Part Of: Journal of autoimmunity. Issue 132(2022)
• Publication Date: 2022-10
• Subjects: Antiphospholipid syndrome -- Renal prognosis -- End-stage renal disease -- Renal histology; Autoimmunity -- Periodicals; Autoimmune diseases -- Periodicals; Autoantibodies -- Periodicals; Autoimmune Diseases -- Periodicals; Auto-immunité -- Périodiques; Maladies auto-immunes -- Périodiques; Electronic journals; 616.978005
• DOI: 10.1016/j.jaut.2022.102889
• Languages: English
• ISSNs: 0896-8411

Abstract

Abstract: Background: Antiphospholipid syndrome (APS) nephropathy (APSN) is a rare pattern with specific features resulting from microvascular lesions. The prognosis of APSN, outside of lupus nephritis, is unknown. The aim of this study was to describe the renal, vascular and overall outcomes of patients with APSN. Methods: Retrospective multicenter study of patients with antiphospholipid antibodies (aPL) associated with histological APSN lesions and no other nephropathy, identified through a national call for medical records. End-stage renal disease (ESRD)-free survival, thrombosis recurrence-free survival and overall survival were assessed. Results: Thirty patients were included (19 women) with a median age of 40 years (34–52 years). Fifteen patients had APS, 26/28 had lupus anticoagulant, and 15/26 had triple positivity for aPL. Median eGFR was 50 (31–60) mL/min/1.73 m 2 . Glomerular thrombotic microangiopathy was found in 12/24 cases, fibrous intimal hyperplasia in 12/22 cases and focal cortical atrophy in 17/29 cases. Nineteen patients had moderate to severe interstitial fibrosis (>25%). Six patients developed ESRD at a median follow-up of 6.2 (1.8–9.1) years. The ESRD-free survival rates at 5 and 10 years were 80.0% (95% CI 57.6%–91.4%) and 72.7% (95% CI, 46.9%–87.4%) respectively. None of the histological factors considered was significantly associated with a decrease in eGFR at 12 months. Thrombosis recurrence-free survival was 77.8% (95% CI 48.2%–91.6%) at 10 years. Overall survival was 94% at 10 years (95% CI 65.0%–99.2%). Conclusions: The renal prognosis of isolated APSN is poor. The severe fibrotic lesions observed are suggestive of late diagnosis. Highlights: Retrospective multicenter study of patients with antiphospholipid antibodies (aPL) associated with histological APSN lesions. Renal prognosis of isolated APSN is poor with six patients who developed ESRD. Severe fibrotic lesions were observed in 19/30 patients and are suggestive of late diagnosis. Anticoagulation should be considered to lower the risk of thrombosis.