Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: Antiphospholipid syndrome (APS) is associated with several patterns of renal involvement. Among them, antiphospholipid nephropathy (APSN) is a rare but specific pattern resulting from microvascular lesions. APSN prognosis, outside lupus nephritis, is unknown. We aimed to describe the renal, vascular and general prognosis of patients with APSN.
Methods: We performed a retrospective multicenter study on patients with antiphospholipid antibodies associated with APSN lesions, and no other nephropathy. Patients were identified through a national call for medical reports. End-stage renal disease (ESRD)-free survival, thrombosis recurrence-free survival and overall survival were assessed. Factors associated with estimated glomerular filtration rate (eGFR) change were also analysed.
Results: Twenty-five patients were included : 18 (72%) women, median age 38.8 (24.6-68.7) years. Seventeen (68%) had APS according to revised Sapporo criteria, 22 (88%) with lupus anticoagulant, 14 (56%) with triple positivity. Median blood pressure was 132 (110-214) / 79.5 (60-124) mmHg, median eGFR was 47.8 (5.8-103) mL/min/1.73m², median proteinuria was 0.95 (0.14-5) g/day. On renal biopsy 13 (52%) had glomerular thrombotic microangiopathy, 17 (68%) fibrous intimal hyperplasia and 3 (12%) focal cortical atrophy. Moderate to severe interstitial fibrosis (≥ 25%) was observed in 11 (44%) patients. Three patients developed ESRD after a median follow-up of 4.4 (0.4-38.3) years,. ESRD-free survival at 5 years was 82.3% (IC95% 65.5%-100%). Two other patients presented a 30% decrease in eGFR. No factor was significantly associated with annual change in eGFR. However patients with interstitial fibrosis (≥ 25%) seemed to have steeper eGFR decline (-0.08 (-4.42;7.005) ; 3.03 (-18.23;14.05) ; p=0.06). Thrombosis recurrence-free survival was 96% at 5 years (IC95% 88.7%-100%), 74% at 10 years (IC95% 51%-100%). One patient died during follow-up. Overall survival was 100% at 5 years and 90% at 10 years (IC95% 73%-100%).
Conclusion: The renal prognosis of isolated APSN is poor in this young population. Up to 20% of patients developed ESRD at 5 years. Severe lesions of renal fibrosis suggest a late diagnosis. The early detection of renal features could improve prognosis with the possibility of evaluating the efficacy of anti-proliferative treatments in APSN.
To cite this abstract in AMA style:Rousselin C, Amoura Z, Karras A, Guerrot D, Boffa JJ, Canaud G, Faguer S, Auxenfants E, Jourde-Chiche N, Lambert M, Quémeneur T. Prognosis of Patients with Antiphospholipid Syndrome Nephropathy [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 10). https://acrabstracts.org/abstract/prognosis-of-patients-with-antiphospholipid-syndrome-nephropathy/. Accessed August 3, 2020.
« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/prognosis-of-patients-with-antiphospholipid-syndrome-nephropathy/