APLS-ASSOCIATED RETINAL VASCULOPATHY AS A PRESENTATION OF THROMBOTIC MICROANGIOPATHY

Background: Persistent antiphospholipid antibodies (aPL) positivity was a recognized risk factor for thrombotic events, obstetric morbidity and a variety of manifestations beyond thrombosis. The presence of some non-criteria manifestations including thrombocytopenia, hemolytic anemia, and APS nephropathy should prompt consideration for thrombotic microangiopathy (TMA). Patients with APS can also present with a variety of ocular and neuro-ophthalmic manifestations, such as retinal artery/vein occlusion, retinal arteritis, optic neuritis and ischemic optic neuropathy, with underlying mechanisms remained elusive. Retinal vasculopathy including retinal artery occlusion (RAO) or retinal vein occlusion (RVO) was recently found occurred more frequently in APS patients with thrombocytopenia , suggested other possible mechanisms besides thromboembolism. Objectives: To explore risk factors and possible mechanisms of retinal vasculopathy among APS patients. Methods: In this single-center case-control study among APS patients, we evaluated patients who fulfilled 2006 Sapporo APS Classification Criteria with or without retinal vasculopathy during 2018-2020 at Peking Union Medical College Hospital. Demographic data, aPL-related manifestations, cardiovascular risk factors and antibodies profile were compared and a logistical regression model was built. Hierarchical cluster analysis with the Euclidean distance and the Ward method was applied to identify clusters of variables. Results: A total of 310 APS patients (67.4% female, mean age 38.1 years) were included, of whom 18 patients were diagnosed with retinal vasculopathy (9 with RVO and 9 with RAO). No significant differences was found among most demographic characteristics, clinical manifestations, or antibody profile. However, APS-related heart valve disease (OR 13.66, 95% confidence interval [CI] 4.55-40.98), APS nephropathy (OR 12.77, 95% CI 4.04-40.35), thrombocytopenia (OR 2.63, 95% CI 1.01-6.89) and high serum IgM (OR 3.67, 95% CI 1.30-10.40) were predictive of retinal vasculopathy ( Figure 1 A). APS-related heart valve disease and nephropathy were also found statistical significant in multivariate logistical regression ( Figure 1 B). They and other non-criteria manfestations were aggregated with retinal vasculopathy from cluster analysis of variables ( Figure 1 C). Conclusion: Patients with APS-related heart valve disease and nephropathy suffered a higher risk of retinal vasculopathy including RAO and RVO. The underlying mechanisms of aPLs-associated retinal vasculopathy may involve TMA, leading to a poor prognosis and therapeutic changes. REFERENCES: [1]Kotzen ES, Roy S, Jain K. Antiphospholipid Syndrome Nephropathy and Other Thrombotic Microangiopathies Among Patients With Systemic Lupus Erythematosus. Adv Chronic Kidney Dis. 2019 Sep;26(5):376-386. [2]Ermakova NA, Alekberova ZS, Reshetniak TM, Kalashnikova LA, Kosheleva NM. [Retinal vascular lesions in systemic lupus erythematosus and secondary antiphospholipid syndrome]. Vestn Oftalmol. 2005 Sep-Oct;121(5):31-6. [3]Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006 Feb;4(2):295-306. Figure 1 . A) Forest plot of univariate analysis; B) Forest plot of multivariate logistic regression; C) Cluster analysis of variables. Disclosure of Interests: None declared Citation: Ann Rheum Dis, volume 80, supplement 1, year 2021, page 641Session: SLE, Sjögren’s and APS - clinical aspects (other than treatment) (POSTERS only)