Ocular findings in asymptomatic patients with primary antiphospholipid syndrome

• Published in: Lupus 2022-12, Vol.31 (14), p.1800-1807
• Main Authors: Neto, Taurino SR, Neto, Epitacio DS, Balbi, Gustavo GM, Signorelli, Flavio, Higashi, Alex H, Monteiro, Mário Luiz R, Bonfá, Eloisa, Zacharias, Leandro C, Andrade, Danieli CO
• Format: Article
• Language: English
• Subjects: Antiphospholipid antibodies; Antiphospholipid syndrome; Asymptomatic; Autoimmune diseases; Eye; Fetuses; Hyperlipidemia; Optic neuropathy; Patients; Retina; Rheumatology; Risk factors; Structure-function relationships; Thrombosis
• ISSN: 0961-2033; 1477-0962
• DOI: 10.1177/09612033221133687

Background Primary antiphospholipid syndrome (PAPS) is characterized by the presence of antiphospholipid antibodies (aPL), repetitive fetal loss, and arterial/venous thrombosis and no association with other autoimmune rheumatic disease. Ocular involvement can also occur including retinal vascular thrombosis and neuro-ophthalmological manifestations, such as optic neuropathy and amaurosis fugax. Early detection of ocular changes is crucial to minimize functional loss. Purpose To perform a multimodal evaluation, including the use of Optical Coherence Angiotomography (OCTA), in patients with PAPS without ocular complaints and compare with healthy individuals. Methods We performed a complete structural and functional ophthalmological evaluation using OCTA and microperimetry exam in patients with PAPS, followed at a tertiary Rheumatology outpatient clinic. Results We included 104 eyes of 52 subjects [PAPS without ocular complaints (N = 26) and healthy individuals (N = 26)]. Among PAPS patients, 21 were female (80.8%) and 21 (80.8%) were Caucasians. PAPS manifestations were venous (65.4%), arterial thrombosis (34.6%), and obstetrical (34.6%) and all of them had lupus anticoagulant. Ophthalmologic findings were more frequent in PAPS compared to healthy individuals (19.2% vs. 0%, p = 0.05). The most common retinal change was paracentral acute middle maculopathy (PAMM) (3 patients, 5 eyes), followed by drusen (1 patient, 2 eyes) and pachychoroid pigment epitheliopathy (PPE) (1 patient, 1 eye). Hypertension and hyperlipidemia were present in 100% of the PAPS patients with PAMM, while only six patients (26.1%) with PAPS without PAMM presented these two risk factors together (p = 0.03). Conclusions We provide novel evidence that approximately 20% of our asymptomatic PAPS patients without ocular symptoms have ophthalmologic findings that require early identification and careful surveillance focusing on minimizing systemic and vascular risk factors.