Deep capillary retinal ischemia and high-titer prothrombin-associated antiphospholipid antibodies: A case series of three patients

Abstract

Purpose: Acute macular neuroretinopathy has been shown to be due to ischemia of the deep capillary retinal plexus and most cases occur in young women; we hypothesized that there may be an association with antiphospholipid antibodies.

Observations: We identified three patients who were diagnosed with deep capillary retinal ischemia after presenting with sudden onset of focal paracentral scotoma who tested persistently positive for antiphospholipid antibodies. All patients had high-titer prothrombin-associated antibodies and two of the three also had low-titer anticardiolipin antibodies. In all patients, the diagnosis was missed at the initial presentation. All patients experienced involvement of both eyes over time with permanent visual deficits and all were female with an average age at symptom onset of 34 years. All patients were using exogenous estrogen and had additional but previously undiagnosed symptoms or signs that may be seen in the antiphospholipid syndrome. One patient was ANA positive with a titer of 1:320, but none had lupus-specific antibodies or clinical features of lupus.

Conclusions and importance: The persistent presence of high-titer prothrombin-associated antiphospholipid antibodies in three women with deep capillary retinal ischemia suggests this may be an important association. Prothrombin-associated antibodies (anti-prothrombin IgG and anti-phosphatidylserine-prothrombin IgG and IgM) as well as the traditional antiphospholipid antibodies (anticardiolipin IgG, IgM and IgA; anti-beta 2 glycoprotein I IgG, IgM and IgA; and the lupus anticoagulant) should be included in the diagnostic work-up of patients diagnosed with deep capillary retinal ischemia. Because of the broader health and treatment implications of high-titer antiphospholipid antibodies, further investigation into this suspected association is warranted.

Keywords: Acute macular neuroretinopathy; Anti-Phosphatidylserine-prothrombin antibodies; Antiphospholipid syndrome; Optical coherence tomography angiography; Paracentral acute middle maculopathy; Retinal ischemia; SD-OCT, Spectral domain optical coherence tomography.

Fig. 1

OCT, OCTA and Amsler grid from Case 2. OCT demonstrating damage to the outer plexiform layer in the left eye (arrow) (A); OCTA demonstrating deep parafoveal capillary loss in the left eye (arrow) (B); OCT demonstrating a hyper-reflective lesion in the outer retina of the right eye (arrow) (C); The corresponding Amsler grid from the right eye of this patient (D).