Dataset Information

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.

SUBMITTER: Schofield JR

PROVIDER: S-EPMC6461562 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

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

Schofield Jill R JR Palestine Alan G AG Pelak Victoria V Mathias Marc T MT

American journal of ophthalmology case reports 20190402

<h4>Purpose</h4>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.<h4>Observations</h4>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-ti ...[more]

PMID: 31011663

Similar Datasets

Project description:PurposeTo study longitudinal changes in retinal capillary circulation in eyes treated with iodine 125 (I125) plaque brachytherapy for uveal melanoma using OCT angiography (OCTA).DesignLongitudinal prospective study of 21 patients undergoing treatment for uveal melanoma with I125 plaque brachytherapy. Eyes with melanoma were imaged with OCTA before treatment and at 12-month intervals until 2 years after brachytherapy.ParticipantsAfter institutional review board approval, participants were enrolled prospectively from an academic ocular oncology clinic.MethodsPeripapillary (4.5 × 4.5-mm) and macular (3 × 3-mm) OCTA scans were acquired with AngioVue (Optovue, Inc, Fremont, CA).Main outcome measuresThe peripapillary nerve fiber layer plexus capillary density (NFLP_CD), macular superficial vascular complex vessel density (mSVC_VD), and foveal avascular zone (FAZ) area were calculated.ResultsBefore treatment, no significant difference was found in the NFLP_CD, mSVC_VD, or FAZ area between eyes with melanoma and normal fellow eyes. By 24 months, 11 eyes had developed clinical signs of radiation retinopathy, radiation optic neuropathy, or both. In treated eyes, the NFLP_CD (48.4±4.1%) was reduced at 12 months (46.7±5.0%; P = 0.04, Wilcoxon signed-rank test) and 24 months (44.5±6.1%; P < 0.001). Similarly, the mSVC_VD (48.4 2±3.6%) was reduced in treated eyes at 12 months (43.5±5.9%; P = 0.01) and 24 months (37.4±9.1%; P < 0.001). The FAZ area (0.26±0.11 mm2) increased in treated eyes at 12 months (0.35±0.22 mm2; P = 0.009) and 24 months (0.81±1.03 mm2; P = 0.001). When only eyes with clinically evident radiation changes were evaluated, the changes in NFLP_CD, mSVC_VD, and FAZ area were more pronounced. OCT angiography measurements correlated with both radiation dose and visual acuity. The mSVC_VD measured at 12 months was found to predict the development of clinically apparent radiation retinopathy within 1 year.ConclusionsOCT angiography demonstrated early emergence of peripapillary and macular capillary vasculature changes after I125 plaque brachytherapy. OCT angiography provided a quantitative measurement of retinal capillary changes associated with ischemia that correlated with visual acuity and radiation dose and may predict future development of radiation-induced retinal toxicity.

Project description:Increasing evidence indicates that pericytes are vulnerable cells, playing pathophysiological roles in various neurodegenerative processes. Microvascular pericytes contract during cerebral and coronary ischemia and do not relax after re-opening of the occluded artery, causing incomplete reperfusion. However, the cellular mechanisms underlying ischemia-induced pericyte contraction, its delayed emergence, and whether it is pharmacologically reversible are unclear. Here, we investigate i) whether ischemia-induced pericyte contractions are mediated by alpha-smooth muscle actin (α-SMA), ii) the sources of calcium rise in ischemic pericytes, and iii) if peri-microvascular glycogen can support pericyte metabolism during ischemia. Thus, we examined pericyte contractility in response to retinal ischemia both in vivo, using adaptive optics scanning light ophthalmoscopy and, ex vivo, using an unbiased stereological approach. We found that microvascular constrictions were associated with increased calcium in pericytes as detected by a genetically encoded calcium indicator (NG2-GCaMP6) or a fluoroprobe (Fluo-4). Knocking down α-SMA expression with RNA interference or fixing F-actin with phalloidin or calcium antagonist amlodipine prevented constrictions, suggesting that constrictions resulted from calcium- and α-SMA-mediated pericyte contractions. Carbenoxolone or a Cx43-selective peptide blocker also reduced calcium rise, consistent with involvement of gap junction-mediated mechanisms in addition to voltage-gated calcium channels. Pericyte calcium increase and capillary constrictions became significant after 1 h of ischemia and were coincident with depletion of peri-microvascular glycogen, suggesting that glucose derived from glycogen granules could support pericyte metabolism and delay ischemia-induced microvascular dysfunction. Indeed, capillary constrictions emerged earlier when glycogen breakdown was pharmacologically inhibited. Constrictions persisted despite recanalization but were reversible with pericyte-relaxant adenosine administered during recanalization. Our study demonstrates that retinal ischemia, a common cause of blindness, induces α-SMA- and calcium-mediated persistent pericyte contraction, which can be delayed by glucose driven from peri-microvascular glycogen. These findings clarify the contractile nature of capillary pericytes and identify a novel metabolic collaboration between peri-microvascular end-feet and pericytes.

Dataset Information

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

Publications

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

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets