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 Table of Contents  
Year : 2018  |  Volume : 9  |  Issue : 1  |  Page : 33-36

Antiphospholipid syndrome presenting with nonarteritic anterior ischemic optic neuropathy

King Faisal Specialist Hospital and Research Centre, Alfaisal University, Riyadh, Saudi Arabia

Date of Web Publication22-Mar-2018

Correspondence Address:
Hessah Altammami
King Faisal Specialist Hospital and Research Centre, Alfaisal University, Riyadh
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joah.joah_73_17

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We report an atypical manifestation of Antiphospholipid syndrome (APS) in a 40-year-old male with nonartertic anterior ischemic optic neuropathy (NAION) presenting with 12-day history of a headache and left eye pain not associated with visual changes. On his initial evaluation, our differential diagnoses included optic neuritis, ischemic optic neuropathy (ION), and intracranial hemorrhage. Onset and course of the disease, lack of vision changes as well as the presence of retinal hemorrhages on fundoscopic examination excluded optic neuritis. The diagnosis was made after exclusion of all other causes including infectious and noninfectious causes of optic neuritis, ION, and intracranial hemorrhage. This case demonstrates the importance of including APS as a differential diagnosis of NAION in middle-aged individuals.

Keywords: Antiphospholipid syndrome, nonartertic anterior ischemic optic neuropathy, optic neuritis

How to cite this article:
Altammami H, Alzahrani H, Elmansouri J, Gashgrey D. Antiphospholipid syndrome presenting with nonarteritic anterior ischemic optic neuropathy. J Appl Hematol 2018;9:33-6

How to cite this URL:
Altammami H, Alzahrani H, Elmansouri J, Gashgrey D. Antiphospholipid syndrome presenting with nonarteritic anterior ischemic optic neuropathy. J Appl Hematol [serial online] 2018 [cited 2023 Sep 22];9:33-6. Available from: https://www.jahjournal.org/text.asp?2018/9/1/33/228336

  Introduction Top

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by arterial or venous thrombosis that can affect virtually any organ, associated with the laboratory presence of antiphospholipid antibody (anti-cardiolipin IgG, anti-cardiolipin IgM, and anti-B2-Glycoprotein I) or lupus antibody. APS can occur as a single entity (primary) or as a manifestation of another underlying disease (secondary) most commonly in systemic lupus erythematosus and other conditions such as drug-induced, infections, and cancers.[1]

The most common manifestation of the disease is deep vein thrombosis, occurring in 29%–55% of patients.[2]

Ocular involvement in APS is seen in 8%–88% of patients.[3],[4] It can be the first clinical sign of APS and has a wide range of presentation that can be unilateral or bilateral, varying from visual acuity reduction and amaurosis fugax to transient scotoma and visual field defect. Conjunctival hyperemia, ocular discomfort, and pain have often been reported in the literature as common symptoms of APS patients.[5]

We report an atypical manifestation of APS in a 40-year-old male patient, who presented with headache and left eye pain.

  Case Report Top

A 40-year-old male patient known to have primary APS presented to our emergency department complaining of severe left-sided headache with the left eye pain that was not worsened by eye movement, with no history of sudden decrease in vision. However, the patient had left eye amblyopia of which he was aware when questioned. The patient's medical history was remarkable for APS with three previous deep venous thrombosis (DVTs) in the right leg. The patient's ocular history was unremarkable. The patient was taking warfarin 10 mg PO daily with a target international normalized ratio (INR) of around 3.5.

The patient was a heavy smoker (20 cigarettes/day for the past 20 years). The family history was remarkable with two sisters having DVT, although their details were unavailable because they were following up in a different hospital.

On examination, entering corrected visual acuity was 20/20 in the right eye and 20/50 in the left eye. The right pupil was round and reactive to light and accommodation, whereas the left pupil showed a very mild relative afferent pupillary defect. Color vision was normal in both eyes. Extraocular muscles were unrestricted in all fields of gaze. Anterior segment examination yielded normal findings for both eyes. Intraocular pressure was 14 mm Hg in both eyes. Dilated funduscopic examination revealed clear vitreous in both eyes. The media was clear in both eyes. The disc vessels of the macula in the right eye were normal, whereas the left eye showed severe left optic disk edema, and retinal hemorrhages surrounding the optic disc [Figure 1]. Visual field testing with automated perimetry showed incomplete superior altitudinal defect [Figure 2].
Figure 1: Fundus photograph of the left eye. Note the left severe optic disk edema, and retinal hemorrhages surrounding the optic disk

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Figure 2: Visual field test of left eye demonstrating an incomplete superior altitudinal defect

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The patient's most recent laboratory tests, including complete blood count, random glucose, cholesterol, and triglycerides, were reviewed and found to be within normal ranges. However, partial thromboplastin time and prothrombin time were 73.8 s and 26.7 s, respectively, and INR was 2.4.

The results of antiphospholipid antibody tests showed positive lupus anticoagulant test (by diluted Russell's viper venom time and Staclot assay) more than 3 months apart.

In addition, anticardiolipin by ELISA IgG level was 56.6 GPL/mL (0.0–15.0) repeated more than 3 months later with a value of 31.2 GPL/mL. Finally, antinuclear antibody tests were repeatedly negative.

Computed tomography angiogram of the brain and magnetic resonance venography were performed, and both showed normal findings. Magnetic resonance imaging of the orbit showed left perioptic sheath enhancement with mild retro-orbital edema, and optic disc swelling. Findings were consistent with an optic neuropathy of the left side.

At that stage, differential diagnoses included an atypical optic neuritis or ischemic optic neuropathy (ION). Onset and prolonged course of the disease, the patient's age, lack of color vision changes, vision deterioration in further follow-ups, and the presence of hemorrhages excluded atypical optic neuritis. The diagnosis was made after the exclusion of all other causes including infectious and noninfectious causes of optic neuritis.

The patient was admitted, and his target INR was kept at 2.5–3.5 due to recurrent thromboembolic events despite near adequate anticoagulation. Because the patient's INR was slightly subtherapeutic at the time of presentation, he was treated with enoxaparin 80 mg subcutaneously twice daily, plus Warfarin 10 mg PO daily.

Furthermore, the patient was started on pulsed steroids, dexamethasone 25 mg every 6 h for 3 days. We started the patient on prednisone PO at 80 mg daily. The patient's symptoms improved and he was discharged with an INR of 3.1 on warfarin 10 mg PO daily and pulsed steroid with a tapering dose of 5 mg every 2 weeks and discontinue after 4 months.

The patient followed up 3 months after the discharge at the ophthalmology clinic. His visual acuity dropped in his left ocular examination to 20/100, which was clinically compatible with nonarteritic ION rather than optic neuritis. His INR was maintained within target range on follow-up without any new thromboembolic event.

  Discussion Top

The systemic features of APS are characterized by an immense variety depending on the involved organs. However, studies on the frequency and clinical presentation of the ocular manifestations of APS are lacking.[6] In this report, we present a case of atypical nonarteritic anterior ION (NAION) as a manifestation of APS, which has only been reported to our knowledge in two case reports.

ION is believed to result from vascular insufficiency of the optic nerve and is typically divided into anterior and posterior forms depending on which part of the nerve is affected.[6],[7],[8] The mean age of onset of NAION is 64 years.[9],[10],[11]

Treatment of optic neuropathy in primary APS is controversial and should be individually tailored. Anticoagulation therapy can prevent future events not only in the eyes but also in other vital organs.[2] Our patient received systemic steroids due to the initial suspicion of atypical optic neuritis. However, high doses of corticosteroids are usually given empirically to patients with severe thrombocytopenia, hemolytic anemia, and catastrophic APS.

Tsironi et al.[6] reported three cases with ocular manifestations of APS. The first patient had bilateral retinal occlusive disease and the second and third patient had unilateral NAION with macular edema.[6]

Reino et al.[12] described optic neuropathy in association with systemic thrombotic manifestations, most commonly seen in neurological disorders followed by cardiac disease, arterial and venous thrombosis, miscarriage, and livedo reticularis.[12] In addition, Tugcu et al.[13] reported a case with NAION associated with positive APS antibodies.[13] In that case report, the patient had a pale optic disc in contrast to our case, where the patient was having hemorrhagic optic disc and lacking sudden decrease in vision with normal color vision. Nevertheless, both patients had steroids as part of their treatment regimen. However, there was no improvement in our patient.

In conclusion, patients with APS can present with ocular manifestations such as NAION. The early recognition of NAION has important diagnostic and therapeutic implications. Thus, internists and ER physicians should think of ION as a differential diagnosis in patients with APS who present with unexplained ocular manifestation and refer these patients for a full ophthalmologic examination. We suggest the consideration of catastrophic APS management in patients with severe one organ involvement since the prompt administration of anticoagulant treatment and steroids is known to be essential for better outcome, and potentially for vision salvation and stabilization.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Behbehani R, Sergott RC, Savino PJ. The antiphospholipid antibody syndrome: Diagnostic aspects. Curr Opin Ophthalmol 2004;15:483-5.  Back to cited text no. 1
Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med 2002;346:752-63.  Back to cited text no. 2
Asherson RA, Merry P, Acheson JF, Harris EN, Hughes GR. Antiphospholipid antibodies: A risk factor for occlusive ocular vascular disease in systemic lupus erythematosus and the 'primary' antiphospholipid syndrome. Ann Rheum Dis 1989;48:358-61.  Back to cited text no. 3
Castañón C, Amigo MC, Bañales JL, Nava A, Reyes PA. Ocular vaso-occlusive disease in primary antiphospholipid syndrome. Ophthalmology 1995;102:256-62.  Back to cited text no. 4
Utz VM, Tang J. Ocular manifestations of the antiphospholipid syndrome. Br J Ophthalmol 2011;95:454-9.  Back to cited text no. 5
Tsironi E, Gatselis N, Kotoula MG, Zachou K, Pefkianaki M, Zacharaki F, et al. Ocular disorders as the prevailing manifestations of antiphospholipid syndrome: A case series. Cases J 2009;2:159.  Back to cited text no. 6
Biousse V, Newman NJ. Ischemic optic neuropathies. N Engl J Med 2015;372:2428-36.  Back to cited text no. 7
Arnold AC. Pathogenesis of nonarteritic anterior ischemic optic neuropathy. J Neuroophthalmol 2003;23:157-63.  Back to cited text no. 8
Johnson LN, Arnold AC. Incidence of nonarteritic and arteritic anterior ischemic optic neuropathy. Population-based study in the state of Missouri and Los Angeles county, California. J Neuroophthalmol 1994;14:38-44.  Back to cited text no. 9
Characteristics of patients with nonarteritic anterior ischemic optic neuropathy eligible for the Ischemic Optic Neuropathy Decompression Trial. Arch Ophthalmol 1996;114:1366-74.  Back to cited text no. 10
Cestari DM, Gaier ED, Bouzika P, Blachley TS, De Lott LB, Rizzo JF, et al. Demographic, systemic, and ocular factors associated with nonarteritic anterior ischemic optic neuropathy. Ophthalmology 2016;123:2446-55.  Back to cited text no. 11
Reino S, Muñoz-Rodriguez FJ, Cervera R, Espinosa G, Font J, Ingelmo M, et al. Optic neuropathy in the “primary” antiphospholipid syndrome: Report of a case and review of the literature. Clin Rheumatol 1997;16:629-31.  Back to cited text no. 12
Tugcu B, Acar N, Coskun CT, Celik S, Yigit FU. Nonarteritic anterior ischemic optic neuropathy as the presenting manifestation of primary antiphospholipid syndrome. Indian J Ophthalmol 2014;62:642-4.  Back to cited text no. 13
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