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 Table of Contents  
Year : 2017  |  Volume : 18  |  Issue : 3  |  Page : 185-189

Hypertensive uveitis: an initial presentation for primary antiphospholipid syndrome

1 Department of Ophthalmology, Kasr Al-Ainy School of Medicine, Faculty of Medicine, Cairo University, Giza, Egypt
2 Department of Rheumatology and Rehabilitation, Kasr Al-Ainy School of Medicine, Faculty of Medicine, Cairo University, Giza, Egypt

Date of Submission14-Jan-2017
Date of Acceptance01-Apr-2017
Date of Web Publication17-Oct-2017

Correspondence Address:
Riham S.H.M Allam
4 Omar Ebn Alkhattab Street, Dokki, Giza 12311
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/DJO.DJO_4_17

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The authors aim to report a case of hypertensive uveitis-associated primary antiphospholipid antibody syndrome (APS). A 25-year-old woman presented with unilateral painless diminution of vision. Examination revealed right chronic anterior uveitis, complicated cataract, and appositional angle-closure glaucoma uncontrolled with medications, for which sequential phacoemulsification–trabeculectomy was performed. Rheumatological consultation revealed the diagnosis of primary APS according to revised Sapporo criteria. Accordingly, systemic steroids and hydroxychloroquine were prescribed. The patient experienced an attack of posterior uveitis 2 years later, which necessitated a maintenance dose of an immunomodulator. Following systemic treatment and intravitreal triamcinolone, the patient’s vision improved from hand motion good projection to 0.3 with no inflammation 8 months following the last relapse. We conclude that hypertensive uveitis can be a presenting feature of primary APS. Antiphospholipid laboratory profile could be added to the workup of uveitis whenever clinical suspicion is present. Thromboprophylaxis could be added when antiphospholipid is positive.

Keywords: hypertensive uveitis, immunomodulators, primary antiphospholipid syndrome, sapporo criteria, thromboprophylaxis

How to cite this article:
Allam RS, Ali BM. Hypertensive uveitis: an initial presentation for primary antiphospholipid syndrome. Delta J Ophthalmol 2017;18:185-9

How to cite this URL:
Allam RS, Ali BM. Hypertensive uveitis: an initial presentation for primary antiphospholipid syndrome. Delta J Ophthalmol [serial online] 2017 [cited 2022 Dec 3];18:185-9. Available from: http://www.djo.eg.net/text.asp?2017/18/3/185/216927

  Introduction Top

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by the presence of arterial and/or venous thrombosis, recurrent fetal loss, and elevated titres of antiphospholipid antibodies (APL), with the three most important being lupus anticoagulant, anticardiolipin, and anti-β2 glycoprotein [1],[2],[3]. APS could be associated with other autoimmune diseases (secondary APS), with systemic lupus erythematosus (SLE) being the most common. However, APS can develop in the absence of an underlying disease (primary APS) [4].

Ocular features of APS have been a subject of growing interest in the last few years. The most important associations are vaso-occlusive retinopathy and neuro-ophthalmologic manifestations [5],[6].

Uveitis has not been known as a feature of primary APS. Anticardiolipin antibodies have been described in patients having uveitis associated with SLE, acute retinal necrosis, and syphilis [7]. Zink et al. [8] reported a case of hypopyon uveitis in the setting of APS associated with SLE.

To the best of our knowledge, panuveitis has not been previously reported with primary APS and therefore this case was reported.

  Case report Top

A 25-year-old female patient presented to the Glaucoma Clinic in June 2014 with painless diminution of vision in the right eye over 6 months. Ophthalmological examination revealed a best-corrected visual acuity (BCVA) of hand motion good projection with good macular functions, sluggish pupillary reflex, poorly dilatable pupil, posterior synechiae, totally opaque lens with intumescence, and shallow anterior chamber. Fine old keratic precipitates were noted on the back of the cornea with no flare or cells. The intraocular pressure (IOP) was 38 mmHg on no treatment and the fundus could not be seen. However, ultrasonography was normal ([Figure 1]a and [Figure 1]b). The left eye was completely normal with a BCVA of 1.00 and IOP of 12 mmHg.
Figure 1 Clinical data of the case (A): slit lamp photo showing a shallow AC and intumescent cataract. (B): B-scan ultrasonography showing an acoustically clear vitreous cavity and retina in place. (C): slit lamp photo after phaco aspiration with no AC reaction. (D): postoperative visual field examination done to evaluate degree of glaucomatous optic neuropathy showing nasal field contraction.

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Gonioscopy of the right eye revealed appositional angle closure with only 2 clock hours of peripheral anterior synechiae. A diagnosis of angle-closure glaucoma with complicated cataract secondary to chronic anterior uveitis was made.

Topical antiglaucoma medications were prescribed (a β-blocker/carbonic anhydrase inhibitor) while awaiting uveitis workup. Follow-up was scheduled 1 week later for IOP monitoring.

Immunologic causes were considered as her sister was known to have SLE. Therefore, the patient was referred to the Rheumatology Clinic to exclude connective tissue disorders. There was no history of arterial or venous thrombosis, no malar rash, photosensitivity, oral ulcers, genital ulcers, alopecia, erythema nodosum, livedo reticularis, Raynaud’s phenomenon, arthralgia, or arthritis, nor neurological manifestations, and no evidence of inflammatory back pain or sacroilitis. She had a history of two consecutive abortions. Laboratory investigations are summarized in [Table 1] and [Table 2].
Table 1 Initial routine laboratory investigations of the patient

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Table 2 Antiphospholipid laboratory profile of the patient

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The patient was diagnosed with primary APS according to the revised Sapporo criteria [9]. She was prescribed acetyl salicylic acid 75 mg/day and hydroxychloroquine 400 mg/day.

IOP in the right eye settled to 20 mmHg on the same prescribed treatment. Hence, decision was made by the glaucoma consultant for phacoaspiration and intraocular lens implantation under cover of systemic prednisolone (40 mg/day) as recommended by the rheumatologist. Surgery was performed in July 2014 and postoperative IOP was maintained in the low teens in the immediate postoperative period with a BCVA of 0.8 and a baseline perimetry showing advanced glaucomatous optic neuropathy ([Figure 1]c and [Figure 1]d). Two weeks later progressive IOP elevation was experienced and a decision for subscleral trabeculectomy was taken, and surgery was performed 6 weeks (24 August 2014) after the cataract surgery with adjuvant mitomycin C application (0.4 mg/ml applied for 2 min; [Figure 2]a and [Figure 2]b).
Figure 2 Slit lamp photos of the patient after trabeculectomy (A): first postoperative day and (B): one month later showing a diffuse bleb with normal vascularization.

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Throughout the follow-up period, the patient did not show any signs of active uveitis with an IOP of 13 mmHg (on single topical treatment in the form of a β-blocker − i.e. qualified success) and a BCVA of 0.3 due to posterior capsular opacification, which improved to 0.8 after YAG laser posterior capsulotomy. Oral prednisolone was gradually tapered over a period of 6 months, until it was discontinued.

On January 2016, the patient came up with acute red eye and diminution of visual acuity, BCVA of 0.16, cells +2 and keratic precipitates on the back of the cornea, IOP of 34 mmHg on same topical treatment, and a hazy view of the fundus (moderate vitritis; [Figure 3]). Gonioscopy showed a partially closed ostium. Fundus fluorescein angiography showed macular edema ([Figure 4]a and [Figure 4]b) and optical coherence tomography macula showed sensory macular detachment ([Figure 5]). Intravitreal triamcinolone (4 mg/0.1 ml), Topical steroids, cycloplegic, and antiglaucoma treatment were started, with oral prednisolone 40 mg/day followed by azathioprine at a dose of 150 mg/day with tapering of prednisolone dose to 20 mg/day.
Figure 3 Ocular ultrasonography showing vitritis (left) before and (right) after intravitreal triamcinolone.

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Figure 4 FFA showing (A) mild vitritis with late leakage at the macula of the right eye and (B) normal FFA in the left eye.

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Figure 5 Patient 2 years after her first presentation (A): flare and cells. (B): keratic precipitates, (C): OCT showing macular edema and (D): sensory detachment.

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The patient’s BCVA improved to 0.3, and IOP reached 15 mmHg on a topical combined β-blocker/carbonic anhydrase inhibitor.

Further tapering of prednisolone was achieved and the patient is now maintained on a dose of 5 mg/day together with azathioprine at a dose of 100 mg/day with no signs of inflammation, 8 months following the last relapse.

Informed consent was obtained from the patient for possible publication of this work without declaring her name or showing personal photos or images that might declare her personality.

  Discussion Top

In the present case the diagnosis of APS was made according to the revised Sapporo criteria with a clinical evidence of pregnancy morbidity as well as laboratory evidence of elevated APL antibodies [anticardiolipin immunoglobulin IgM/IgG, lupus anticoagulant, and antiB2GP IgM/IgG], which were positive on repeated examinations. A 2-year follow-up period did not unmask any hidden autoimmune diseases, which confirmed the diagnosis of primary APS.

The present case first presented with hypertensive uveitis and complicated cataract and was responsive to systemic steroids, and then posterior uveitis was evident 2 years later; a situation that necessitates a maintenance dose of an immunomodulator. Positive APL antibodies carry a higher risk for thrombotic crises, which mandates thromboprophylaxis [10].

To the best of our knowledge this is the first case to be reported in the literature of primary APS presenting with hypertensive panuveitis, and hence we can conclude that uveitis (anterior or posterior or panuveitis) with or without IOP elevation can be a presenting feature of primary APS. APL laboratory profile could be added to the workup of uveitis whenever a clinical suspicion is present. Thromboprophylaxis could be added under the supervision of the internist to protect the patients from life and/or ocular threatening thrombotic crises, particularly when APL is positive.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Asherson RA, Cervera R, Piette JC, Shoenfeld Y. The antiphospholipid syndrome: history, definition, classification, and differential diagnosis. In: Asherson RA, Cervera R, Piette JC, Shoenfeld Y, editors. The antiphospholipid syndrome. Boca Raton, FL: CRC Press; 1996. pp. 3–12.  Back to cited text no. 1
Cervera R, Reverter JC, Khamashta MA. Antiphospholipid syndrome in systemic autoimmune diseases. Amsterdam: Elsevier; 2009.  Back to cited text no. 2
Miserocchi E, Baltatzis S, Foster SC. Ocular features associated with anticardiolipin antibodies: a descriptive study. Am J Ophthalmol 2001; 131:451–456.  Back to cited text no. 3
Asherson RA, Cervera R, Piette JC, Font J, Lie JT, Burcoglu A et al. Catastrophic antiphospholipid syndrome: clinical and laboratory features of 50 patients. Medicine (Baltimore) 1998; 77:195–207.  Back to cited text no. 4
Gelfand YA, Dori D, Miller B, Brenner B. Visual disturbances and pathologic ocular findings in primary antiphospholipid syndrome. Ophthalmology 1999; 106:1537–1540.  Back to cited text no. 5
Castañón C, Amigo MC, Bañales JL, Nava A, Reyes PA. Ocular vaso-occlusive disease in primary antiphospholipid syndrome. Ophthalmology 1995; 102:256–262.  Back to cited text no. 6
Klok AM, Geertzen R, Rothova A, Baarsma GS, Kijlstra A. Anticardiolipin antibodies in uveitis. Curr Eye Res 1992; 11 (Suppl):209–213.  Back to cited text no. 7
Zink JM, Singh-Parikshak R, Johnson CS, Zacks DN. Hypopyon uveitis associated with systemic lupus erythematosus and antiphospholipid antibody syndrome. Graefes Arch Clin Exp Ophthalmol 2005; 243:386–388.  Back to cited text no. 8
Miyakis S, Lockshin MD, Atsumi T, Branch DW, Bray RL, Cervera R et al. International consensus statement on an update of the classification criteria for the definite antiphospholipid syndrome (APS). J Throm Haemost 2006; 4:295–306.  Back to cited text no. 9
Gómez-Puerta JA, Cervera R. Diagnosis and classification of the antiphospholipid syndrome. J Autoimmun 2014; 48–49:20–25.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

  [Table 1], [Table 2]

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