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Saudi Journal of Ophthalmology (2013) 27, 247—251

Original Article

Ocular manifestations of Behçet’s disease in Jordanian patients
Mohammed A. Abu-Ameerh, FRCS ⇑; Sawsan F. Mohammed, MD; Mona T. Mohammad, MD; Osama H. Ababneh, MD;
Muawyah D. Al-Bdour, MD

Abstract

Purpose: To study the prevalence, manifestations and severity of ocular involvement of Behçet’s disease in Jordanian patients.
Methods: The study population consisted of 43 patients diagnosed to have Behçet’s disease through Rheumatologist’s examin-
ations conducted at Jordan University Hospital between January 2002 and July 2009. The sample involved patients who displayed
ocular manifestations. This included 18 patients; 12 males and 6 females with a mean age of 35 years (SD = 17.26). Ophthalmo-
logical examinations and retrospective analysis of medical files were carried on.
Results: Ocular manifestations were seen in 41.9% of patients. The most common manifestation for Behçet’s disease was vitritis
with a prevalence of 55.6%, followed by anterior uveitis and retinal vasculitis (50% for each). On the other hand, the most frequent
complications involved were cataract, cystoid macular edema (CMO), posterior synechiae and glaucoma with a prevalence of
(44.4%), (33.3%), (11.1%) and (5.6%), respectively.
Conclusion: The prevalence and severity of ocular lesions in Behçet’s disease is relatively low in Jordanian patients. This result indi-
cates that early diagnoses and intervention might delay or even prevent vision loss for those patients.

Keywords: Behçet’s disease, Ocular manifestation, Behcet’s complications

Ó 2013 Production and hosting by Elsevier B.V. on behalf of Saudi Ophthalmological Society, King Saud University.
http://dx.doi.org/10.1016/j.sjopt.2013.06.012

Introduction criteria, including recurrent genital ulcers, skin lesions, uveitis
and a positive pathergy test.3
Behçet’s disease is a chronic, recurrent, multisystem, auto- Behcet’s disease has a worldwide distribution, being more
immune disorder of unknown etiology. This disease is charac- common among populations with a higher prevalence of HLA
terized by the triad of oral ulcers, genital ulcers, and ocular B5 and its split, HLA B 51, in the Mediterranean basin, the
lesions. Middle East and Far East.4
In 1937 Dr. Hulusi Behçet, a Turkish Dermatology profes- The etiopathogenesis of the disease is still unknown. It is
sor described the triple symptom complex of recurrent oral thought that in genetically susceptible individuals there is
ulcers, genital ulcers and iritis as a distinct entity. Professor an enhanced nonspecific inflammatory response, evident as
Behçet did not associate the disease with any known etiol- a skin pathergy reaction. Environmental triggers most proba-
ogy, but blamed unknown virus.1 bly microbial antigens, induce an antigen-driven specific
Several sets of diagnostic criteria had been in use until inflammatory response superimposed on this hyperreactivity.
1990 when the International Study Group (ISG) for Behcet’s This leads to endothelial dysfunction and occlusive
disease proposed a new set of criteria.2 The ISG requires vasculopathy that may affect all types and sizes of vessels in
recurrent oral ulcers as a mandatory criterion plus two of four the body.5

Received 3 February 2013; received in revised form 2 May 2013; accepted 17 June 2013; available online 1 July 2013.

Ophthalmology Department, Jordan University Hospital, Amman, Jordan

⇑ Corresponding author. Address: Jordan University Hospital, P.O. Box 13046, Amman 11942, Jordan. Tel.: +962 799060613; fax: +962 6 5353399.
e-mail address: Mohammd_73@yahoo.com (M.A. Abu-Ameerh).

Peer review under responsibility Access this article online:
of Saudi Ophthalmological Society, www.saudiophthaljournal.com
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05 to . This 50–59 1 5. nevertheless.76 .09–1. Table 2.163 patients was reported in the third (44.8 All patients who displayed ocular manifestations (18) were 16–20 1 5.1 Ocular lesions ginal sterile corneal ulcers. The intraocular pressure (IOP) was measured (n = 18) value using an applanation tonometer.7% 33. Abu-Ameerh et al.6 tival ulcers.27 .7–0.24 to .4–0.06 to .6 . anterior uveitis (50%).6 .3 .3 Results Age group (Year) 1.3% population at Jordan University Hospital.05 to .262 .3%).4 .2 Statistical analysis was performed using SPSS version 17.7%).6 .85). chart) .7% 33.000 Descriptive statistics were presented to describe the distribu.002 vascular occlusion (16.06 to .88 years (SD = 13.11). Patients’ mean age was Duration (Year) 1.3 According to Rheumatologist diagnoses at Jordan Univer.19 to .06 to .331 festations are vitritis (55.3% Rheumatology Department and then referred to the Oph.17 .1 .7%) and one fe.163 male (33.17 .52–2. A review of medical profiles re. Glaucoma 1 5.57 . a total of 43 patients were identified with Be. There were 12 males (66.248 M.81 .1 .6 hçet’s disease between January 2002 and July 2009.22 years (SD = 9. slit lamp biomicroscopy and a detailed fundus Variable No % 95% CI P examination.2 patients manifested signs of recurrent conjunctivitis.06 to .7 Female 6 33. conjunc. Patients aged between 1 and 72 years with a mean Optic atrophy 50% (1) 50% (1) age of 36.6%).17 .79 years (SD = 9.2% (7) (2) Vitritis 70% (7) 30% (3) The Ethics board at Jordan University Hospital approved a Retinal vasculities (vascular sheathing) 44. Meanwhile.6 was 10.06 to . vitreous hemorrhage.1 included in this study.57 .163 (77. and reti. 6–10 5 27. filamentary keratitis.3%) with a mean age of 35 years (SD = 17.17 .A. .7% 33.000 males (33.4%) of Behcet’s disease Epiretinal membrane (ERM) 66. Those patients were diagnosed by the Vascular occlusion 66.331 Retinal vasculitis 9 50 .4% 55.6% retrospective chart review for 43 patients diagnosed with (4) (5) Behçet’s disease. Two males (66. CF to PL 2 11. neovascular glaucoma or phthisis bulbi.6%). Neovascularization 1 5. Papileodema 1 5.88 (SD = 13.010 (33.3% 2002 and July 2009.6 .000 detachment.27 .001 retinal detachment. Sex 1. study variables. external ocular manifestations. 40–49 1 5.8 mean duration of the disease was 9. exudative Uveitis 9 50 . BRVO 1 5.06 to .3%) were involved in this manifestation.17 .3746– .331 Moreover.7%) and 6 fe. examination. episcleritis. tractional retinal Vitritis 10 55.8% 22. Macular hole 1 5.99 . Our study aims to identify ocular manifestations and their Table 1.6 60 & More 2 11.6 .27–.05 to .09 to . Male cases accounted for the majority (CMO) (4) (2) with 29 patients representing (67.5 2 11. (2) (1) Multifocal retinitis 100% (2) 0 thalmology Department in the period between January Macular edema especially cystoids type 66. Visual acuity (Snellen . Although higher prevalence was seen Retinal atrophy 1 5.001 nal vasculitis (50%).11) as a mean duration of the Neovascularization elsewhere (NVE) 100% (1) 0 disease. 27 Papillitis 1 5. The Unilateral 5 27.81 . The prevalence of Male versus female patients distributed on severity in Jordanian patients with Behçet’s disease. lower prevalence for male Optic nerve atrophy 2 11.1 Results displayed in Table 2. 30–39 8 44.70 . Papildema 50% (1) 50% (1) ported 10.57–1. Side 1.331 Posterior syneachea 2 11.95). scleritis.30 to .2 4 22. CRVO 1 5. mar. our results report a low prevalence for retinal Cataract 8 44.331 toid macular edema (CMO) and epiretinal membrane (ERM) Cystoids macular edema 6 33.6 +20 2 11.17 .1 .4 sity Hospital.24 to . retinal exudation.26).17 .06 to . 0.4%).8%) respectively.000 36. findings illustrate an equal prevalence for cys.76 . Ocular manifestation % (n) Methods Males Females Extraocular muscle paralysis 0 100% (2) Participants Anterior uvitis 77. the (4) (2) Retinal atrophy 0 100% (1) number of female patients reached 14 with a percentage of Papillitis 0 100% (1) (32.17 . The mean duration of the disease 1–5 10 55.8 9 50 tion of the sample on study variables (Table 1).06 to . Distribution of patients on study variables and ocular ducted including assessment of visual acuity. 0.79 years (SD = 9. Materials Comprehensive ophthalmological evaluations were con.6 . show that none of the 0.6 .1 included 29 males and 14 females.000 19–29 6 33.6 .331 In addition.1 1 5.000 Male 12 66.6 .331 among male cases in the first two manifestations (70%) and Cranial nerve palsy 2 11.85). Bilateral 13 72.7710 1–0. More males than females were affected 4:2 in both.331 Results also suggest that the most common ocular mani.

Moreover. 1). involvement has also been reported in Arab countries such as male cases (100%) manifested extraocular muscle paralysis Morocco (67%)10 and Jordan (60%). While two fe.Ocular manifestations of Behçet’s disease in Jordanian patients 249 Furthermore. A female patient with the first study was 2:1.1%) which indicates a lower value than what was previously re- each. Discussion However.40:1). a hallmark of Be- (referred to as the five nation’s studies) reported the following hçet’s vasculitis may be both leaky and occlusive.7. 0. Studies conducted in Iran. Japan. Cataract was the most frequent complication present Although the international study group of Behçet’s Dis- in 8 (44.34:1. the prevalence was 41.13.11 Finally. Korea and Germany and retinal vasculitis in 50%.63:1 and 1.3%) patients. rioles and capillaries are also involved. however. Different ratios comparing males to females two manifestations versus male patient with the last (NVEs).15 In the current study we found that all af- Behçet’s disease is a systemic vasculitis involving many fected females and 75% of affected males had bilateral uve- organ systems and leading to a wide spectrum of itis.9 tions.1%) patients is the primary consideration in the diagnosis process.4%) patients. China.14 Uveitis is classically defined as a bilateral nongranulomatous panuveitis and retinal vasculitis. followed by cystoid macular edema in ease has emphasized that the presence of recurrent oral ulcer 6 (33. papillitis The male (with Behcet’s disease) to female ratio in our and NVEs (5. Occlusion of retinal Figure 1.15–18 Diffuse vitritis is a constant This paper aims to address the prevalence and ocular feature of the posterior segment involvement.98:1.14 In our sample. Additionally. a single case of retinal atrophy (Fig.6 as a primary manifestation of Behçet’s disease. (a) Left mauclar OCt image shows significant reduction in retinal thickness (160 l) as compared with OCT image (b) of the right eye (254 l) in patient with longstanding intraocular inflammation secondary to Behcet’s disease.9% these four manifestations rated a prevalence of two (11. and two males (100%) displayed signs of multifocal retinitis In the current study. and spontane- manifestations involved in Behçet’s disease in a Jordanian ous resolution is an important diagnostic feature.6%) was reported. ported in Jordan (60%). Research in the medical field has proven that the eye is the Panuveitis and retinal vasculitis (Fig. (35%).12 Oth- and glaucoma in 1 (5. study we reported vitritis in 55.9 A high prevalence of eye manifested optic atrophy and papilloedema. posterior synechiae in 2 (11.6%) patient. results also revealed evidence of complica. Periphlebitis.8 quently reported eye lesions. uveitis may remain unilateral for many years in some patients.19: 1. respectively. respectively. ers argue that uveitis is the initial manifestation of the disease in 10–20% of patients. 0. none of patients were diagnosed with uveitis manifestations. . (69%). 2) were the most fre- most commonly involved vital organ in Behçet’s disease. retinal arte- percentages of ocular manifestations: (55%). were reported in the five nation’s studies (1. an equal percentage of males and females (51%) and (55%). anterior uveitis in 50%.6%. 1.

Behcet H.6%) had NVEs. Sasaki H. a loss was higher in females. or full-thickness macular hole. Sydnor CF. a case of simultaneous had visual acuity of 0. Ohara K. it was reported that the esti- or retinal ischemia induced by uncontrolled intraocular mated risk of losing useful vision after 10 years was 30% in inflammation. Matsuda S. Ohno S. macular ede. hçet’s uveitis are cataract. Über rezidivierende aphthose durch ein Virus verursachte complication which can evolve into cystoid degeneration Geschwure am Mund. sual acuity of 0. leading to blindness with References recurrent attacks. sarcoidosis. and glaucoma.22 Posterior segment involvement is the most serious ocular complication of Behçet’s disease. Abu-Ameerh et al.14 In a Turkish study.6% were found.3%) and macular hole (5. Optic neuropathy is a rare ocular involvement in Behçet’s though the prevalence of male was higher.18 the most common complications of Be.11% for each and glaucoma 6. disease with acute neuro papillitis and 6th nerve palsy.24 The development of partial Wochenschr 1973.23 Macular edema is the most common 1. Ophthalmol Clin North Am beside macular edema 33. cause serious vision loss. a significant vision disease.3%. Sarcoidosis and its ophthalmioc manifestations. (B) shows avascular peripheral retina with perivascular leakage 2ry to peripheral retinal vasculitis in patient with behcet disease.50:297–350. 3). Aizawa M. It was proven that retinal ischemia is responsi. Figure 3.27 In the current study. FFA of the same eye (b) shows typical pattaloid appearance of cystoid macular edema with disk hyperfloresence due to associated papillitis. The same finding was reported in a case of Behcet’s The authors declared that there is no conflict of interest.15:319–26. Surv Ophthalmol 2005.25 In the current study.7% of patients. 5.105:1152–7. ma was reported in (33. Obenauf CD. where 3 out of 6 females had vi- stasis Papilloedema complicating a benign intracranial hyper. Am J Ophthalmol 1978. veins may occur at any location from the central retinal vein Despite modern treatment and intervention strategies. disease in Korea revealed that females have a higher fre- itis.250 M.15. In the current study Ophthalmol 1982. 7 out of the 12 males tension or an ischemic neuropathy.A.4%% posterior 2002. Current concepts in the etiology and treatment of 5.13 In this study we reported retinal the disease still carries a poor visual prognosis with one-quar- vein occlusion in 16. Shaw HE. (A) Colored fundus photograph showing grade 1 vitritis (clear disk and vessels but retinal fiber layer obscured) with retinal vasculitis involving superior temporal branch of retinal vein.8 and higher. may 2. Sarcoidosis and uveitis. Intraocular manifestations of systemic nificant vision loss was also noticed. Jones NP. Meanwhile. Behçet disease.20 it can be related to an inflammatory neuropathy. Sydnor CF. Klintworth GK. ter of the patients being blind. synechiae and optic atrophy 11. though rarely reported. Close association of HLA Bw51 with Behcet’s disease. quency of ocular lesions than males.6%) with sig- 3. optic atrophy. cataract 44. posterior synechiae. macular ede. study on Behçet’s ble for 13% of eyes with NVD associated with Behçet’s uve. Wakisaka A. Okubo A.21 In the current Conflict of interest study one patient had bilateral papillitis and right 6th nerve palsy.86:648–55.36:452–7. to the tiny small branches.26 The disease is more severe Neovascularization of the disc (NVD) or elsewhere (NVE) and the risk of losing useful vision is higher in males than in may develop as a complication of retinal vascular occlusion females.100:1455–8. 4. Dermatol and occasionally macular hole. Evereklioglu C. males and 17% in females. bilateral anterior ischemic optic neuropathy (AION) with Be- hçet’s disease was reported in one study. As reported8. Hirosa S.19 In this study only one patient (5. Figure 2. Ohguchi M. . Arch ma (Fig.1 and less. Jpn J Ophthalmol 1992.14 In contrast. OCT of the left eye (a) shows cystoid macular edema involving the outer plexiform and inner nuclear layers of the sensory retina with small sensory retinal detachment 2ry to viritis. am Auga und an den Genitalien.

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