Year : 2021 | Volume
: 14 | Issue : 3 | Page : 131--133
An overview of ophthalmic manifestations and prognostic factors of Behcet's disease in Omani patients
Nadiya Al Kharousi, Washoo Mal
Department of Ophthalmology at Sultan Qaboos University Hospital, Muscat, Sultanate of Oman
Dr. Washoo Mal
Department of Ophthalmology, Sultan Qaboos University Hospital, P.O. Box 38 Al-Khod, P C 123, Muscat
Sultanate of Oman
|How to cite this article:|
Al Kharousi N, Mal W. An overview of ophthalmic manifestations and prognostic factors of Behcet's disease in Omani patients.Oman J Ophthalmol 2021;14:131-133
|How to cite this URL:|
Al Kharousi N, Mal W. An overview of ophthalmic manifestations and prognostic factors of Behcet's disease in Omani patients. Oman J Ophthalmol [serial online] 2021 [cited 2021 Dec 8 ];14:131-133
Available from: https://www.ojoonline.org/text.asp?2021/14/3/131/328605
Behçet's disease (BD) is a chronic relapsing multisystem inflammatory disorder characterized by recurrent episodes of oral aphthous ulcers, genital ulcers, and lesions involving skin, eyes, vascular, and other organ systems.
BD generally appears in the third or fourth decade of life and rarely affects children or people over fifty. Gender prevalence varies in different countries, higher male prevalence in the Middle-East region and female predominance reported in studies from the Western territories.
BD is observed worldwide but more prevalent in countries along the ancient “Silk Road” from Japan to the Middle East. In the Mediterranean region, the prevalence has been reported as high as 14–20/100,000, with the highest prevalence reported in Northern Turkey (420/100,000. The disease was named after Turkish Dermatologist Dr. Hulusi Behçet, who elaborated the features of BD and highlighted the triad of recurrent painful oral aphthous ulcers, genital ulcers, and uveitis in 1937.
The exact etiology of BD is unknown. It is presumed to be multifactorial and caused by an autoimmune response triggered by environmental factors, such as infectious agents (various bacteria and viruses) and immunological factors. Genetic susceptibility, particularly major histocompatibility complex antigen HLA B51 thought to play a significant role. However, BD is considered sporadic, although varying frequencies of patients with a positive family history for BD have been reported.
Clinical manifestations encompass multiple organ systems, including the ocular, mucocutaneous, vascular, central nervous, gastrointestinal, cardiopulmonary, and musculoskeletal systems. Vasculitis is the key feature of BD. BD is unique among vasculitides that it tends to affect any of the small, medium, or large vessels. The most common presenting symptoms of the disease are mucocutaneous manifestations, while the most severe and debilitating are uveitis, large-vessel vasculitis leads to thrombosis, and neurological involvement.
More than 70% of patients with BD have ocular involvement. Eye involvement is usually not the presenting feature of BD but usually occurs within the initial few years of diagnosis and is rare to occur late in the disease. Young males are affected more than elderly females. The classic finding is relapsing, chronic, bilateral nongranulomatous iridocyclitis with a cold hypopyon that moves or shifts with head tilt due to low content of fibrin and posterior uveitis with retinal occlusive vasculitis. The majority of affected patients develop panuveitis. Posterior uveitis with consequent macular edema, occlusive retinal vasculitis, papillitis, or neovascular glaucoma leads to blindness in approximately 25% of patients with BD. Keratitis, scleritis, episcleritis, conjunctivitis, optic neuritis, and optic atrophy are rare manifestations. A detailed ophthalmic examination is mandatory in patients with BD, which involves a comprehensive assessment of the posterior segment.
Diagnosis is usually clinical and confirmation can be difficult as no single specific laboratory test exists that is pathognomonic to BD. However, “The International Study Group Behcet's Disease” criteria are widely used to diagnose the condition that includes ocular lesions, oral aphthous ulcerations, and genital aphthous ulcerations. Each of these is assigned 2 points, while skin lesions, central nervous system involvement, and vascular manifestations are assigned 1 point each. The pathergy test (papulopustular lesion >2 mm in 24–48 h after needle prick), if performed, is assigned 1 point. A patient scoring ≥4 points is classified as having BD. Supportive laboratory tests are carried out to monitor the disease activity, progression, and to rule out other conditions.
The management of BD is individualized because of the variable natural course and requires a multidisciplinary approach (ophthalmology, rheumatology, dermatology, neurology, and internal medicine). The main focus is suppressing the active phase and recurrent inflammatory attacks to prevent irreversible organ damage. Corticosteroids are very effective in short-term control of acute disease with immunomodulatory therapy (IMT) resorted to overcome active stage, avoid relapse, and maintain remission.,
The 2018 European League Against Rheumatism recommendations advised the use of systemic steroids along with azathioprine as first-line treatment. Other immunosuppressive agents such as mycophenolate mofetil, cyclosporin-A, interferon-alpha 2b, monoclonal anti-TNF antibodies, infliximab, adalimumab, or tacrolimus can be considered for severe or refractory disease. Colchicine with steroids or in combination with other IMT is advisable for arthritis and cutaneous lesions. In contrast, the advent of some novel agents such as apremilast, anti-interleukins, etanercept, and tocilizumab is used for other systemic manifestations.
Surgical intervention may be indicated to manage ocular complications such as glaucoma, cataract, vitreous hemorrhage, epiretinal membrane, and tractional retinal detachment. Surgery is also carried out to manage fistulas, intestinal stenosis, perforation, and pulmonary artery aneurysms.
BD has no definitive cure and has waxing and waning course, no matter whichever treatment is administered. Prognostic factors of visual outcome depending on age, gender, ethnicity, the severity of ocular involvement, onset and presentation stage, especially posterior uveitis with vasculitis. Association with HLA B51 positivity and other system involvement carries a bad prognosis. Compliance with medication and control of inflammation impacts the outcome. Despite the availability of a wide range of potent anti-inflammatory and biological blockers, ocular BD is associated with a high degree of morbidity and sight-threatening complications. The overall mortality due to BD reaches 5% at 10 years, probably due to large-vessel disease, especially pulmonary artery aneurysms, parenchymal CNS disease, stroke, chronic renal failure, ischemic heart disease, and congestive heart failure. Early diagnosis and treatment are crucial and can assist to reduce the morbidity and mortality associated with BD. Some case reports of ocular Behcet's as well as case series on extraocular manifestations have been published from Oman region. However, there are no studies describing the characteristic ophthalmic features of BD in Omani patients. Previous studies have shown that ethnicity and environmental and genetic factors influence BD prevalence, distribution, and characteristics.
The Sultan Qaboos University Hospital (SQUH) is an academic and tertiary referral hospital in Oman that includes an Ophthalmology Department with a uveitis clinic and provides multidisciplinary care for patients with BD and other uveitides. A recently conducted study on BD patients presenting to the uveitis clinic in SQUH is in the reporting stage. This first ocular BD study from Oman is expected to pave the way toward a better understanding of epidemiological factors, clinical characterization, and factors affecting the outcome of BD and ocular involvement in the country.
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