Author: Hala El Belidi (Morocco)
Co-authors: Yahya Saoiabi, Lalla Ouafae Cherkaoui
Purpose
behçet desease is an autoimmune rare disease, classified as vasculitis. It is a desease with multisystemic involvement characterised by recurrent oral aphthous ulcers, genital ulcers, skin lesions, and both anterior and posterior uveitis . Individual patients develop all of the symptoms with various combinations and that is why the treatment should be customised depending on the clinical manifestations. Ocular manifestations have a prevalence that varies between 50–70%, can lead to sight-threatening complications and are characterised by recurrent attacks of ocular inflammations involving the eye from the anterior to the posterior segment . The risk of blindness, mainly due to macular involvement or retinal vasculitis, increases progressively reaching 25% at 10 years and remains constant thereafter . we report the case of a subhyaloid haemorrhage as first presentation of behçet desease .
Setting/Venue
HOPITAL DES SPECIALITES . RABAT . MOROCCO
Methods
we report a case of a 30-year-old male presented with overnight onset loss of vision in his right eye . Best corrected visual acuity was 6/6 in the unaffected left eye and hand movements in the right eye. Intraocular pressures were 19 mm Hg bilaterally. Fundus examination revealed a large subhyaloid haemorrhage with a pre-retinal neovascularization in infero-nasal quadrant and inferior retinal occlusive vasculitis ; The left eye exam was normal . After deep history taking he reported recurrent oral ulcers , genital ulcers and an erythema nodosum 3 months before . Physical examination showed genital scarring. Biologically he had an inflammatory syndrome .The patient was treated with Nd:YAG laser hyaloidotomy, achieving immediate drainage with 15 shots at 4 mJ. One-week review demonstrated dispersion of haemoglobin throughout the vitreous, though this resolved within one week with best corrected visual acuity returning to 6/15. Complete resolution occurred by eight weeks. behçet desease was diagnosed according to the International Study Group for Behçet’s Disease Criteria.
Results
In recent years much diagnostic and therapeutic progress has been made, clinical manifestations and presentation of behçet desease slightly changed. Ocular inflammation in behçet desease can be the first manifestation in 20% of cases and can involve all the uveal tract; for this reason, uveitis can be anterior (11%), posterior (28.8%) or panuveitis (60.2%) . The eye may be involved 2 to 3 years after the beginning of the extraocular signs . Initially, the involvement is unilateral with a remitting-relapsing course, becoming then bilateral. Usually, the posterior segment of the eye is the main site of inflammation. Uveitis seem negatively associated with genital ulcers , gastrointestinal involvement , pseudofolliculitis and central nervous system signs , vascular involvement and erythema nodosum. Late onset behçet desease, despite a relatively mild course, could be associated with a higher rate of blindness (10.5%) as recently reported by Saadouli et al. Our case shows sub-hyaloid hemorrhage following bleeding from the pre-retinal neovascularization in patient with occlusive retinal vasculitis ;To the best of our knowledge, this is the first report of subhyaloid hemorrhage revealing a Behçet's disease.
Conlusions
Behçet's disease is a chronic disorder, characterized by a relapsing and remitting course. Male patients with younger age at onset and severe lesions at presentation are at higher risk of severe visual loss over time. The main goals in the management of patients with behçet's disease-associated uveitis are rapid suppression of intraocular inflammation, preservation of vision, and prevention of recurrences. The treatment is based on the use of systemic glucocorticosteroids and immunosuppressive agents. Posterior segment involvement requires the use of corticosteroids and immunosuppressants, primarily azathioprine. This treatment does not appear to be sufficient for severe uveitis with reduced visual acuity or retinal vasculitis that requires anti-TNF α or interferon α. Additionally,subhyaloid hemorrhage can be easily and safely treatedusing Nd:YAG laser with a rapid visual recovery.
Financial Disclosure
We have no financial interest to disclose
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