Dr.ARPITHA PEREIRA,Dr.Padmamalini Mahendradas,Dr.Ankush Kawali,Dr.Shetty Bhujang K
Introduction
Behcet’s disease is an autoimmune, rare, and severe multisystemic inflammatory disease characterized by recurrent oral aphthous ulcers, genital ulcers, skin lesions, and both anterior and posterior uveitis.
The pathology is characterized by episodic inflammations which may affect every tissue and organ of the body: joints, gastrointestinal tract, nervous system, and others. Generally, Behcet’s disease is a secondary occlusive systemic vasculitis, which affects both arteries and veins of all sizes and tissue types.
Demographic characteristics, clinical features, and familial occurrence of the disease observed in the groups differ due to environmental and/genetic factors.
Amongst various genetic markers, class I, HLA-B5, and its subclass B51 allele have the highest reported association with Behcet’s disease.
Methods
It was a retrospective case analysis where patients with ocular Behcet’s disease presenting to the Uveitis department at a tertiary eye care center, between the years 2006 to 2017 were included. Information on patients’ gender, age, systemic manifestations, ocular features and various complications, visual acuity, and systemic treatment were analyzed.
Results
The study cohort comprised of 22 patients out of which 15 (68.18 %) were males and 7 (31.82 %) were females. The mean age of the population was 32.13 ± 13.3 years (age range being 4 to 46 years).
Amongst the 22 pts, 13 (59.09 %) subjects had bilateral and 9 (40.9 %) had unilateral ocular disease.
Table 1 depicts the systemic manifestations of Behcet’s disease in the study population. The most common systemic pathology was recurrent oral ulcers followed by genital ulcers and skin lesions.
| Systemic manifestations | n | % |
| Recurrent oral ulcers | 4 | 18.18 |
| Genital ulcers | 3 | 13.63 |
| Skin lesions | 1 | 4.54 |
HLA B 27 was positive in 5 (22.72 %) and 7 (31.82 %) were positive for HLA B 51.
The various ocular manifestations noted in the subjects are listed in Table 2.
| Ocular manifestations | n | % |
| Anterior uveitis | 8 | 36.36 |
| Posterior uveitis | 10 | 45.45 |
| Panuveitis | 5 | 22.72 |
| Intermediate uveitis | 2 | 9.09 |
As shown in the table, posterior uveitis (45.45 %) was the most common ocular manifestation followed by anterior uveitis (36.36 %). Amongst the subjects having posterior uveitis, 5 pts had retinal vasculitis.
Complications of uveitis as a result of Behcet’s disease were as follows: Complicated cataract in 4 cases (18.18 %) and secondary glaucoma in 3 cases (13.63 %). Dry eye and conjunctival ulceration was seen in one case each. One subject developed retinal detachment following posterior uveitis for which vitrectomy was required.
Discussion
Eye is the most common organ involved in Behcet’s diseasewith ocular involvement is seen in about 70% of patients. In most cases, the ocular symptoms follow the oral and genital ulcers by 3-4 years, although ocular disease is the initial manifestation in about 20% of cases.
Ocular Behçet’s disease is characterized by severe recurrent attacks of intraocular inflammation. In a case series of 33 eyes by Torres et al, anterior uveitis was present in 59% of cases; posterior uveitis was present in 76% of cases; and panuveitis was present in 88.1% of cases.
Other systemic manifestations of Behcet’s include recurrent oral or aphthous ulcers, genital and skin lesions.
In a retrospective case series of 76 pts done by Paovic et al, the incidence of different systemic manifestations was: recurrent oral aphthous ulcers 89.5%, genital ulcers 61.8%, articular involvement 72.4% and vasculitis 81.6%. In the same study most frequent ocular manifestations in the examined group were retinal periphlebitis 81.6%, periphlebitis and periarteritis 65%, and serofibrinous uveitis 63.2%. Macular edema as a complication was present in 63.2%.
In our study, the most common systemic manifestation was recurrent oral ulcers 18.18 % followed by genital ulcers 13.63 % and skin lesions 4.54 %.
In terms of HLA typing, 31.82 % were positive for HLA B 51 and 22.72 % were positive for HLA B 27.
Bilateral ocular disease was present in 59.09 % subjects whereas only one eye was involved in 49.9 % of the cases.
In ocular manifestations, posterior uveitis (45.45 %) was the most common followed by anterior uveitis (36.36 %). Amongst the subjects having posterior uveitis, 50 % pts had retinal vasculitis.Panuveitis was diagnosed in 22.72 % cases, while 2 cases had intermediate uveitis.
Complicated cataract (18.18 %) was the most common complication followed by secondary glaucoma (13.63 %). One case each of conjunctival ulceration and dry eye were seen. Also one case developed retinal detachment which needed surgical intervention.
In our case series we also diagnosed the youngest reported case of ocular Behcet’s disease at 4 yrs.
Conclusion
Behcet’s disease is a multisystemic inflammatory disease, the manifestations of which include recurrent painful oral ulcers, genital ulcers, skin lesions and uveitis.
Previously thought to occur at around 20 – 30 yrs of age, Behcet’s disease has now been proved to affect individuals as early as 4 yrs of age as shown in this study.
In our case series, posterior uveitis followed by anterior uveitis was the most common ocular manifestations. It also showed that pts with Behcet’s disease might present with ocular signs independent of systemic manifestations.
Behcet’s disease can lead to irreversible visual loss but visual prognosis is good if the disease is identified early using ocular and systemic signs and genetic phenotyping using HLA B 51 and HLA B 27.
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