Prevalence and Characteristics of Ocular and Systemic Manifestations in Iraqi Patients with Behçet’s Disease

1. INTRODUCTION

Behçet’s disease is a systemic vasculitis most common in individuals of Mediterranean and Eastern origin, though it also affects Caucasians. The etiology remains unknown, but a widely held hypothesis is that a profound inflammatory response is triggered by an infectious agent in a genetically susceptible host. This is supported by the consistent association of disease susceptibility with HLA B51 [1-3].

Diagnostic criteria for Behçet’s disease (International Study Group for Behçet’s Disease) include recurrent oral ulceration (at least three times per year) plus two of the following: genital ulceration, ocular inflammation, skin lesions (e.g., erythema nodosum and acneiform lesions), and a positive pathergy skin test [4].

2. METHODS

Patients diagnosed with Behçet’s disease were evaluated at the outpatient clinic of the Ophthalmology Department at Baghdad Medical City between November 2019 and September 2022. Referrals to this department were made from the dedicated Behçet’s disease clinic at Baghdad Teaching Hospital within the Medical City complex.

Data collection included comprehensive medical history, demographic details (age, gender), anterior segment examination using slit lamp biomicroscopy, posterior segment evaluation via indirect ophthalmoscopy, Snellen visual acuity assessment, intraocular pressure (IOP) measurement using air-puff and Goldmann tonometry, and systemic examination for associated features. All patients underwent HLA-B51 testing and pathergy testing.

The Behçet’s disease clinic at Baghdad Medical City serves as the primary referral center in Iraq for the diagnosis and management of Behçet’s disease. Patients are routinely directed to the Ophthalmology Department within the medical complex for the diagnosis and treatment of ocular involvement, both at the time of initial diagnosis and during subsequent follow-up appointments. This study adhered to the principles outlined in the Declaration of Helsinki. It was conducted after gaining approval from the Ethics Committee on Human Research in Baghdad Medical City (Approval No. 221 on the 10th of March 2019). Additionally, both verbal and signed informed consent forms were obtained from the patients before they participated in the study. All data were kept anonymous, and no divulging of information occurred; it was only for research purposes.

3. RESULTS

A total of 76 patients with Behçet’s disease were included in the study. Oral ulceration was observed in all patients (100%, n=76), making it the most common manifestation. Genital ulceration was present in 56 patients (74%). Ocular involvement was noted in 50 patients (66%). Arthritis affected 37 patients (48%), and skin lesions were seen in 12 patients (16%). Venous involvement was identified in 6 patients (8%), gastrointestinal ulceration in 3 patients (4%), and neurological involvement also in 3 patients (4%). The prevalence of HLA-B51 positivity was found in 41 patients (54%), and the pathergy test was positive in 27 patients (36%) (Table 1).

Among the 50 patients with ocular involvement, bilateral manifestations were observed in 37 patients (74%), while 13 patients (26%) had unilateral involvement. Of the bilateral cases, 19 (38%) were symmetric, and 18 (36%) were asymmetric. Non-granulomatous anterior uveitis was detected in 24 patients (48%). Vitritis was found in 20 patients (40%); vitritis was defined using the Standardization of Uveitis Nomenclature (SUN) guidelines, which grades vitreous haze from 0 (no haze) to +4 (severe haze). Retinal vasculitis was observed in 17 patients (34%) (Fig. 1), retinitis was observed in 4 patients (8%), and hypopyon was observed in 3 patients (6%) (Table 2, Fig. (2)). These findings highlighted the diverse ocular manifestations that can occur in Behçet’s disease.

Regarding gender distribution, 62% of the patients were male (n=47), and 38% were female (n=29), resulting in a male-to-female ratio of approximately 3:2 (Table 3). This distribution indicates a higher prevalence of Behçet’s disease among males in the studied population.

4. DISCUSSION

The male to female ratio in our study was approximately 3:2, aligning well with the gender distribution observed in Eastern Mediterranean populations. This contrasts with Far Eastern Asian populations, where females are more frequently affected [16, 17].

Ocular involvement was observed in 66% of the patients, which falls within the global range of 30-70% and is fairly close to the rate in Turkey (80%) (Tugal-Tutkun et al.) [6]. In comparison, the rate is 65% in the Saudi population and 55% in Iran (El-Asrar et al., Davatchi et al.) [5, 18]. Anterior uveitis, vitritis, and retinal vasculitis appeared at relatively similar rates in this study compared to Turkish figures (45%, 40%, and 27%, respectively) and Iranian statistics (41%, 44%, and 30%, respectively) (Gul, Davatchi et al.) [5, 17]. Hypopyon was observed in 6% of the patients, which is lower than the rates typically reported in other populations. Both retinitis and papillitis were found at much lower rates. The smaller number of patients in this study might have played a role, or this may reflect different rates of ocular manifestations in the populations studied [5, 6, 17, 18].

HLA-B51 was found positive in 54% of patients in this study, compared to 47% in the Turkish population and 33.9% in Iran (Davatchi et al.) [5]. The pathergy test was positive in 36% of patients, falling within the global range of 15-60%, with a rate of 57% in Iran [19, 4].

Arthritis, vascular disease, and genital ulceration were found at rates similar to those reported globally in Turkey, Saudi Arabia, and Iran (Davatchi et al., Kural-Seyahi et al.) [5]. Neurological symptoms were reported in 4% of the cases in this study, while the global rate is 5-10%, 3.2% in Iran, 0.2% in Turkey, and an unusually high 44% in Saudi Arabia (Al-Araji & Kidd, Siva et al.) [20, 21].

Skin lesions were reported in only 16% of patients compared to 80% globally, 69% in Iran, 57% in Saudi Arabia, and 27% in Turkey (Davatchi et al., Gul, Seyahi et al.) [5, 17, 22, 23]. The smaller number of patients in this study likely influenced these discrepancies. Additionally, the fact that this is a hospital-based study might have contributed to the differences observed, or it may reflect different tendencies in the Iraqi population.

A trend was observed where patients with severe systemic symptoms, such as recurrent and extensive genital ulceration, exhibited more severe ocular involvement (e.g., retinal vasculitis and vitritis). However, statistical significance could not be established due to the limited sample size. This observation warrants further investigation in larger cohort studies.

CONCLUSION

Our study sheds light on the diverse spectrum of ocular and systemic manifestations observed in Iraqi patients with Behçet's disease. Notably, we found that non-granulomatous anterior uveitis (48%) and vitritis (40%) emerged as the most prevalent ocular features, underlining the significant burden of ocular involvement in this population.

Moreover, our findings highlighted the predominant systemic manifestations of Behçet's disease among Iraqi patients. Oral ulceration was universally present, observed in 100% of cases, underscoring its diagnostic significance and the immense impact it has on patients' quality of life. Additionally, genital ulceration was prevalent in 74% of patients, further emphasizing the multisystemic nature of the disease.

These insights contribute to a deeper understanding of Behçet's disease in the Iraqi population, providing valuable clinical data that can inform more targeted and effective management strategies. Further research and collaborative efforts are warranted to elucidate the underlying mechanisms driving these manifestations and to optimize therapeutic interventions tailored to the specific needs of Iraqi patients with Behçet's disease.

REFERENCES