regardless of the gender [50]. The frequency and severity of the attacks tend to diminish as the patients get older.

The role of HLA-B51 positivity and a positive family history on the prognosis of Behçet’s disease is uncertain. While some studies found poorer prognosis in HLA-B5-positive patients [7], in other studies, neither HLA-B51 positivity nor a positive family history was found to be significant [50, 51].

Juvenile Behçet’s Disease

The diagnosis of Behçet’s disease in children may be challenging due to the long interval before the onset of enough manifestations to satisfy the diagnostic criteria. The prevalence of juvenile Behçet’s disease and the rate of ocular involvement range widely in published studies.

The clinical spectrum is similar to that of adults; however, the prevalence of certain manifestations varies: less genital ulceration, less vascular thrombosis, more gastrointestinal signs and symptoms, and more arthralgia have been observed in juvenile Behçet’s disease. Perianal aphthosis, reported in 7% of patients in one study, was suggested to be a specific feature of juvenile Behçet’s disease [52]. Posterior uveitis was the most common ocular manifestation, detected in about 75% of the cases [53].

The frequency and ocular prognosis of juvenile Behçet’s disease compared to adult cases vary among the studies. While some studies found ocular complications to be less frequent in juvenile cases [54, 55], others found the contrary [16]. There are also conflicting reports on the ocular prognosis in juvenile patients. Some studies report it to be better [16, 55, 56], whereas in an international collaborative study of 86 cases of childhood Behçet’s disease, uveitis was reported to have a very severe course, especially in male patients [52].

Common features of childhood Behçet’s disease reported by the majority of published studies are onset of uveitis in late childhood, occurrence of oral ulcers as the initial manifestation of the disease in the majority of patients, a

high rate of family history, and male predominance [17].

Pregnancy and Behçet’s Disease

The influence of pregnancy on the clinical course of Behçet’s disease is quite variable between patients and even during different pregnancies in the same patient [57]. In patients with exacerbation of the disease during pregnancy, it most commonly occurs during the first trimester [58]. A study that reviewed 31 Behçet’s patients who had 135 pregnancies found that remissions were significantly more frequent during both pregnancy and postpartum periods, while exacerbations were observed only in one-sixth of the patients. However, pregnancy complications, cesarean section, and miscarriage rates were significantly higher in the study group [59].

Differential Diagnosis

The differential diagnosis varies according to the systems involved. In the presence of uveitis, the key to diagnosis is to identify systemic manifestations such as oral and genital ulcers and skin lesions. Recurrent oral aphtha is present in the vast majority of Behçet’s patients, and its

Table 12.3 Differential diagnosis of ocular Behçet’s disease

Ankylosing spondylitis

Reiter’s syndrome

Inflammatory bowel disease

Crohn’s disease

Ulcerative colitis

Whipple’s disease

Sarcoidosis

Polyarteritis nodosa

Vogt-Koyanagi-Harada syndrome

Eales’ disease

Infectious diseases

Syphilis

Lyme disease

Tuberculosis

Acute retinal necrosis