Lessons of the month 2: A case of Behçet’s disease: 70% have ophthalmic involvement

Abstract

A 34-year-old man presented to the emergency department with acute painless loss of vision of the left eye. Past medical history included painful lumps in the legs and frequent mouth ulcers, which were undiagnosed. The patient's visual acuity was 6/5 and counting fingers in the right and left eye, respectively. There were extensive intraretinal haemorrhages and venous sheathing in the superior quadrant of the left eye with associated disc oedema. The case was discussed in a multidisciplinary team meeting in the presence of ophthalmology, dermatology and immunology and a diagnosis of Behçet's disease was reached. The patient was commenced on intravenous methylprednisolone for 3 days followed by a switch to oral prednisolone. Due to recalcitrant uveitis, an intravitreal dexamethasone implant was administered. Eventually, systemic azathioprine and infliximab were commenced with frequent review by ophthalmology and immunology. The macular oedema improved but, unfortunately, the patient's visual acuity did not recover. Behçet's disease is a complex vasculitis involving multiple organ systems. Ocular manifestations can occur in 70% of patients, comprising retinal vasculitis, anterior uveitis, iridocyclitis, chorioretinitis, scleritis, keratitis, vitreous haemorrhage, optic neuritis, conjunctivitis, retinal vein occlusion and retinal neovascularisation. A tailored multidisciplinary approach is required, with corticosteroids being the mainstay of treatment.

Epidemiologie okulärer Manifestationen bei Autoimmunkrankheiten

“…Morbus Behçet ist gekennzeichnet durch eine chronische Vaskulitis mehrerer Organe, einschließlich der Augen und des Nervensystems. Morbus Behçet weist eine hohe Inzidenz von okulären Manifestationen auf, wobei bei 70% der Patienten irgendeine Form der Augenerkrankung festgestellt wird [25]. Bei Morbus Behçet sind die Augensymptome in der Regel nicht die ersten Symptome, die sich bemerkbar machen, ihre Bedeutung darf jedoch nicht unterschätzt werden.…”

“…Bei einem männlichen Patienten, der neben einem Makulaödem und einem Bandscheibenödem eine verminderte Sehschärfe und intraretinale Blutungen aufwies, wurde ein Versuch mit systemischen Kortikosteroiden, Immunsuppressiva und einem intravi­trealen Dexamethason-Implantat durchgeführt. Obwohl sich das Makulaödem zurückbildete, blieb eine verminderte Sehschärfe zurück, was die irreversiblen Auswirkungen der Behçet-assoziierten Uveitis verdeutlicht [25]. In einer Studie trat bei über der Hälfte der Patienten ein Makulaödem auf.…”

Epidemiology of Ocular Manifestations in Autoimmune Disease

“…Bechet’s Disease is characterised by chronic vasculitis of several organs, including the eyes and nervous system. Bechet’s disease has a high incidence of ocular manifestations, with 70% of patients haven being found to show some form of the ocular disorder ( 25 ). With Bechet’s disease, ocular symptoms are generally not the first to manifest, however, their significance cannot be underestimated.…”

“…One male patient who presented decreased visual acuity and intraretinal haemorrhages alongside macular oedema and disc oedema was given a trial of systemic corticosteroids, immunosuppressants and a dexamethasone intravitreal implant. Although macular oedema resolved, decreased visual acuity remained, conveying the irreversible effects of Bechet’s disease-associated uveitis ( 25 ). In one study, macular oedema occurred in over half of patients.…”

“…With Bechet’s disease, the highest prevalence is found in the Mediterranean basin ( 25 ). In Turkey, the estimated prevalence is 421 per 100,000 population ( 26 ).…”