O16 A diagnosis of BehÇet’s Disease with secondary antiphospholipid syndrome

Abstract Case report - Introduction Behçet’s disease (BD) is a systemic inflammatory, variable-vessel vasculitis manifesting most commonly with recurrent oral or genital ulcers and uveitis. The heterogeneous presentation may also include skin, joint, gastrointestinal and central nervous system involvement. There is a geographical prevalence in countries along the “Old Silk Road” and a strong association with HLA-B51 antigen. Paediatric-onset BD commonly has an incomplete clinical picture thereby causing a diagnostic challenge. We present the case of a 10-year-old boy of Lithuanian origin whose history, imaging and results led to a rapid diagnosis which is unusual in the paediatric population. Case report - Case description The patient presented with a 1-month history of abdominal pain, distension and a cough that did not respond to oral antibiotics or inhalers from his GP. His mother recalled swollen proximal interphalangeal joints on both hands, a rash and red eyes approximately 4 days earlier which had now resolved. She also reported mouth ulcers occurring at least once a month from a young age and cold sores without any genital or anal ulcers. He also had a history of reactive arthritis at the age of 3 with bilateral knee swelling and anaemia. He was afebrile, tachycardic with respiratory distress and an oxygen requirement to maintain saturations over 94%. Chest X-ray showed large bilateral pleural effusions and cardiomegaly. Echocardiography confirmed a large pericardial effusion with signs suggestive of tamponade physiology. After admission to PICU he was intubated and sedated to facilitate pericardial and pleural drain insertion. Post-procedure he was haemodynamically stable. Empirical antibiotic therapy was commenced with IV ceftriaxone. Investigations showed a CRP 58mg/L, ESR 37mm/hr, LDH 325U/L, Haemoglobin 98g/L, platelets 223x109 and a blood film showed non-specific changes. All microbiology samples were negative including T spot, mantoux and broncho-alveolar lavage. Serology included negative Anti-Nuclear Antibody, Extractable Nuclear Antigen, double stranded-DNA, and Rheumatoid Factor with normal complements, immunoglobulins and serum amyloid. HLA B51 was reported as positive. Faecal calprotectin was high at 539µg/g Antiphospholipid antibody screen was positive (anticardiolipin antibody 68U/mL, Beta2-glycoprotein 111U/mL). CT demonstrated multiple pulmonary emboli (PE), splenomegaly and moderate volume ascites. Ophthalmology assessment was un