A CLINICAL OBSERVATION OF BEHÇET'S DISEASE IN A 36-YEAR-OLD FEMALE PATIENT

Abstract

Introduction. Behçet’s disease (BD) is a systemic vasculitis of unknown etiology, characterized by involvement of vessels of any type and caliber, and manifested by recurrent ulcerative lesions in the oral cavity and genital area, as well as ocular, joint, gastrointestinal (GI), central nervous system (CNS), and other organ involvement. The disease follows a chronic course with unpredictable exacerbations and remissions. Environmental and genetic factors may contribute to the etiopathogenesis, indicating both autoimmune and autoinflammatory mechanisms in BD. The variability of clinical presentation and absence of pathognomonic laboratory markers make early diagnosis challenging. Establishing the diagnosis requires a multidisciplinary approach, differential diagnosis, and the development of a comprehensive treatment strategy. In recent years, treatment of BD has expanded from symptomatic management to the use of biological diseasemodifying anti-rheumatic drugs (bDMARDs), addressing the full spectrum of clinical manifestations. Objective. To present a clinical case of Behçet’s disease in a 36-year-old woman and evaluate the efficacy of bDMARDs. Results. This article describes a clinical case of a 36-year-old woman who initially presented with oral ulcers. Subsequently, she developed left-sided inguinal lymphadenitis and genital ulcers that spontaneously ruptured, accompanied by pain and discomfort. Two years later, in an inpatient setting, the diagnosis of Behçet’s disease was confirmed according to the ICBD classification criteria. The manifestations included recurrent aphthous stomatitis, recurrent genital ulcers, and cervical lymphadenitis. The patient was treated with immunosuppressive agents, glucocorticosteroids (GCS), and colchicine at 1g per day. Due to resistance to the previous treatments, bDMARDs with infliximab at 5 mg/kg (300 mL) was initiated. Following combination therapy including infliximab, the patient reported complete resolution of stomatitis and genital ulcers. Any recurrent oral or genital lesions were rare, small, painless, and resolved quickly. Symptoms of lymphadenopathy, joint syndrome, and myalgia also completely resolved. Conclusions. For timely and accurate diagnosis of BD, clinicians should rely on the ICBD classification criteria (EULAR 2018). In cases of resistance to standard therapy, bDMARDs is indicated.