Behçet’s syndrome: providing integrated care

Abstract

Behçet’s syndrome (BS) is a multisystem vasculitis that presents with a variety of mucocutaneous manifestations such as oral and genital ulcers, papulopustular lesions and erythema nodosum as well as ocular, vascular, gastrointestinal and nervous system involvement. Although it occurs worldwide, it is especially prevalent in the Far East and around the Mediterranean Sea. Male gender and younger age at disease onset are associated with a more severe disease course. The management of BS depends on the severity of symptoms. If untreated, morbidity and mortality are considerably high in patients with major organ involvement. Multidisciplinary patient care is essential for the management of BS, as it is for other multisystem diseases. Rheumatologists, dermatologists, ophthalmologists, neurologists, cardiovascular surgeons and gastroenterologists are members of the multidisciplinary team. In this study, we reviewed the epidemiology, etiology, diagnostic criteria sets, clinical findings and treatment of BS and highlighted the importance of the multidisciplinary team in the management of BS.

Multiorgan involvement of Behçet’s disease in a young woman

“…Behçet's disease is a systemic vasculitic disorder that is characterized by recurrent oral and genital ulcers, uveitis, skin lesions such as papulopustular lesions, erythema nodosum, and ocular, vascular, gastrointestinal, and nervous system complications (2,3). The condition was recognized by Hippocrates in the fifth century BC and Adamantiades in 1931.…”

Neurobehçet in a Young Adult in Lima, Peru: Case Report

“…Estas lesiones que pueden ser parenquimales (afección de vasos que irrigan zonas focales) y no parenquimales (trombosis de seno cavernoso, pseudotumor cerebri, mononeuritis, mielitis,etc. ), pueden ser controlables y hasta reversibles clínicamente si reciben un tratamiento oportuno y sin extensión ni compromiso permanente, lo cual es consistente con los hallazgos y evolución en nuestro paciente 5,6,14 .…”

“…Se creía que existía una relación entre las manifestaciones sistémicas de la enfermedad y su pronóstico desfavorable, siendo las expresiones clínicas en parénquima cerebral, trombosis venosa en vasos cerebrales, aneurismas de arteria pulmonar y uveítis anterior o posterior, las más peligrosas al causar limitaciones en calidad de vida y esperanza de vida 6 . No obstante, los hallazgos encontrados recientemente sobre el manejo terapéutico oportuno y la reducción de progresión de la enfermedad y de exacerbaciones han sido significativos para la mejoría clínica de los pacientes 10 .…”

Trisomy-8-Positive Hematologic Malignancies Associated with Intestinal Behçet’s Syndrome: Keep This Entity in Mind

“…The prevalence of gastrointestinal tract involvement varies from 3 to 25% and has a distinct geographical variation. There are typically ulcerations in the ileocecal area [1]. Myelodysplastic syndromes (MDS) are clonal hematopoietic stem cell disorders presenting with ineffective hematopoiesis, increased bone marrow cellularity, myeloid lineage dysplasia, and peripheral cytopenia with an increased risk of developing acute myeloid leukemia.…”