Behçet‘s Syndrome Apart From the Triple Symptom Complex: Vascular, Neurologic, Gastrointestinal, and Musculoskeletal Manifestations. A Mini Review

Abstract

Behçet‘s Syndrome (BS) is a variable vessel vasculitis according to the Chapel Hill Consensus Nomenclature (1) and may thus affect any organ, including major and minor arterial and venous vessels to a varying degree and with varying frequency. Although the main features of BS are recurrent oral and genital aphthous ulcers, cutaneous lesions, ocular inflammation and arthritis—major vessel and life—or organ threatening involvement of internal organs and the central and peripheral nervous system occur. In general, BS in Europe appears to form six phenotypes of clinical manifestations (2), which are (1) mucocutaneous only, (2) predominant arthritis/articular involvement, (3) vascular phenotype, (4) ocular manifestations, which are most likely associated with CNS manifestations and HLA-B51, (5) dominant parenchymal CNS manifestations (being associated with the ocular ones), and (6) gastrointestinal involvement. Mucocutaneous manifestations are present in almost all patients/all phenotypes. In the following review, we summarize the current knowledge concerning vascular, neurologic, gastrointestinal and musculoskeletal manifestations of the disease.

Case report and analysis: Behçet’s disease with lower extremity vein thrombosis and pseudoaneurysm

“…Vascular involvement is observed in 40% of BD patients and tends to occur in active male patients ( 26 ). Moreover, 75% of vascular events occur within 5 years of BD onset ( 27 ). Veins and arteries of all sizes are affected, although veins are usually more severely affected than arteries.…”

Inflammatory Disorders of the Central Nervous System Vessels: Narrative Review

“…Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by a wide range of dysregulation of the immune system, leading to systemic inflammation and multiple organ damage [ 46 ]. The disease has a wide spectrum of clinical features and affects women more often [ 9 , 37 ]. In 90% of cases, the disease manifests as skin vasculitis, but it can also cause visceral involvement.…”

“…In 90% of cases, the disease manifests as skin vasculitis, but it can also cause visceral involvement. Lupus vasculitis is more likely to occur during active disease and is accompanied by general inflammatory symptoms and laboratory parameters such as increased inflammatory markers, fever, fatigue and weight loss [ 37 ].…”

“…SLE often affects both central and peripheral nervous systems, as a syndrome collectively known as neuropsychiatric lupus erythematosus (NPLE) [ 38 , 47 ]. NPLE is a severe complication of SLE, associated with high mortality, and can occur in approximately 30–50% of patients [ 19 , 36 , 37 , 48 , 49 , 50 ]. The mechanism of the NPLE is still unclear.…”

“…It is thought that a disruption of the blood-brain barrier allows peripheral blood antibodies and immune components to penetrate the CNS, leading to brain inflammation and damage [ 47 ]. The main histopathological finding of NPLE is non-inflammatory microangiopathy associated with cerebral microinfarcts and thrombosis [ 37 ]. Antiphospholipid antibody-induced thrombosis is the second important mechanism of ischemic brain injury.…”