Internal and emergency medicine
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Review Historical Article
From sadness to stiffness: the spleen's progress.
The spleen is a lymphoid organ that has been poorly studied compared to other solid organs, probably because it has been considered a useless and unnecessary part of the body. For many centuries it has been considered a mysterious organ with uncertain functions. The first descriptions of the spleen date back to ancient ages. ⋯ The study of hyposplenic states, which occur much more commonly than originally thought in many immune-mediated disorders, has rapidly increased after the validation of a simple method for assessing spleen function, namely pitted red cell count. In recent years, spleen morphology, in particular spleen stiffness, has been proposed as a marker of portal hypertension. In this paper, we retrace the fundamental steps of the discovery of the functions of the spleen.
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Behçet's syndrome (BS) is a complex disease that shows important heterogeneity in clinical findings and physiopathology. Its treatment can be problematic as BS manifestations in different organs may respond differently to the same drug. ⋯ Tumor necrosis factor alpha inhibitors, especially infliximab and adalimumab, are increasingly used for various refractory BS manifestations despite the lack of controlled studies. In this review, we aim to focus on both the traditional and new treatment modalities for BS, with more emphasis on recent data on newer agents.
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Behçet's syndrome (BS) is a complex vasculitis, characterised by peculiar histological, pathogenetic and clinical features. Superficial venous thrombosis (SVT) and deep vein thrombosis (DVT) are the most frequent vascular involvements, affecting altogether 15-40% of BS patients. Atypical thrombosis is also an important clinical feature of BS, involving the vascular districts of the inferior and superior vena cava, suprahepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses and right ventricle. ⋯ More recently, the anti-TNF-α drugs have also been reported as a valid alternative for the treatment also of venous events, especially DVT. An exception to the use of anticoagulant in BS could be represented by cerebral veins thrombosis. In this review, we will depict the main characteristics of the vascular involvement in BS, briefly describing histological and pathogenetic features, while focusing on the clinical and therapeutical approaches of the vascular manifestations of BS.
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Environmental and other triggering factors are suggested to cause the onset and the clinical relapses of Behçet's syndrome (BS), a multi-systemic inflammatory disorder. In this review, environmental factors are discussed according to their interactions with etiopathogenesis, immune response and disease activity. Stress is a common self-triggering factor for most BS patients. ⋯ Infections are associated with BS, and microbial stimuli can activate inflammation in mucosal surfaces with increased Th1/Th17 responses. Fecal and oral microbiome patterns change in diversity and composition in BS. Better oral hygiene applications and anti-microbial interventions might be helpful to suppress oral ulcers in BS.
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Behçet syndrome (BS) is a multi-systemic complex disorder with unknown etiology and a unique geographic distribution. It could not be possible to include it into specific classification schemes and it is certainly not a uniform disease. Several cluster and association studies revealed that it has been composed of multiple phenotypes ascribing the principal problem such as skin-mucosa, joint, eye, vascular, neurological and gastrointestinal involvement. ⋯ In this setting, BS resembles rather a construction made of several dynamic and interactive LEGO pieces of different shapes and colors. These pieces presenting phenotypes with their own disease mechanism have presumably different genetic determinants. The analysis of phenotyping could help us to identify this disorder and hence could contribute to find better ways of treatment.