117,833 research outputs found
Paraproteinemias Associated with Autoimmune Diseases
No full textParaproteinemia is a frequent laboratory feature in the course of many organ specific or systemic autoimmune diseases. It can be persistent or transient. Autoimmune diseases are secondary to chronic activation of the immune system by antigenic stimuli on a genetic predisposing background. Various pathogens, drugs, and toxins have been implicated as putative etiological agents. Due to hyperactivity of the immune system, individuals with autoimmune disorders may, in occasional cases, be predisposed to the development of paraproteinemia. In this context, paraproteinemia is more likely linked to the biological activity of the underlying disease rather than to an overt neoplastic disorder. Thus, there is growing interest also in studying the role of free light chains (FLCs) as biomarkers of disease activity with the aim of disclosing their exact biological role and their potential use in clinical routine. Moreover, secondary amyloidosis due to chronic inflammation is a well-known late manifestation of several systemic autoimmune disorders. Targeting the pathogenic mechanisms of the disease or the downstream inflammation is the best approach to avoid this complication
Paraproteinemia in Autoinflammatory Diseases
No full textAutoinflammatory diseases represent clinical entities characterized by recurrent episodes of systemic and organ-specific inflammation determined by the primary deregulation of the innate immune system. In addition to monogenic diseases, caused by gene mutations of specific genes involved in the innate immunity, other disorders have been recently classified as multifactorial autoinflammatory diseases on the basis of clinical, pathogenic, and treatment features shared with the monogenic entities. Both in relation to monogenic and multifactorial diseases, a specific chapter referring to autoinflammatory disorders associated with paraproteinemia and other bone marrow disorders is emerging. Considering that many of the innate immunity cells involved in autoinflammatory diseases pass through the bone marrow at some point in their life lends support to this concept. Therefore, recent scientific research is trying to identify bone marrow disorders as a possible cause of autoinflammatory diseases. This would probably allow a considerable diagnostic improvement for adult patients with acquired autoinflammatory conditions currently classified as undifferentiated. Among autoinflammatory diseases related to paraproteinemia and bone marrow disorders, particular attention has to be paid to Schnitzler’s syndrome, but also to Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes (POEMS) syndrome, and other recently described clinical entities, especially VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome, which represents an intriguing bridgehead for future scientific research on the field of bone marrow-associated autoinflammatory diseases
Rheumatologists at a crossroads: blocking tumour necrosis factor or interleukin 6 in disease-modifying anti-rheumatic drug inadequate responder patients with rheumatoid arthritis
No full textAnswer to Vieira et al. “Cytokine profile as a prognostic tool in coronavirus disease 2019”. Joint Bone Spine 2020. Doi:10.1016/j.jbspin.2020.09.006
No full textThe paradigm shift in ANCA-Associated vasculitis: Prime time for the complement targeting
No full textRecent updates in the diagnosis and management of cryoglobulinemic vasculitis
No full textIntroduction: Cryoglobulinemic vasculitis (CV), also known as mixed cryoglobulinemic syndrome (MCS), is a systemic vasculitis that affects small blood vessels. It exhibits a wide range of clinical manifestations, making its treatment a continuing challenge for physicians. Areas covered: We conducted a comprehensive review to evaluate the current status of diagnosis, management, and treatment of mixed cryoglobulinemia (MC). The accurate clinical and serological evaluation plays a vital role in diagnosing MC, identifying potential comorbidities, and monitoring its main manifestations and complications. Treatment strategies should be individualized based on the underlying etiopathogenesis, the severity of organ involvement, and the associated underlying disease. At present, the two mainstays of CV treatment are direct antiviral agents (for HCV-related CV) and B-cell-targeted therapy. Expert opinion: MC remains one of the few autoimmune diseases where the etiology is known, at least for the majority of patients. Its pathogenetic mechanism offers a unique opportunity to investigate the interplay between infections and the immune system. Moving forward, the primary challenge will continue to lie in the treatment of resistant or refractory cases of CV, particularly those associated with autoimmune diseases, or cases classified as ‘essential’ CV
Cryoglobulinemia
No full textCryoglobulins are immunoglobulins that precipitate in vitro at temperatures below 37 ̊C. Cryoglobulin-associated disease is heterogeneous, as not all patients present with it, includes various syndromic presentations (vasculitis is the most common, hyperviscosity syndrome is more exceptional), and can be associated with acute clinical pictures with high mortality. Until the appearance of specific antiviral treatments, the main aetiology has been chronic HCV infection, and currently it is mainly associated with systemic autoimmune diseases, malignant neoplasms and cases with no identified aetiology (essential cryoglobulinemia). Treatment should be modulated according to the predominant etiopathogenesis (vasculitis or hyperviscosity), the severity of internal organ involvement and, especially, the associated underlying disease. Due to the complex aetiological, clinical and pathological scenario of cryoglobulinaemia, early recognition of the most common clinical presentations, a comprehensive clinical assessment of the different organs that may be affected, and multidisciplinary work led by a unit specialised in systemic autoimmune diseases is essential
An immersive virtual reality exergame as a patient education approach in fibromyalgia: Pilot study
No full textBackground Immersive Virtual Reality (VR) has been applied in pain management for various conditions, but its use in fibromyalgia (FM) remains underexplored. While physical activity plays a role in treating FM, patients’ low tolerance often limits its effectiveness. After reviewing the literature on VR and games for FM, we designed a novel VR exergame to assist FM patients in performing physical activity, and evaluate its feasibility. Materials and Methods This pilot study involved three female subjects with FM and four healthy female volunteers. The main outcomes included qualitative assessments of exertion, pain levels, psychological states experienced during the VR session, but also device comfort. Results Improvements in perceived exertion and pain intensity were observed during the VR exergame session in comparison to pre-exergame levels, along with a reduction in depression, stress and anxiety levels while using the VR immersive system. Most participants experienced also increase of relaxation and positive emotions during the exergame. Only one participant was not able to complete all levels of the exergame due to musculoskeletal pain exacerbation; nevertheless, this patient reported an improvement in motivation and enjoyment during the gameplay. Many participants expressed a greater motivation to perform the exercises in the VR environment compared to traditional training methods. Conclusion The proposed VR exergame is a feasible system that might reduce depression, stress and anxiety, while boosting motivation and relaxation in both healthy and FM subjects. A calibration protocol is required to tailor the system to each user's pain levels and physical abilities
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