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Spondyloarthritides (SpA) are a group of interrelated rheumatic disorders that includes ankylosing spondylitis (AS), psoriatic arthritis (PsA), arthritis related to inflammatory bowel disease and reactive arthritis. Since the latest classification criteria published from the ASAS (Assessment of SpondyloArthritis international Society), patients with these diagnoses can be classified either as having axial or peripheral SpA. In this study, these new criteria of ASAS will be applied to all patients with a clinical diagnosis of SpA that are followed in the Rheumatology Clinic of University Hospital of Heraklion. Furthermore, patients with non-radiographic axial SpA (nrAxSpA) will be monitored, both retrospectively and prospectively, for their long-term outcome in terms of imaging and clinical aspects (remission, disability, severe complications, eg, uveitis). This study is expected to give valuable information of the performance of these new criteria in daily clinical practice and of the prognosis of patients with non-radiographic axial SpA. © 2019 The Mediterranean Journal of Rheumatology (MJR).Background Systemic Sclerosis (SSc) is a rare, multisystemic connective tissue disease associated with significant morbidity. Early recognition of patients at risk for adverse prognosis may help towards optimized monitoring and treatment, thus improving disease outcome. Objective To correlate nailfold videocapillaroscopy (NVC) findings ('early', 'active', 'late' scleroderma patterns and non-specific capillary abnormalities) with major organ involvement and prognosis in patients with systemic sclerosis (SSc). Methods Patients from the Scleroderma cohort followed at the Rheumatology clinic of the University Hospital of Heraklion will be included. The study will include a prospective and a retrospective part. Prospective part All newly diagnosed patients will undergo NVC at baseline and subsequently every six months. We will review demographics, clinical features and autoantibodies status. Major organ involvement will be monitored (Pulmonary Function Test, DLCO, heart echocardiogram, chest XR, modified Rodnan skin score) at baseline and then every 6-12 months. Retrospective part Existing SSc patients with available NVC data at diagnosis will be included. We will correlate the NVC findings at the time of diagnosis with disease outcomes such as major organ involvement, end stage organ failure, need for hospitalization, and death. We will also correlate longitudinal changes of the NVC patterns with treatment responses and outcomes. © 2019 The Mediterranean Journal of Rheumatology (MJR).We present a case of a 75-year-old woman who admitted in the internal medicine department for a recent onset of persisting moderate daily fever and fatigue that started 30 days prior to her hospitalization. Her past medical history is remarkable for mild pulmonary fibrosis, megaloblastic anaemia, and hypergammaglobulinaemia of no obvious causes. On presentation, she was febrile (38°C) and had high ESR and CRP levels, but most of her laboratory tests were within normal levels and had no signs of arthritis or rash. She was hospitalized for suspected lower urinary tract infection and started on antibiotics. During hospitalization, her renal function deteriorated together with microscopic haematuria, proteinuria and granular urine casts in urine analysis and her inflammation markers raised further. A renal biopsy revealed glomerulonephritis with pauci-immune crescents, and serology tests were positive for anti-MPO p-ANCA, both suggesting a diagnosis of microscopic polyangiitis (MPA). While high-dose methylprednisolone pulses and cyclophosphamide were introduced intravenously, there was no remission, but respiratory failure occurred that led to patient's intubation and transfer to the ICU. She died a few days later due to septic shock. Asymptomatic pulmonary fibrosis can precede microscopic polyangiitis for several years and is associated with a poor prognosis. © 2019 The Mediterranean Journal of Rheumatology (MJR).Background Rheumatoid arthritis (RA) is associated with an increased risk for venous thromboembolism. However, so far, relatively few and small size-based studies have been conducted. CCT128930 We aimed to investigate the link between RA and venous thromboembolism utilizing a large sample of subjects originating from a large data base. Materials and methods The study was performed utilizing the medical database of Clalit Health Services, the largest healthcare provider in Israel. We enrolled all patients with RA and age- and gender-matched controls. Chi-square and t-tests were used for univariate analysis and a logistic regression model was used for a multivariate analysis. RA patients were compared to controls regarding the proportion of venous thromboembolic events (defined as deep vein thrombosis, pulmonary embolism or both). Multivariate logistic regression was employed to assess factors associated with thromboembolic events. Results The study included 11,782 patients with RA and 57,973 age- and gender-matched controls. RA patients had a higher rate of venous thromboembolism events compared with controls (6.92% vs. 3.18%, respectively, p less then 0.001). RA and mean C-reactive protein levels were found to be independently associated with the proportion of thromboembolic events (OR 2.27 for RA and 1.07 for each 1 mg/dL increment of mean C-reactive protein, respectively). Conclusion RA and C-reactive protein levels are independently associated with venous thromboembolic events. Physicians should be aware of such findings and have a lower threshold for suspecting detecting such events in patients with RA, mainly those with mean high levels of C-reactive protein. © 2019 The Mediterranean Journal of Rheumatology (MJR).Systemic sclerosis (SSc) is a complicated multisystem disease which is characterized by the highest standardized mortality ratio among all systemic rheumatic diseases with no approved therapies so far. From a pathogenetic point of view it is generally considered that autoimmunity, vasculopathy and fibrosis are the main pathophysiologic processes. In this opinion article/minireview we will discuss current and future options for SSc-related fibrotic manifestations (skin thickening and lung fibrosis). Based on the results of SLS II the best treatment option for skin involvement in SSc is mycophenolate mofetil (MMF). Methotrexate (MTX) is another option which is safe and of low cost but evidence supporting its use is weak. The standard of care for SSc-ILD nowadays is MMF. Patients not responding to MMF could be treated with rituximab (RTX) or cyclophosphamide (CYC) (tocilizumab [TCZ] could be an option as well but only for patients with increased inflammatory markers). Hematopoietic stem cell transplantation (HSCT) could be considered in patients with severe/life-threatening disease who have failed conventional treatment. The most promising therapeutic approach currently been evaluated in phase 3 trials is probably the combination of MMF plus pirfenidone. © 2019 The Mediterranean Journal of Rheumatology (MJR).Autoantibody measurement is the chosen tool, in addition to clinical observations, for the diagnosis of autoimmune diseases. Hence, it is essential for these measurements to be reliable and in the longer run to be standardised. Due to the intrinsic variability of analytes and reagents, and the heterogeneity of the available techniques, standardisation cannot be taken for granted, and results may vary between laboratories. As a consequence, diagnoses can be missed or wrong and unnecessary costs may burden individuals and healthcare systems. Standardisation of autoantibody testing is a demanding and multi-parameter task, but could be part of the solution. So as to achieve standardisation, the development and availability of suitable certified reference materials for calibration and/or quality control is crucial. © 2019 The Mediterranean Journal of Rheumatology (MJR).Complex regional pain syndrome (CRPS) is a perplexing painful syndrome of the extremities usually following a harmful event. It is distinguished in two types, mainly depending on the presence of nerve injury. Although its prevalence may vary depending on social and ethnic factors, middle-aged women seem to suffer most often and the upper limb is the most commonly affected extremity. Apart from pain, which is the dominating feature, the clinical picture unfolds across several domains sensory, motor, autonomic and trophic. This syndrome develops in two phases, the acute (warm) phase, with the classic symptoms of inflammation, and the chronic (cold) phase, often characterized by trophic changes of the soft tissues and even bones. Although the syndrome has been studied for over two decades, no imaging or laboratory test has been established for the diagnosis and recently proposed diagnostic criteria have not yet been validated and are only occasionally applied. Its pathophysiology is still quite obscure, although the most likely mechanisms involve the classic and neurogenic paths of inflammation mediated by cytokines and neuropeptides, intertwined with changes of the autonomic and central nervous system, psychological mechanisms and, perhaps, autoimmunity. Although plenty of treatment modalities have been tried, none has been proven unequivocally efficacious. Apart from information and education, which should be offered to all patients, the most effective pharmacological treatments seem to be bisphosphonates, glucocorticoids and vasoactive mediators, while physical therapy and rehabilitation therapy also make part of the treatment. © 2019 The Mediterranean Journal of Rheumatology (MJR).Stroke is a major cause of morbidity, mortality and disability in systemic lupus erythematosus (SLE). Patients with SLE have a two-fold increase in the risk of stroke with younger patients (ie, less than 50 years of age) having an ever-higher risk (up to 10-fold). Although the prognosis of SLE has improved, mortality due to cerebrovascular events (CVE) remains unchanged. Cerebrovascular disease may be directly attributed to the disease per se, as a manifestation of neuropsychiatric SLE, or be the result of traditional cardiovascular risk factors accompanying the disease. Elucidation of the underlying mechanism(s) of CVE is essential as it may guide the type of therapy (ie, antithrombotic or anticoagulant therapy versus immunosuppressive). Strokes attributed to lupus usually occur early in the course of the disease and are often accompanied by evidence of activity in other organs; those related to antiphospholipid antibodies can occur at any time, in patients with either active or inactive SLE. In this review, we discuss the epidemiology, work-up, management and primary prevention of CVE in patients with lupus. In view of the effectiveness of thrombolysis, physicians need to educate lupus patients and their families for the early recognition of the signs of stroke and the need to seek prompt attention. To this end acronyms, such as FAST (Facial drooping, Arm weakness, Speech difficulties and Time to call emergency service) can be used as a mnemonic to help detect and enhance responsiveness to the needs of a person having a stroke. © 2019 The Mediterranean Journal of Rheumatology (MJR).