1,721,054 research outputs found
Anticardiolipin antibodies in Klinefelter's syndrome [4]
No full textA case of Klinefelter's syndrome with cohexisting aCl antibodies is reported. Some HLA aplotipes may have relevance as predisposing factors
Single photon emission computed tomography in neuropsychiatric systemic lupus erythematosus
No full textIn comparison to MRI, SPECT detect more hypoperfused areas. The reliance on a multeplicity of diagnostic tests will be required for the foresseble future in NPSLE
Antisynthetase syndrome
No full textWe present a case of pulmonary involvement associated to idiopathic inflammatory myopathy in a relatively homogenous clinical profile known as antisynthetase syndrome. This syndrome is characterized by myositis, arthralgias/arthritis, interstitial lung disease, fever, mechanic's hands, and Raynaud's phenomenon. Autoantibodies against aminoacyl-tRNA synthetases are associated, with anti-Jo-1 being the one most commonly found. The clinical appearance of pulmonary involvement is heterogeneous, slowly progressive or acute. Myositis precedes or is concurrent with the development of lung disease in the majority of cases, but the occurrence of lung involvement before myositis occurs in more than one third of cases. Therapy of these respiratory manifestations varies with the kind of clinical onset of the disease and with the level of functional impairment and must be personalized on the basis of clinical, functional and radiological findings of the follow up
Primary pathologic role of interleukin-6 in rheumatoid arthritis
Full text linkBACKGROUND: Interleukin-6 (IL-6) is a polyfunctional cytokine that regulates a very large number of cellular activities. Its implication in acute-phase reactant production by hepatocytes is of particular interest, as is its involvement in chronic inflammatory diseases, mainly rheumatoid arthritis, Crohn's disease, and Castleman's disease. Transgenic mice lacking IL-6 expression were completely protected against collagen-induced arthritis, and Tumor Necrosis Factor (TNF-alpha) induces synovial cells to produce IL-6 and their proliferation. However, there is still some controversies regarding the unique proinflammatory activity of IL-6. Some studies have demonstrated that IL-6 and TNF-alpha may have an opposite effect in synovial cultured cells since IL-6 could represent a negative loop for TNF-alpha induced synovitis. However, phase III studies of rheumatoid arthritis patients treated with anti IL-6 receptor (tocilizumab) indicate an acceptable safety profile relative to the clinical benefit. AIM OF THE STUDY: In this review, we summarized the rationale and the main evidence regarding the therapeutic benefit of blocking IL-6 activity in rheumatoid arthritis. © 2008 Elsevier Srl
Long-term outcome of giant cell arteritis
No full textGiant cell arteritis is usually a self-limiting disease with a variable duration of months to years. However, in a subset of patients the disease may follow a protracted course, requiring long-term treatment with glucocorticoids. To date, glucocorticoids are the only agents whose efficacy has been unquestionably proven. More specifically, they can both improve the clinical symptoms of giant cell arteritis and also prevent its complications, including visual loss. Glucocorticoids therapy is notoriously fraught with numerous side effects, therefore it is sensible to taper glucocorticoids as quickly as possible. Flares are not uncommon and tend often to occur upon tapering of glucocorticoids dosage or on withdrawal of glucocorticoids therapy. However, in most cases flares are mild and appear to respond favorably to an increase in glucocorticoids dosage or reintroduction of glucocorticoids therapy, respectively. Mortality rates of giant cell arteritis patients are comparable to those of the general population, but there is evidence for an increased frequency of potentially life-threatening ischemic events, such as myocardial infarction and cerebro-vascular accidents, especially early on in the disease course. The risk conferred by the disease appears to decrease with time, presumably as a consequence of glucocorticoids treatment, whereas it can remain significantly elevated in patients whose disease activity is not sufficiently controlled by the treatment. By contrast, there is no evidence that giant cell arteritis is associated with an increased prevalence of malignancies or that it may represent a paraneoplastic syndrome
Pre-pregnancy counselling of patients with vasculitis
Full text linkThe knowledge about the risk of pregnancy in vasculitides mostly derives from single case reports or at best from retrospective studies with all the caveats that these observations include. Primary systemic vasculitides are uncommon, encompassing a broad spectrum of severity, from mild to life-threatening manifestations and with different natural histories, from self-limiting to relapsing or chronic active disease. The treatments require a cautious use of immunosuppressants tailored to each specific condition. Furthermore, most of the cytotoxic drugs necessary to treat vasculitis act by modifying the cell cycle and cell differentiation, biological effects that are particularly hazardous for the foetus. In order to have an uncomplicated pregnancy, conception should be planned when the disease is inactive. Moreover, organ failure or damage, due to previous disease activity, must also be taken into account since it can lead to adverse obstetrical and fetal outcomes
HYPEROSTOSIS AND MULTIFOCAL OSTEITIS - A PURELY RHEUMATOLOGICAL SUBSET OF THE SAPHO SYNDROME
No full textSapho has recently proposed as an acronim identifying a syndrome characterized by synovitis, acne, pustulosis, hyperostosis and osteomyelitis. The work reported here presents the case of a $3 yrs old man with the skeletal involvement alone. These data confirm that the clinical picture of hyperostosis and osteitis even without cutaneous involvement is nonetheless related to SAPHO syndrome
Systemic sclerosis lung disease in limited (C.R.E.S.T.) and diffuse subset
No full textFibrosing alveolitis and arterial pulmonary hypertension are the main cardiopulmonary findings in patients with systemic sclerosis. Clinically significant interstitial lung disease occurs in about 50% of the cases while 20% of the cases has pulmonary arterial hypertension. The onset of interstitial lung disease is characterized by cellular infiltration (alveolitis) and microvascular injury. Nonspecific interstitial pneumonia is the most common outcome of this pathological process. The extent of damage by means of high resolution CT and of disease severity by measuring the reduction of forced vital capacity with co-existing or isolated reduction of diffusing capacity, at the onset of the disease, are predictive of pulmonary fibrosis or pulmonary arterial hypertension. A case report with a limited cutaneous scleroderma leading to a fatal pre-capillary pulmonary arterial hypertension is presented
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