1,721,065 research outputs found
Classification criteria for Sjogren's syndrome: A critical review.
No full textOver the years, several different criteria sets have been proposed for the classification of Sjögren's syndrome (SS), but none of them has been widely adopted by the scientific community until the publication of the 1993 Preliminary European Classification criteria. These Classification criteria have been largely employed both in clinical practice and in observational and interventional studies for many years. In 2002 the Preliminary European Criteria were re-examined by a joint American and European Committee. The result of this revision were the American and European Consensus Group classification criteria (AECG-criteria) which introduced more clearly defined rules for classifying patients with primary or secondary SS, and provided more precise exclusion criteria. These AECG-criteria set is now considered to be valid to ensure a specific diagnosis of SS by the vast majority of the expert in the field. To date, the AECG-criteria have been cited more than 1.304 in literature and have been used to estimate the point prevalence of the disease in several studies conducted in Greece, UK, Turkey and Norway. However, when employed in epidemiologic studies or i
One year in review 2017: novelties in the treatment of rheumatoid arthritis
No full textRheumatoid arthritis (RA) is a chronic disease characterised by inflammation of the synovial tissue in joints, which can lead to joint destruction. The primary goal of the treatment is to control pain and inflammation, reduce joint damage and disability, and maintain or improve physical function and quality of life. The present review is aimed at providing a critical analysis of the recent literature on the novelties in the treatment of RA, with a particular focus on the most relevant studies published over the last year
Cryoglobulinemic vasculitis: a 2023 update
No full textPurpose of review Cryoglobulinemic vasculitis (CV) is an immune complex mediated small vessel vasculitis characterized by the presence of cryoglobulins in serum, often associated with hepatitis C infection, systemic autoimmune diseases or hematological conditions. The focus of this review is to provide an update on new insights into pathogenesis, epidemiology and therapies of infectious and noninfectious type II and type III CV. Recent findings The introduction of new antiviral drugs for treatment of hepatitis C infection implied major changes in HCV-related CV, allowing to shed new lights on CV pathogenesis and mechanisms of relapse and, therefore, to increase the relevance of autoimmune diseases in CV epidemiology. Specific B-cell clones are involved in the production of pathogenic immune complexes that leads to small-vessel vasculitis. Therefore, both antiviral treatments [direct-acting antivirals (DAAs) and oral nucleot(s)ide analogues] and targeted anti-CD20 therapies (rituximab) prove to be safe and effective options, leading to a better prognosis. Association of Sj & ouml;gren syndrome and CV defines a specific phenotype of patients, characterized by severe manifestations and poor outcome. Summary Removing viral stimulation on B-cells through direct-acting antivirals and blocking B-cells proliferation and differentiation with rituximab are the goals of treatment of CV. However, further research is needed to identify prognostic factors of refractory and relapsing disease
Systemic vasculitis: an annual critical digest of the most recent literature
No full textHerewith we provide our annual digest of the recent literature on systemic vasculitis in which we reviewed all the articles published during the last 12 months on large-, medium- and small-vessel vasculitis, and selected the most relevant studies regarding the epidemiology, pathogenesis and management of systemic vasculitis. In particular, we focused the attention on giant cell arteritis, ANCA-associated vasculitis and cryoglobulinaemia
Skin manifestations and sjögren’s syndrome
No full textSjögren’s syndrome (pSS) is a common systemic autoimmune disease which primarily affects the salivary and lachrymal glands and usually leads to a persistent dryness of the mouth and eyes due to the lymphocytic infiltration and functional impairment of the exocrine glands. The disease is commonly included in the spectrum of connective tissue diseases (CTDs), and together with the other members of this disease family, it shares the possibility of a multisystemic involvement, a variable clinical picture, and a very large range of clinical and serological manifestations. Cutaneous manifestations are considered one of the most common extra-glandular features of pSS and can be distinguished as nonvascular lesions (i.e., xerosis, angular cheilitis, eyelid dermatitis, annular erythema) and vascular lesions (i.e., Raynaud’s phenomenon and vasculitis). In this chapter, the definition, differential diagnosis, and histological features of skin manifestations in pSS will be provided
Long-Term Outcome of Idiopathic Uveitis: A Prospective Cohort Study
No full textBackground Uveitis may be associated to systemic immune-mediated diseases or may be due to infectious agents, traumatisms, malignancies or medications. A variable part of uveitis remains idiopathic and few data are available on their evolution.
Objectives The primary aims of the study were: 1) to explore the clinical pattern of the uveitis in a cohort of patients, who have been followed in the last 7 years in a multidisciplinary uveitis clinic of a rheumatologic referral center; 2) to prospectively study the long-term outcome of patients with a previous diagnosis of idiopathic uveitis.
Methods The study design consists of two stages:
1) Cross-sectional study, evaluating the clinical pattern and diagnosis of all consecutive patients with uveitis examined from January 2007 to January 2014.
2) Prospective cohort study, exploring the long-term (at least 5 years of follow-up) outcome of patients with a diagnosis of idiopathic uveitis.
Patients were classified according to International Uveitis Study Group definitions. All patients had a comprehensive rheumatologic and internist evaluation. The diagnosis of autoimmune systemic disease was performed according to international criteria currently used in clinical practice. The diagnosis of infectious uveitis was performed by mean of clinical examination and serology. Moreover, all patients had a comprehensive ophthalmological evaluation, including: ocular clinical history, Snellen visual acuity, slit-lamp examination, applanation tonometry and dilated fundus examination; visual field, fluorescein-angiography and optical coherence tomography were performed when required. Moreover, all patients underwent the standard protocol of serological examinations for uveitis. Patients with a diagnosis of idiopatic uveitis were prospective evaluated every 6 months.
Results One hundred and fifty-seven patients (M/F: 99/58, mean age ± SD: 42±7 years) were studied in the cross-sectional stage. The most common ocular feature was represented by anterior uveitis, that was present in 64 (41%) patients, followed by posterior uveitis reported in 50 (32%); panuveitis was present in 18% of the cohort and intermediate uveitis in 9%. For 92 of 157 (59%) patients with uveitis, a specific etiologic diagnosis was established, while 65 (41%) of patients were found to have an idiopathic uveitis. Fuchs' heterochromic iridocyclitis and Behçet's disease (BD) represented the most common causes of anterior and posterior uveitis respectively, while among infectious uveitis we reported a relatively high frequency of types secondary to herpetic infection. Fifty-six patients out of 65 with a diagnosis of idiopathic uveitis were followed for at least 5 years in the prospective cohort stage. After this period, 10/56 patients had a diagnosis of BD, the other still remained idiopathics
Conclusions Our results have shown that the most common anatomical site of inflammation is still represented by the anterior segment. Notably, data from our prospective cohort study, suggest that also after several years, a significant part of idiopathic uveitis may still express symptoms suggestive of a systemic autoimmune disease, such as BD; this is of particular interest, since a tight control during the follow-up is mandatory in all case of idiopathic uveitis
Classification criteria
No full textOver the past 25 years, several classification criteria sets have been proposed for primary Sjögren’s syndrome (pSS). In this chapter, we describe the different features of these criteria and summarize the debate that has arisen in the scientific community over this period. We devote particular attention to a critical analysis of the American and European consensus group criteria (AECG), which have achieved important consensus in the literature. Proposals for the future are also outlined
One year in review 2017: systemic vasculitis
No full textSystemic vasculitis is a group of heterogeneous, disabling disorders. Great interest has recently arisen in pathophysiology, clinical phenotypes and therapy of large- and small-vessel vasculitis. The general work hypothesis has been to promote research focused on disease-related pathogenetic pathways, with the ultimate goal of identifying novel diagnostic and prognostic biomarkers, thus leading towards more effective targeted treatments. Following the previous annual reviews of this series, we will hereby provide a critical digest of the recent literature on small- and large-vessel systemic vasculitis, with a specific focus on novel possible disease-related biomarkers and their impact on current and future therapies
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