A Vaglio

..Patients with chronic periaortitis (CP) often show clinical and laboratory findings of a systemic autoimmune disorder. The aim of the present study was to investigate the role of the HLA system in CP...

..07) and skin manifestations of all kinds (P?=?0.000047, ?(2?)=?16.567, OR?=?3.73). Conclusion. The PTPN22 620W allele confers susceptibility to the development of GPA (but not of MPA or CSS), and particularly of its ANCA-positive subset...

..Ureterolysis and aneurysm repair are frequently performed, but the inflammatory and chronic-relapsing nature of the disease often compels the use of medical therapy, which is based on steroids and immunosuppressants...

..To explore the association between HLA alleles and Churg-Strauss syndrome (CSS), and to investigate the potential influence of HLA alleles on the clinical spectrum of the disease...

..Glucocorticoids alone or in combination with immunosuppressive agents are usually effective treatment options, but the disease frequently has a chronic relapsing course...

..The study aims to report the frequency and the clinical characteristics of peripheral inflammatory arthritis in a cohort of CP patients, and to review the literature regarding the association between arthritis and CP...

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..Therefore, a careful long-term follow-up of EBV-related encephalopathy is advisable...

..Several reports have suggested tamoxifen as an alternative to glucocorticoids. We compared the efficacy of prednisone with that of tamoxifen in maintainance of remission in patients with idiopathic retroperitoneal fibrosis...

..Here, we review the different aspects of retroperitoneal fibrosis, focusing on idiopathic retroperitoneal fibrosis and on the differential diagnosis associated with the secondary forms...

..This study will review recent findings on the pathogenesis of CSS and its therapeutic approaches...

..No patient relapsed during the follow-up. CONCLUSIONS: Post-treatment residual masses are frequent in IRF patients but, in most cases, probably represent metabolically inactive tissue...

..In particular, we consider the role of Th2-mediated immune reactions and of immunogenetic factors such as HLA genotype as common determinants of these disorders...

..Our study indicates that a sclerotic background with myofibroblasts associated with a diffuse and perivascular infiltrate mainly consisting of T and B lymphocytes may be a pathological hallmark of IRF...

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..Steroids and tamoxifen are the most used drugs, whereas other agents such as azathioprine, methotrexate and cyclosporine are usually given to non-responder patients...

..However, the prevalence and severity of interstitial lung lesions in long-term, stable kidney transplant patients receiving either traditional or newer immunosuppressants is not known...

..Long-term low-dose immunotherapy may bring about an effective anti-tumour response even late in the course of the disease and after an initial disease progression...

..030 and 0.007, respectively). CONCLUSIONS: UAE is an independent prognostic factor that is related to disease stage in patients with RCC...

..The paper reviews the literature concerning ANCA-positive RPF and discusses the relationship between ANCA-positive vasculitis and RPF...

..Corticosteroids, immunosuppressants and endoscopic or surgical procedures must be appropriately combined for the correct management of chronic periaortitis patients...

..Its clinical course is chronic-relapsing, so a careful follow-up is essential. Further studies are needed to investigate the pathogenetic mechanisms and the most appropriate therapeutic options...

..It often progresses slowly and only rarely leads to clinically overt thrombosis; collateral circles thus develop and, as in our patients, may be a late manifestation of the disease...

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..Intravenous immunoglobulins or interferon-alpha may be useful for patients refractory to conventional treatment...

..Otolaryngologists may play a pivotal role in making an early diagnosis of this disease...

..As we could not identify any specific pathogenetic process, our final diagnosis was idiopathic subglottic tracheal stenosis...

..CONCLUSION: These clinical and pathologic features support the hypothesis that, at least in some patients, chronic periaortitis is a systemic autoimmune disease, perhaps involving a vasculitic process of small and medium vessels...

..To identify the prognostic factors of relapse and/or death during the course of primary small-vessel vasculitides (PSVV), and to differentiate their prognostic relevance by the type of vasculitis...

..In patients in whom antiviral therapy is ineffective, contraindicated or not tolerated, rituximab, a monoclonal anti-CD20 antibody, may be an alternative to standard immunosuppression...

..After a course of antituberculous treatment, a complete resolution of both adenopathy and RPF was observed. We discuss the potential pathogenetic mechanisms linking the 2 diseases...

..To evaluate the presence and extent of large-vessel inflammation in patients with chronic periaortitis (CP) using (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET)...

..HES can cause severe myocarditis with extensive myocyte loss, probably due to both necrosis and apoptosis. Myocardial fibrosis may occur despite treatment, and patients may be at risk for fatal arrhythmias...