Etanercept in spondyloarthropathies. Part I: current evidence of efficacy.

Etanercept is a recombinant soluble tumour necrosis factor alpha receptor administered subcutaneously at the dose of 50 mg weekly (or 25 mg/twice weekly) for the treatment of the main chronic arthritides: rheumatoid arthritis and spondyloarthropathies. It shows high qualities in terms of efficacy and manageability. Favourable results were reported in all localisations of spondyloarthropathies: axial disease, peripheral arthritis, and enthesitis.

New indications for biological therapies.

Biological agents have originally been developed to treat refractory arthritis, but evidence has been accruing, supporting their use in vasculitis as well. In the large-vessel vasculitides giant cell arteritis and Takayasu arteritis, TNF-α inhibitors have shown some efficacy in patients with relapsing disease. In contrast, in patients with recent onset of giant cell arteritis, TNF-α inhibitors failed to provide a significant benefit over and above that conferred by glucocorticoids alone.

PLA1/A2 polymorphism of the platelet glycoprotein receptors IIIA in Behçet's disease.

Objectives: To investigate potential associations between the PlA1/A2 polymorphism of the platelet glycoprotein receptor IIIA (GpIIIa) gene and venous thrombosis and other clinical manifestations in Italian patients with Behçet's disease (BD).

Methods: Two hundred consecutive Italian patients satisfying the International Study Group criteria for BD who were followed up for seven years and 241 healthy Italian age- and gender-matched blood donors were molecularly genotyped for the PlA1/A2 polymorphism of the platelet GpIIIa gene; 118 and 117 of the 200 BD patients were also respectively genotyped for factor V Leiden and prothrombin gene G20210A polymorphisms. A standard microlymphocytotoxicity technique was used to type serological HLA class B51.

Primary central nervous system vasculitis presenting with intracranial hemorrhage.

Objective: To describe a subset of cases in a large retrospectively identified cohort of patients with primary central nervous system vasculitis (PCNSV) who present with intracranial hemorrhage.

Methods: The study consisted of a cohort of 131 consecutive patients with PCNSV who were seen at the Mayo Clinic over a 25-year period from 1983 to 2007. The diagnosis of PCNSV was based on findings of brain or spinal cord biopsy, cerebral angiography, or both.

Recommendations for the use of biologic therapy in the treatment of psoriatic arthritis: update from the Italian Society for Rheumatology.

Objectives: To update the 2006 Italian Society for Rheumatology recommendations for the use of biologic (TNF-α blocking) agents in the treatment of psoriatic arthritis (PsA).

Methods: A panel of experts performed a literature search and identified the items that required updating on the basis of new published data. A draft of the updated recommendations was circulated to a group of Italian Rheumatologists with a specific expertise in PsA and in therapy with biologic agents, and their suggestions were incorporated in the final version.

Recommendations for the use of biologic therapy in rheumatoid arthritis: update from the Italian Society for Rheumatology I. Efficacy.

Given the availability of novel biologic agents for the treatment of rheumatoid arthritis (RA), various national scientific societies have developed specific recommendations in order to assist rheumatologists in prescribing these drugs. The Italian Society for Rheumatology (Società Italiana di Reumatologia, SIR) decided to update its recommendations and, to this end, a systematic literature review was carried out and the evidence derived from it was discussed and summarised as expert opinions. Levels of evidence, strength of recommendations and levels of agreement were reported.

Small-vessel vasculitis surrounding an uninflamed temporal artery and isolated vasa vasorum vasculitis of the temporal artery: two subsets of giant cell arteritis.

Objective: To evaluate the frequency and clinical characteristics of periadventitial small-vessel vasculitis (SVV) and isolated vasa vasorum vasculitis (VVV).

Methods: We identified 455 temporal artery biopsies performed in residents of Reggio Emilia, Italy between 1986 and 2003. Slides of temporal artery biopsy specimens were reviewed by a pathologist who was blinded with regard to clinical data.

A putative functional variant within the UBAC2 gene is associated with increased risk of Behçet's disease.

Objective: Using a genome-wide association scan and DNA pooling, we previously identified 5 novel genetic susceptibility loci for Behçet's disease. We undertook this study to establish the genetic effect within the UBAC2 gene, in the course of which we replicated this genetic association and identified a functional variant within this locus.

Methods: We studied a total of 676 Behçet's disease patients and 1,096 controls.

Imaging findings in primary central nervous system vasculitis.

Primary central nervous system vasculitis (PCNSV) is a rare primary vasculitis limited to the brain and spinal cord. It can affect any age group, but has a predilection for subjects aged 40 to 60 years without clear gender predominance. Clinical manifestations are nonspecific, including headache, non-focal neurological features and, less frequently, focal neurological signs.

Idiopathic aortitis: an underrecognized vasculitis.

Aortitis is a general term denoting inflammation of the aortic wall. Various infectious and non-infectious diseases can be complicated by aortitis; in addition, isolated idiopathic aortitis has also been described. In a 12-year nationwide Danish population-based study, the prevalence of aortitis among 1,210 resected thoracic aorta samples was 6.

[Disease activity assessment in large vessel vasculitis].

Disease activity assessment in large vessel vasculitis (LVV) is often challenging for physicians. In this study, we compared the assessment of disease activity based on inflammatory markers, clinical indices (Indian Takayasu Activity Score [ITAS] and the Kerr/National Institute of Health indices [Kerr/NIH]), and 18F-Fluorodesossiglucose (FGD) vascular uptake at positron emission tomography (Pet). We found that Pet results did not statistically correlate with the clinical indices ITAS and Kerr/NIH, because FDG uptake was increased (grade>2 on a 0-3 scale in at least one evaluated vascular segment) in many patients with inactive disease according to clinical and laboratory parameters (i.

Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial.

Background: Glucocorticoids are the mainstay of treatment of idiopathic retroperitoneal fibrosis, but they often have substantial toxic effects. 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.

Expression of interleukin-32 in the inflamed arteries of patients with giant cell arteritis.

Objective: Giant cell (temporal) arteritis (GCA) is a vasculitis that mainly affects the large and medium arteries, especially the branches of the proximal aorta. Interleukin-32 (IL-32) is a recently described Th1 proinflammatory cytokine, and is mainly induced by interferon-γ (IFNγ), IL-1β, and tumor necrosis factor α (TNFα). This study was undertaken to investigate the expression and tissue distribution of IL-32 in artery biopsy specimens from patients with GCA.

Role of 18F-fluorodeoxyglucose positron emission tomography in the workup of retroperitoneal fibrosis.

Retroperitoneal fibrosis is a syndrome characterised by the presence of fibrosclerotic tissue in the retroperitoneum, often encasing the ureters. In most cases, retroperitoneal fibrosis is idiopathic, but may also be associated with large vessel vasculitis at distant sites, with the so-called IgG4-related sclerosing disease, as well as with exposure to some medications, infections, malignancies, surgery, or radiation. 18-Fluorodeoxyglucose (FDG) positron emission tomography (PET) is a nuclear medicine technique which is able to accurately identify in vivo areas characterised by elevated glucose metabolism, such as inflammatory, infective, and neoplastic lesions.

The effectiveness of a biologic agent on axial manifestations of psoriatic arthritis. A twelve months observational study in a group of patients treated with etanercept.

Objectives: To investigate the effectiveness of etanercept on axial manifestations of a group of patients with established psoriatic arthritis (PsA).

Methods: This was a multicentre observational study. PsA was classified based on the CASPAR criteria.

Patient global assessment in psoriatic arthritis: a multicenter GRAPPA and OMERACT study.

Objective: During OMERACT 8, delegates selected patient global assessment (PGA) of disease as a domain to be evaluated in randomized controlled trials in psoriatic arthritis (PsA). This study assessed the reliability of the PGA, measured by means of 0-100 mm visual analog scale (VAS), and the additional utility of separate VAS scales for joints (PJA) and skin (PSA).

Methods: In total, 319 consecutive patients with PsA (186 men, 133 women, mean age 51 ± 13 yrs) were enrolled.

CC chemokine receptor 5 polymorphism in chronic periaortitis.

Objective: Chronic periaortitis (CP) is a rare disease characterized by a fibro-inflammatory tissue surrounding the abdominal aorta, and includes non-aneurysmal [idiopathic retroperitoneal fibrosis (IRF)] and aneurysmal forms [inflammatory abdominal aortic aneurysm (IAAA)]. We investigated whether CC chemokine receptor 5 (CCR5)Δ32 polymorphism confers susceptibility to CP.

Methods: One hundred CP patients and 180 healthy controls were genotyped for CCR5Δ32 polymorphism by molecular methods.

Vasculitis of the gastrointestinal tract in chronic periaortitis.

The term "chronic periaortitis" (CP), proposed by Mitchinson in 1984, comprises 3 main entities: idiopathic retroperitoneal fibrosis (IRF), inflammatory abdominal aortic aneurysms (IAAAs), and perianeurysmal retroperitoneal fibrosis (PRF).The presence of constitutional symptoms, high acute-phase reactants, positive autoantibodies, and associated autoimmune diseases suggests a systemic inflammatory process. Histopathologic findings show vasculitis with fibrinoid necrosis involving the aortic vasa vasorum as well as the small and medium retroperitoneal vessels.

Aortitis and periaortitis in ankylosing spondylitis.

Aortic involvement is a potential life-threatening complication of ankylosing spondylitis, usually occurring late in the course of this frequent disease. Inflammatory lesions evolving to fibrosis are primarily localized in the aortic root causing regurgitation, but this process can extend into the left atrium (subaortic bump) involving the mitral valve and the heart conduction system. First, second and third degree atrioventricular blocks are the most common conduction alterations described and they can be temporary.

Chronic periaortitis: a large-vessel vasculitis?

Purpose Of Review: Chronic periaortitis is characterized by a fibro-inflammatory process spreading from the abdominal aorta and the iliac arteries. Originally, chronic periaortitis was considered a localized inflammatory response to severe aortic atherosclerosis. However, subsequent studies have shown that chronic periaortitis may also involve other arteries and present with features of auto-immune diseases.

Definition of remission and relapse in polymyalgia rheumatica: data from a literature search compared with a Delphi-based expert consensus.

Objective: To compare current definitions of remission and relapse in polymyalgia rheumatica (PMR) with items resulting from a Delphi-based expert consensus.

Methods: Relevant studies including definitions of PMR remission and relapse were identified by literature search in PubMed. The questionnaire used for the Delphi survey included clinical (n=33), laboratory (n=54) and imaging (n=7) parameters retrieved from a literature search.