1,721,148 research outputs found
Established and new treatments of the idiopathic inflammatory myopathies: dermatomyositis and polymyositis
No full textThe aim of this article was to critically review and summarize the evidence on established and novel treatments for adult-onset DM and PM derived from randomized controlled trials (RCT). In the absence of evidence from such trials, data from open studies and case reports have been reported. Abstract data 2002 through 2006, American College of Rheumatology and 2002 through 2007 European League against Rheumatism have been included if relevant, if sufficient information could be extracted with regard to diagnosis ascertainment, treatment modalities, and outcome measures, and if the reported data had not been published as a full paper
Immunosuppressive treatment for giant cell arteritis: where do we stand?
No full textEditorial on the treatment of giant cell arteriti
Up-to-date treatment and management of myositis
Full text linkPURPOSE OF REVIEW: Myositis, or idiopathic inflammatory myopathy, is an overarching concept that includes dermatomyositis, polymyositis, immune-mediated necrotizing myopathy and the antisynthetase syndrome. Glucocorticoids are still considered the mainstay of treatment of myositis but some patients require add-on immunosuppressive therapy because of insufficient response to glucocorticoids, relapses when glucocorticoids are tapered, or because they incur glucocorticoid-related side effects. RECENT FINDINGS: The goal of this article was to review (PubMed search from January 2019 through June 2020) the efficacy and safety of standard and novel agents used in adult dermatomyositis, polymyositis, immune-mediated necrotizing myopathy and the antisynthetase syndrome. SUMMARY: Established therapies beyond glucocorticoids continue to have a major role in managing patients with myositis. In addition, novel agents are being tried for refractory manifestations of myositis
Polyarteritis nodosa
No full textPolyarteritis nodosa (PAN) is a rare vasculitis affecting middle-sized arteries. Its annual incidence in Europe is estimated to range between 0 and 1.6 cases per million, while the prevalence is about 31 cases per million. The frequency of hepatitis B virus (HBV)-related PAN has declined in developed countries since vaccination against HBV has been implemented. Specifically, before vaccination against HBV was implemented on a large scale, more than one-third of adults with PAN were infected by HBV, whereas currently only 5 % of European adults with PAN are infected by HBV. PAN is usually considered an immune-complex-driven vasculitis. However, the evidence of abundant CD4+ T cells in vascular inflammatory infiltrates suggests that PAN may also be induced by a T-cell response. Clinically, PAN usually presents with constitutional manifestations as well as symptoms and signs related to the organs affected. Organ ischemia is thought to be due to vascular stenoses, while ruptured aneurysms can result in tissue hemorrhage. The most frequent clinical features include constitutional manifestations, myalgia, arthralgia, peripheral neuropathy and mononeuritis multiplex. There are no specific blood tests to diagnose PAN, but inflammatory markers are typically elevated. Therefore, the diagnosis rests on histological changes in affected organs, showing a transmural vessel wall infiltrate, or angiographic findings, including small saccular or fusiform aneurysms and stenoses. Treatment includes glucocorticoids in patients without poor prognostic factors or cyclophosphamide if the disease is life- or organ-threatening
A case of arthritis and vasculitis associated with the refractory anemia with excess of blasts syndrome resistant to glucocorticoid treatment that responded favorably to TNF-alpha blockade
No full textTo describe a case of arthritis and vasculitis associated with the refractory anemia with excess of blasts syndrome resistant to glucocorticoid treatment that responded favorably to TNF-alpha blockade
Skin manifestations in giant cell arteritis
No full textGiant cell arteritis is a primary systemic vasculitis involving large- and medium-sized vessels which affects almost exclusively patients aged 50 years or older. Cutaneous manifestations are rare because giant cell arteritis spares the small vessels, that is, those vessels that are typically associated with skin lesions. The most common skin abnormality observed in giant cell arteritis is thickening and, less frequently, erythema or nodules of the superficial temporal arteries. Necrosis of the scalp and tongue has also been occasionally described in patients with severe vascular ischemia induced by inflammation. Glossitis and facial edema are other rare lesions that reflect active inflammation
The role of PET/CT in disease activity assessment in patients with large vessel vasculitis
No full textPurpose of reviewThe aim of this article was to review the recent contributions on the role of PET in assessing disease activity in patients with large-vessel vasculitis (giant cell arteritis and Takayasu arteritis).Recent findings18FDG (fluorodeoxyglucose) vascular uptake in large-vessel vasculitis at PET shows moderate correlation with clinical indices, laboratory markers and signs of arterial involvement at morphological imaging. Limited data may suggest that 18FDG (fluorodeoxyglucose) vascular uptake could predict relapses and (in Takayasu arteritis) the development of new angiographic vascular lesions. PET appears to be in general sensitive to change after treatment.SummaryWhile the role of PET in diagnosis large-vessel vasculitis is established, its role in evaluating disease activity is less clear-cut. PET may be used as an ancillary technique, but a comprehensive assessment, including clinical, laboratory and morphological imaging is still required to monitor patients with large-vessel vasculitis over time
Comment on: Risk factors for severe cranial ischaemic events in an Italian population-based cohort of patients with giant cell arteritis: Reply
No full textPrimary CNS vasculitis: Pathophysiological diversity
No full textPrimary central nervous system vasculitis (PCNSV) is a rare vasculitis that affects the brain and occasionally the spinal cord of adults and children. Extensive progress has been achieved in the recognition of clinicopathological subsets that has translated into useful nosology reflective of the natural history and response to immunosuppressant treatment. Affected patients with small-artery vasculitis characterized by positive histology, negative cerebral angiography and prominent leptomeningeal enhancement on MRI after intravenous gadolinium administration have a milder disease course and more favorable outcome compared to those with large-artery PCNSV with multiple large-artery stenoses on cerebral angiography and extensive lesions on MRI. Subsets of patients presenting with rapidly progressive PCNSV, solitary tumor-like mass lesion and vascular amyloid deposits have the worst prognosis
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