8,820 research outputs found
Retroperitoneal fibrosis
No full textRetroperitoneal fibrosis encompasses a range of diseases characterised by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighbouring structures--eg, ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Idiopathic disease was thought to result from a local inflammatory reaction to antigens in the atherosclerotic plaques of the abdominal aorta, but clinicolaboratory findings--namely, the presence of constitutional symptoms and the high concentrations of acute-phase reactants--and the frequent association of the disease with autoimmune diseases that involve other organs suggest that it might be a manifestation of a systemic autoimmune or inflammatory disease. Steroids are normally used to treat idiopathic retroperitoneal fibrosis, although other options--eg, immunosuppressants, tamoxifen--are available. The outlook is usually good, but, if not appropriately diagnosed or treated, the disease can cause severe complications, such as end-stage renal failure. Here, we review the different aspects of retroperitoneal fibrosis, focusing on idiopathic retroperitoneal fibrosis and on the differential diagnosis associated with the secondary forms
Upper limb lymphedema in inflammatory arthropathy
No full textUpper limb lymphedema in inflammatory arthropath
Management of large-vessel vasculitis
No full textPURPOSE OF REVIEW: Glucocorticoids are the mainstay of therapy for large-vessel vasculitis, but potential toxicity and frequent relapses led to studies with nonbiologic and biologic glucocorticoid-sparing agents. The aim of this review is to discuss the recent evidence for the management of giant cell arteritis (GCA) and Takayasu arteritis (TAK). RECENT FINDINGS: Tocilizumab proved to be a powerful glucocorticoid-sparing agent for GCA in a randomized placebo-controlled trial, whereas the trials with tocilizumab and abatacept failed to show a significant difference from placebo in relapse-free survival rate in TAK. Further trials are awaiting for establishing the role of abatacept and ustekinumab for GCA, and rituximab and tumor necrosis factor inhibitors, including certolizumab for TAK, as well as nonbiologic agents for both indications. SUMMARY: Despite recent randomized controlled trials with biologic agents, management of large-vessel vasculitis largely depends on observational studies. Well designed controlled trials using validated outcome measures in large number of patients, identification of biologic markers that could guide the choice of targeted treatments, and standardization of disease assessment including imaging modalities are unmet needs for the management of large-vessel vasculitis
Idiopathic aortitis: an underrecognized vasculitis
No full textAortitis 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 6.1%, with nearly three-quarters of cases being idiopathic. Identified risk factors for aortitis included advanced age, a history of connective tissue disease, diabetes mellitus, and heart valve pathology. As in virtually all pathological studies, this study has a bias toward reporting the most severe cases of aortitis requiring surgical repair
CD25 blockade for refractory polymyositis
No full textWe report a patient with refractory PM treated with the high-affinity interleukin-2 receptor (CD25) blocker basiliximab
Systemic vasculitis: state of the art and emerging concepts
No full textSystemic vasculitis: state of the art and emerging concept
Improving therapeutic options for patients with giant cell arteritis
No full textGlucocorticoids remain the mainstay of treatment of giant cell arteritis. The aim of this review is to establish the optimal schedule of glucocorticoid administration, and to ascertain which other treatments may be used as glucocorticoid-sparing agents
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