1,721,302 research outputs found
Comment on: First report of FIP1L1-PDGFRα-positive eosinophilic granulomatosis with polyangiitis: Reply
No full textIgG4-related kidney disease: from renal histopathology and immunopathogenesis to novel pharmacological interventions
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The new look of classification criteria for systemic vasculitis
No full textAn ambitious project to update the classification of vasculitis syndromes has culminated in the development of new classification criteria for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, Takayasu arteritis and giant cell arteritis. Endorsed by the ACR and EULAR, the new criteria reflect progress in the understanding and assessment of these conditions
Immunoglobulin G4-related disease: some missing pieces in a still unsolved complex puzzle
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Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases
No full textAortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic wall in aortitis, and spreads to the periaortic space in periaortitis. Aortitis is classified as non-infectious or infectious. Non-infectious aortitis represents a common feature of large-vessel vasculitides but can also be isolated or associated with other rheumatologic conditions. Periaortitis can be idiopathic or secondary to a wide array of etiologies such as drugs, infections, malignancies, and other proliferative diseases. Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease, a recently characterised fibro-inflammatory systemic disease. Prompt recognition, correct diagnosis and appropriate treatment are essential in order to avoid life-threatening complications. (C) 2020 Elsevier Masson SAS. All rights reserved
Chronic periaortitis: a spectrum of diseases
No full textPURPOSE OF REVIEW:
Chronic periaortitis includes idiopathic retroperitoneal fibrosis, perianeurysmal retroperitoneal fibrosis, and inflammatory abdominal aortic aneurysms. This review analyses the different aspects of the disease and highlights evolving concepts concerning its pathogenesis, diagnosis, and management.
RECENT FINDINGS:
It has recently been reported that asbestos exposure is a major risk factor for idiopathic retroperitoneal fibrosis. An increasing number of studies showing an association with autoimmune diseases clearly support the hypothesis of a close link between autoimmunity and chronic periaortitis. Furthermore, various findings (eg, constitutional symptoms, the involvement of other organs, high acute-phase reactant levels) support the hypothesis that chronic periaortitis may be a manifestation of a systemic disease and challenge the well-known theory of a local immune response to antigens in atherosclerotic plaque. In addition to CT and MRI, which are the diagnostic modalities of choice, positron emission tomography may be useful in monitoring disease activity and response to therapy. Although there is a lack of prospective randomized trials, recent studies have highlighted the role of steroids, immunosuppressive agents, and tamoxifen in the medical treatment of chronic periaortitis.
SUMMARY:
Chronic periaortitis is a rare disease with protean manifestations but, if correctly diagnosed, can be successfully managed. It should be approached in the setting of a systemic process, and clinicians must be aware that other organs may be affected. 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
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