1,721,649 research outputs found
Neurological manifestations of vasculitis
No full textPrimary vasculitis syndromes are diseases characterized by inflammation and necrosis of blood vessel, resulting in vessel occlusion and secondary ischemia of involved tissues. The presentation and clinical manifestations of the vasculitides are extremely variables, and an accurate diagnosis is often difficult. Because neurologic symptoms may be the presenting, the first and in some cases the only manifestations of the underlying disease process, a systematic diagnostic and therapeutic approach to the vasculitic syndromes is essential for the neurologist. Furthemore, a wide variety of clinical manifestations, particularly neurological, of connective tissue disorders may also be related to vasculitis. This review focuses on the neurological manifestations of the late primary vasculitis and of the vasculitis associated with connective tissue disorders. An early diagnosis and the begun of appropiate therapy will prevent the development or progression of central nervous system and peripheral nervous system manifestations
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
Vasculitides E. miscellaneous vasculitis (Behçet's Disease, Primary Angiitis of the Central Nervous System, Cogan's Syndrome, and Erythema Elevatum Diutinum)
No full textThe prevalence of Behçet's disease is highest in countries of the eastern Mediterranean, the Middle East, and East Asia. Aphthous oral ulcers are usually the first and most persistent clinical feature of Behçet's disease. Aphthous ulcers also occur frequently on the genitals (e.g., the scrotum or vulva). Uveitis-either anterior or posterior-is common in Behçet's disease and a source of major morbidity. Many forms of central nervous system disease may occur in Behçet's disease. These include aseptic meningitis and white matter lesions in the brainstem. Human leukocyte antigen (HLA)-B51 is a strong risk factor for Behçet's disease. The diagnosis of primary angiitis of the central nervous system is predicated upon either biopsy evidence of vasculitis or angiographic findings suggestive of vasculitis in the setting of other compelling features, for example, strokes demonstrated by magnetic resonance imaging or the findings of a cerebrospinal fluid pleocytosis. The diagnosis of primary angiitis of the central nervous system should never be made on the basis of an angiogram alone. Patients with benign angiopathy of the central nervous system are predominantly female, tend to present acutely with headache (with or without focal symptoms), and have normal or near normal cerebrospinal fluid. Cogan's syndrome refers to the association of inflammation in both the eyes and ears: specifically, the occurrence of nonsyphilitic interstitial keratitis and immune-mediated inner ear disease, resulting in audiovestibular dysfunction. Any type of ocular inflammation may occur in Cogan's syndrome (e.g., scleritis, uveitis, orbital pseudotumor). The inner ear disease associated with this condition often leads to deafness. In erythema elevatum diutinum, skin lesions consist of purple, red, or brown plaques and often have an annular or nodular appearance. The skin lesions have a predilection for the extensor surfaces of the distal extremities and often overlie joints, but may be generalized. © 2008 Springer Science+Business Media, LLC. © 2008 Springer-Verlag New York
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
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