La Revue de médecine interne
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Behçet's disease (BD) is a systemic large-vessel vasculitis characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, vascular, neurological, articular, and gastrointestinal manifestations. Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, non-steroidal anti-inflammatory agents and topical treatments with corticosteroids are often sufficient for mucocutaneous and joint involvement, a more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis, vascular, neurological and gastrointestinal involvement. ⋯ Recent improvements in the understanding of the pathogenic mechanisms have led to the identification of potential targets and future therapies for BD. In contrast to current non-specific immunosuppressive agents, the emergence of immunomodulatory drugs provides the possibility of interfering with specific pathogenic pathways. Novel targeted immunosuppressive therapies might be used in the future for BD.
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Comparative Study
[Clinical, laboratory, radiological features, and outcome in 26 patients with aortic involvement amongst a case series of 63 patients with giant cell arteritis].
Aortic involvement that occurs in temporal arteritis is probably underestimated because it is usually asymptomatic. The characteristics of giant cell arteritis with aortic involvement are still poorly described and the relationship between aortitis and vascular outcome of the disease has not been clearly delineated. The objective of this retrospective study of 63 patients with giant cell arteritis, including 26 with aortic involvement, was to compare the features of patients with and without aortitis, and to assess the contribution of CT-scan and FDG-PET-scan in screening for vascular disease, monitoring, and therapeutic management of patients. ⋯ This case series shows no specific features of aorta and its main roots involvement in giant cell arteritis, justifying a systematic screening by CT-scan. The high frequency of this arterial involvement could help physicians in the diagnosis of giant cell arteritis. Aortitis seems to be associated with vascular complications as highlighted by the frequency of aortic aneurysm and a case of early aortic dissection. Finally, the role of PET-CT-scan for screening vascular disease and therapeutic monitoring remains to be clarified.
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Polymyalgia rheumatica (PMR) is a frequent cause for long-term corticosteroid therapy. Management of PMR is difficult and recommendations (regarding diagnosis and treatment) from the British Society of Rheumatology have been recently published in order to avoid false diagnosis and unnecessary corticosteroid therapy. On the other hand, late onset spondyloarthropathies are difficult to diagnose due to their various presentation (peripheral and axial manifestations, usually associated with severe systemic manifestations) and the absence of validated diagnosis criteria in the elderly. ⋯ Among PMR patients with poor response to corticosteroids and multiple flares, the possibility of a late onset spondyloarthropathy should be discussed. There is an unmet need for validated diagnosis criteria in such patients.
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Recent advances in neuroimaging have simplified the diagnostic criteria of multiple sclerosis. Indeed, the diagnosis of multiple sclerosis could be obtained during the first bout of disease flare, very early in the disease course. ⋯ At the same time, major therapeutic advances have been obtained and new drugs that are well tolerated and more effective, despite the possible rare but potentially severe side effects are been developed. This article reviews the modern diagnostic and therapeutic strategies in multiple sclerosis in accordance with the recent obtained advances.