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E. Liozon K.H. LyE. Vidal-Cathala A.-L. Fauchais 《La Revue de médecine interne / fondée ... par la Société nationale francaise de médecine interne》2014
Introduction
A malignancy must be carefully excluded before ruling in the diagnosis of adult onset Still's disease (AOSD). However, an occult or poorly symptomatic malignancy can easily be overlooked.Case report
We report a 50-year-old female patient who presented with features of adult onset Still's disease (AOSD), in fact heralding a malignant melanoma with fatal outcome since discovered lately, at a metastatic stage. In retrospect, the only significant atypical feature was cholestatic hepatitis, which soon disappeared upon institution of glucocorticoid treatment. The literature review identified 27 additional cases of AOSD-like disease associated with malignancy published since 1980 including solid cancer in 61% of the cases (especially breast and lung) and haematological malignancies in 39% of the cases (especially malignant lymphoma). The interval between OASD-like symptoms and malignancy averaged 8 months, and AOSD most often preceding malignancy. Although idiopathic AOSD and neoplastic AOSD-like disease are often indistinguishable initially, some features could point toward the latter: an onset of AOSD after the age of 40 years, the presence of atypical clinical, biological, or immunological features in less than one third of the cases, and a poor response to NAIDS or systemic glucocorticoids in 61% of the cases.Conclusion
Making the differential diagnosis of malignancy-associated AOSD in a timely fashion remains a primary goal, even in the most typical cases and those showing good initial therapeutic response. 相似文献48.
P. Kieffer O. HinschbergerE. Ciobanu F. Jaeger-BizetA. Drabo T. MostoufizadehL. Martzolff 《La Revue de médecine interne / fondée ... par la Société nationale francaise de médecine interne》2014
Introduction
Treatment of giant cell arteritis is based on prolonged corticosteroid therapy but adverse side effects are common especially in the elderly.Case reports
We report three patients with giant cell vasculitis treated by tocilizumab, an interleukin-6 receptor antibody, owing to resistance or intolerance to corticosteroid therapy. A favorable outcome was rapidly observed both on clinical and biological data allowing a corticoid therapy sparing.Conclusion
Tocilizumab is a promising treatment of giant cell arteritis but controlled trials are needed to confirm its efficacy. 相似文献49.
M.H. Houman N. Bel Feki 《La Revue de médecine interne / fondée ... par la Société nationale francaise de médecine interne》2014
Although the precise pathogenesis and etiology of Behçet's disease (BD) still remains unknown, current evidence suggests that inflammatory reaction in BD arises from disruption of homeostasis in genetically susceptible individuals, resulting in altered innate and adaptive immunity responses, pathogenic T cell activation in the peripheral blood, and in inflammatory sites. Association with HLA-B51 is known as the strongest genetic susceptibility factor for BD. Recent GWAS (genome-wide association studies) have confirmed this relationship, and reported new susceptibility genes (IL-10, IL-23R, IL-12RB2) for the disease. A triggering infectious agent could operate through molecular mimicry, and the disease could subsequently be perpetuated by an abnormal immune response to an auto-antigen in the absence of ongoing infection. Several potential bacteria have been investigated but the most commonly implicated microorganism is Streptococcus sanguis. Recent data have showed that the T cell homeostasis perturbation consisted mainly of Th1 and Th17 expansions, while regulatory T cell response was suppressed. Cytokine such as IL-17, IL-23 and IL-21 play a significant role in the pathogenesis of BD. Inflammatory cells within BD inflammatory lesions include mostly neutrophils, CD4+ T cells, and cytotoxic cells. Lastly, endothelium dysfunction has been clearly established. This improved understanding of the pathophysiology of BD will certainly lead to the development of new therapeutic agents, potentially more effective than current therapy. In this review, we have studied the etiopathogenesis of BD in the light of recent advances. 相似文献
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N. Ene P. Bélénotti A. Benyamine S. Sovaila M.H. Ben Sahla Talet P. Kaminsky J. Serratrice P.-J. Weiller 《La Revue de médecine interne / fondée ... par la Société nationale francaise de médecine interne》2014