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Rémi Philip Anael Dumont Nicolas Martin Silva Hubert de Boysson Achille Aouba Samuel Deshayes 《Autoimmunity reviews》2021,20(9):102885
IntroductionDouble-positive patients (DPP) exhibiting anti-glomerular basement membrane (GBM) and anti-neutrophil cytoplasmic antibodies (ANCAs) belong to an entity that is newly and poorly described, mainly in short series. We aimed to better characterize the epidemiological features, clinical presentation and therapeutic outcomes of these patients through a systematic review.MethodsWe performed a systematic review of English-, German-, Spanish- and French-written publications from February 1987 to March 2020 reporting cases of DPP using the following databases: PubMed, Scielo, ScienceDirect, Google Scholar, The Cochrane Library, Open Grey, The Grey Literature Report, Clinicaltrials.gov and International Clinical Trial Registry Platform of the World Health Organization.ResultsIn total, 538 DPP were identified from 90 articles. Their clinical presentations were often severe, and the majority exhibited acute kidney failure (91.8%) with a median initial serum creatinine level of 873 μmol/L; 50.7% had alveolar haemorrhage. Other manifestations were present in 30.3% of DPP, mainly ear, nose, throat and articular manifestations. ANCAs were predominantly directed against MPO (n = 377/523; 72.1%) compared to PR3 (n = 107/523; 20.5%), with rare cases of triple positivity (n = 15/538; 2.9%). Although most patients received initial immunosuppressive therapy (n = 285/317; 89.9%), the one-year overall, renal and relapse-free survival rates were 64.8%, 38.7% and 71.1%, respectively.ConclusionDPP are associated with the characteristics of two eponymous vasculitis types, responsible for a poor overall and renal prognosis. Thus, simultaneous testing of both antibodies and systematic renal biopsy should be recommended in every patient with rapidly progressive glomerulonephritis to recognize this difficult-to-treat and rare disease. 相似文献
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Bozec Alexandre Schultz Philippe Gal Jocelyn Chamorey Emmanuel Chateau Yann Dassonville Olivier Poissonnet Gilles Peyrade Frédéric Saada Esma Guigay Joël Benezery Karen Leysalle Axel Santini Laure Giovanni Antoine Messaoudi Lila Fakhry Nicolas 《European archives of oto-rhino-laryngology》2019,276(9):2531-2539
European Archives of Oto-Rhino-Laryngology - Providing cancer patients with adequate information is essential to their confidence and satisfaction regarding medical care. The aims of this study... 相似文献
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Guignard Nicolas Chambon Guillaume Chambert Benjamin Najaf Yaser Lallemant Benjamin 《European archives of oto-rhino-laryngology》2019,276(5):1541-1544
European Archives of Oto-Rhino-Laryngology - Characterization of thyroid nodules is crucial to propose surgical intervention for histological verification. Cervical ultrasound potentially combined... 相似文献
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François-Régis Duss Katia Jaton Peter Vollenweider Nicolas Troillet Gilbert Greub 《Clinical microbiology and infection》2021,27(6):910.e9-910.e13
Our institution has performed microbiological diagnosis of Tropheryma whipplei since 2001, initially with a PCR targeting 16S rRNA before the development of a quantitative PCR in 2012. Here we report the clinical characteristics of a cohort of patients suffering from Whipple disease (WD) and evaluate the impact of these molecular techniques. Patients with a positive PCR for T. whipplei between 2001 and 2016 were retrospectively collected from microbiological databases. Two infectious diseases specialists reviewed their medical records and classified them as definite WD, probable WD or carriage of T. whipplei without disease. A total of 1153 samples were tested for T. whipplei; 76 samples taken from 36 patients were positive. Fifteen were considered as presenting a definite WD, seven as a probable WD and 14 as carriers. Median age was 56.4 years (extremes, 6.6–76.1). Median time from symptoms to diagnosis was 3 years (2.5 months to 13.3 years). About 60% were immunosuppressed. The most frequent clinical presentations were joint pain (16/22), weight loss (15/22) and/or digestive tract disorder (15/22); 41% had neurological manifestations, 32% pulmonary involvement and 32% lymphadenopathies. Bacterial load in faeces or saliva were 88 425 copies/mL (IQR 6175-292 725) in definite and probable WD and 311 copies/mL (IQR 253–2090) in carriers, respectively. We observed a 90% PPV above 32 200 copies/mL in faeces. WD is a chronic multisystemic disease with frequent pulmonary involvement. Underlying immunodeficiency is commonly observed leading to more complex clinical presentation. Positive T. whipplei PCR in both stool and saliva has a high positive predictive value. Moreover, patients with WD present higher bacterial load in faeces with a threshold of >32 200 copies/mL predicting ongoing infection. 相似文献
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