Docteur RAPHAELLE MEUNIER

Bibliographie

• FIP1L1-PDGFRA-Associated Hypereosinophilic Syndrome as a Treatable Cause of Watershed Infarction.

  2021

  ArticleStroke

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Top publications · les plus citées

• 1

  FIP1L1-PDGFRA-Associated Hypereosinophilic Syndrome as a Treatable Cause of Watershed Infarction

  Stroke · 2021

  📚 16 citations🎯 RCR 1.12🔓 Open Access📄 PDF gratuit ↗

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  Background and Purpose: Ischemic stroke has been reported in various conditions associated with eosinophilia. FIP1L1-PDGFRA fusion ([Fip1-like 1-platelet-derived growth factor receptor alpha]; F/P ) leads to the proliferation of the eosinophilic lineage and thus to a clonal hypereosinophilic syndrome that is highly responsive to imatinib. Methods: We previously reported on a nationwide retrospective study of 151 patients with F/P -associated clonal hypereosinophilic syndrome. Patients from this cohort with a clinical history of ischemic stroke (as well as 2 additional cases) were further analyzed to better define their clinical picture and outcomes. Results: Sixteen male patients (median age, 51 [43–59] years) with low-to-intermediate cardiovascular risk were included. Median National Institutes of Health Stroke Scale was 4 (range, 1–6). Most cerebral imaging disclosed multiple bilateral infarctions of watershed distribution (69%). Despite frequent cardiac involvement (50%), cardiac thrombus was evidenced in a single patient and, according to the TOAST classification (Trial of ORG 10172 in Acute Stroke Treatment), 62.5% of strokes were presumably of undetermined etiology. Among the 15 patients treated with imatinib, and after a median follow-up of 4.5 years, stroke recurred in only 3 patients (consisting of either cardio embolic or hemorrhagic events, unrelated to the first episode). Conclusions: F/P + clonal hypereosinophilic syndrome is a diagnosis to consider in patients with unexplained ischemic stroke and hypereosinophilia (especially in the setting of multiple cortical borderzone distribution) and warrants prompt initiation of imatinib.

• 2

  Assessment and comparison of probability scores to predict giant cell arteritis

  Clinical rheumatology · 2024

  📚 10 citations🎯 RCR 3.58Top 13% NIH🔓 Open Access📄 PDF gratuit ↗

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  Abstract Introduction/objectives To assess and compare the performance of the giant cell arteritis probability score (GCAPS), Ing score, Bhavsar-Khalidi score (BK score), color Doppler ultrasound (CDUS) halo count, and halo score, to predict a final diagnosis of giant cell arteritis (GCA). Method A prospective cohort study was conducted from April to December 2021. Patients with suspected new-onset GCA referred to our quaternary CDUS clinic were included. Data required to calculate each clinical and CDUS probability score was systematically collected at the initial visit. Final diagnosis of GCA was confirmed clinically 6 months after the initial visit, by two blinded vasculitis specialists. Diagnostic accuracy and receiver operator characteristic (ROC) curves for each clinical and CDUS prediction scores were assessed. Results Two hundred patients with suspected new-onset GCA were included: 58 with confirmed GCA and 142 without GCA. All patients with GCA satisfied the 2022 ACR/EULAR classification criteria. A total of 5/15 patients with GCA had a positive temporal artery biopsy. For clinical probability scores, the GCAPS showed the best sensitivity (Se, 0.983), whereas the BK score showed the best specificity (Sp, 0.711). As for CDUS, a halo count of 1 or more was found to have a Se of 0.966 and a Sp of 0.979. Combining concordant results of clinical and CDUS prediction scores showed excellent performance in predicting a final diagnosis of GCA. Conclusion Using a combination of clinical score and CDUS halo count provided an accurate GCA prediction method which should be used in the setting of GCA Fast-Track clinics. Key Points• In this prospective cohort of participants with suspected GCA, 3 clinical prediction tools and 2 ultrasound scores were compared head-to-head to predict a final diagnosis of GCA.• For clinical prediction tools, the giant cell arteritis probability score (GCAPS) had the highest sensitivity, whereas the Bhavsar-Khalidi score (BK score) had the highest specificity.• Ultrasound halo count was both sensitive and specific in predicting GCA.• Combination of a clinical prediction tool such as the GCAPS, with ultrasound halo count, provides an accurate method to predict GCA.

• 3

  Comparison of Interstitial Lung Disease Between Antineutrophil Cytoplasmic Antibodies Positive and Negative Patients: A Retrospective Cohort Study

  ACR open rheumatology · 2024

  📚 8 citations🎯 RCR 1.73🔓 Open Access📄 PDF gratuit ↗

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  ObjectivePositive antineutrophil cytoplasmic antibodies (ANCAs) may occur in the setting of interstitial lung disease (ILD), with or without ANCA‐associated vasculitis (AAV). We aim to compare the characteristics and clinical course of patients with ILD and positive ANCA (ANCA‐ILD) to those with negative ANCA.MethodsWe performed a single‐center retrospective cohort study from 2018 to 2021. All patients with ILD and ANCA testing were included. Patient characteristics (symptoms, dyspnea scale, and systemic AAV), test results (pulmonary high‐resolution computed tomography and pulmonary function tests), and adverse events were collected from electronic medical records. Descriptive statistics and the Fisher exact test were used to compare the outcomes of patients with ANCA‐ILD to those with ILD and negative ANCA.ResultsA total of 265 patients with ILD were included. The mean follow‐up duration was 69.3 months, 26 patients (9.8%) were ANCA positive, and 69.2% of those with ANCA‐ILD had another autoantibody. AAV occurred in 17 patients (65.4%) with ANCA‐ILD. In 29.4% of patients, AAV developed following ILD diagnosis. Usual interstitial pneumonia was the most common radiologic pattern in patients with ANCA‐ILD. There was no association between ANCA status and the evolution of dyspnea, diffusing capacity of the lungs for carbon monoxide, and lung imaging. Forced vital capacity improved over time in 42% of patients with ANCA‐ILD and in 17% of patients with negative ANCA (P = 0.006). Hospitalization occurred in 46.2% of patients with ANCA‐ILD and in 21.8% of patients with negative ANCA (P = 0.006). Both groups had similar mortality rates.ConclusionRoutine ANCA testing should be considered in patients with ILD. Patients with ANCA‐ILD are at risk for AAV. More research is required to better understand and manage patients with ANCA‐ILD.

Publications scientifiques (9) — classées par pathologie

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