12 results match your criteria: "Centre de référence des maladies pulmonaires rares (site constitutif)[Affiliation]"

Sarcoidosis and Emergency Hospitalization.

Chest

August 2024

Service de pneumologie, Centre de référence des maladies pulmonaires rares (site constitutif), hôpital Avicenne, Assistance Publique Hôpitaux de Paris, UMR Inserm U1272, Université Sorbonne Paris Nord, Bobigny, France.

Background: Sarcoidosis is an idiopathic systemic granulomatosis whose evolution is self-limiting in most cases. However, it can progress to organ damage that menaces the vital or functional prognosis of patients. Sarcoidosis itself, but also its comorbidities, can pose a threat to the patient, require rapid initiation of treatment, and justify emergency hospitalization.

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Article Synopsis
  • * The majority of patients were women, with 47.2% testing positive for certain antibodies; about 65% had another connective tissue disease, and ILD was typically diagnosed years after the SLE diagnosis.
  • * Despite a generally good prognosis, with high survival rates, nearly half of the patients experienced ILD progression, and factors like cutaneous symptoms were linked to better survival outcomes.
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Background And Aim: Few questionnaires are available for routine assessment of dyspnea. The study aimed to design a self-administered questionnaire assessing the impact of chronic dyspnea on daily activities, named DYSLIM (Dyspnea-induced Limitation).

Methods: The development followed 4 steps: 1: selection of relevant activities and related questions (focus groups); 2: clinical study: internal and concurrent validity vs.

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Background: Standard of care for interstitial lung disease (ILD) with a nonspecific interstitial pneumonia (NSIP) pattern proposes mycophenolate mofetil (MMF) as one of the first-step therapies while rituximab is used as rescue therapy.

Methods: In a randomised, double-blind, two-parallel group, placebo-controlled trial (NCT02990286), patients with connective tissue disease-associated ILD or idiopathic interstitial pneumonia (with or without autoimmune features) and a NSIP pattern (defined on NSIP pathological pattern or on integration of clinicobiological data and a NSIP-like high-resolution computed tomography pattern) were randomly assigned in a 1:1 ratio to receive rituximab (1000 mg) or placebo on day 1 and day 15 in addition to MMF (2 g daily) for 6 months. The primary end-point was the change in percent predicted forced vital capacity (FVC) from baseline to 6 months analysed by a linear mixed model for repeated measures analysis.

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Patients diagnosed with coronavirus disease 2019 (COVID-19) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection frequently experience symptom burden post-acute infection or post-hospitalisation. We aimed to identify optimal strategies for follow-up care that may positively impact the patient's quality of life (QoL). A European Respiratory Society (ERS) Task Force convened and prioritised eight clinical questions.

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Carriers of germline telomerase-related gene (TRG) mutations can show poor prognosis, with an increase in common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT in recipients carrying a germline TRG mutation and to identify the predictors of survival. In a multicenter cohort of LT patients, we retrospectively reviewed those carrying pathogenic TRG variations (n = 38; TERT, n = 23, TERC, n = 9, RTEL1, n = 6) between 2009 and 2018.

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Background: There is growing evidence of gender-specific phenotypic differences among patients with idiopathic pulmonary fibrosis (IPF), which may affect patient outcomes.

Objectives: We present the characteristics of patients with IPF at inclusion in the French Rare Disease Cohort - Interstitial Lung Disease (RaDiCo-ILD) with the aim of characterizing gender-specific phenotypic differences.

Methods: Patients with IPF who were enrolled in the national, multicentre RaDiCo-ILD cohort were included.

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Air pollution and poverty: a deadly combination in idiopathic pulmonary fibrosis?

Eur Respir J

October 2021

Centre de référence des maladies pulmonaires rares (site constitutif), AP-HP, Service de Pneumologie, Hôpital Avicenne - Université Sorbonne Paris Nord, INSERM 1272, «Hypoxie et Poumon: pneumopathies fibrosantes, modulations ventilatoires et circulatoires», Bobigny, France.

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Low income and outcome in idiopathic pulmonary fibrosis: An association to uncover.

Respir Med

July 2021

Centre de Référence des Maladies Pulmonaires Rares (site Constitutif), AP-HP, Service de Pneumologie, Hôpital Avicenne, Bobigny, France; Université Sorbonne Paris Nord, INSERM, 1272, « Hypoxie et Poumon: Pneumopathies Fibrosantes, Modulations Ventilatoires et Circulatoires », Bobigny, France. Electronic address:

Article Synopsis
  • - The study investigates the impact of low income on patients with idiopathic pulmonary fibrosis (IPF), a chronic respiratory disease, hypothesizing that lower income worsens patient prognosis.
  • - Researchers analyzed data from the French COFI cohort, categorizing patients into "low income" and "higher income" groups based on their annual income, and compared survival rates using statistical models.
  • - Results showed that low-income patients had significantly worse progression-free survival and overall survival rates, alongside a higher likelihood of having non-European origin and occupational exposures, underscoring the adverse effects of low income on IPF outcomes.
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Article Synopsis
  • * Each group focused on important topics such as idiopathic interstitial pneumonias, known origin ILDs, granulomatous ILDs, and rare ILDs, highlighting advancements in pathogenesis, diagnosis, and treatment.
  • * Sharing new research and experiences at the Congress is crucial for both physicians specializing in ILDs and patients affected by these rare diseases.
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Adult interstitial lung diseases and their epidemiology.

Presse Med

June 2020

Service de pneumologie, centre de référence des maladies pulmonaires rares (site constitutif), centre de compétence des maladies auto-immunes et systémiques rares, hôpital Avicenne, assistance publique-Hôpitaux de Paris, 93000 Bobigny, France; Inserm 1272, « Hypoxie et Poumon: pneumopathies fibrosantes, modulations ventilatoires et circulatoires », université de Sorbonne Paris Nord, 93000 Bobigny, France. Electronic address:

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