Publications by authors named "Uzunhan Y"
Background: Interstitial lung disease (ILD) is rarer in children (chILD) than adults, but with increasing diagnostic awareness, more cases are being discovered. chILD prognosis is often poor, but increasing numbers are now surviving into adulthood.
Aim: To characterize chILD-survivors and identify their impact on adult-ILD centers.
Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease that may progress towards pulmonary fibrosis. Data about fibrosis prevalence and risk factors are lacking.
Methods: In this retrospective multicentre nationwide cohort, we included patients newly diagnosed with aPAP between 2008 and 2018 in France and Belgium.
Background: Despite antifungal treatment, chronic pulmonary aspergillosis (CPA) is associated with substantial morbidity and mortality. We conducted a systematic review and meta-analysis to evaluate rates of mortality and its predictors in CPA.
Methods: A systematic literature search was conducted across MEDLINE (PubMed), Scopus, Embase, and Web of Science to identify studies in English, reporting mortality in CPA, from database inception to Aug 15, 2023.
Background: The PEXIVAS (Plasma exchange and glucocorticoids in severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis) trial showed that a reduced-dose glucocorticoid regimen (redGC) was non-inferior to a standard-dose regimen (standGC) with respect to death or end-stage kidney disease (ESKD) in patients with ANCA-associated vasculitis (AAV). However, the primary endpoint did not include disease progression or relapse, cyclophosphamide was the main induction therapy and rituximab (RTX)-treated patients tended to have a higher risk of death or ESKD with redGC. We aimed to evaluate the real-world use of redGC.
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- Interstitial lung disease (ILD) is a common complication in primary Sjögren's disease (pSD), but its long-term impact on lung function and patient outcomes is not well understood.
- A study of 73 pSD-ILD patients over an average of 9.3 years found that while lung function was generally stable, a significant portion experienced declines, particularly those with specific health characteristics.
- Key findings include two groups with different lung function trajectories: one with stable lung function and one with a decline of approximately 2.4% per year, where the latter group faced higher risks of death or needing a lung transplant.
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.
View Article and Find Full Text PDFBecause Systemic Lupus Erythematosus (SLE) is a rare disease, and due to the significant prognostic impact of early management, a diagnosis confirmed by a physician with experience in SLE is recommended, for example from an expert center. Once the diagnosis is confirmed, existing manifestations should be identified in particular, renal involvement by an assessment of proteinuria, disease activity and severity should be determined, potential complications anticipated, associated diseases searched for, and the patient's socioprofessional and family context noted. Therapeutic management of SLE includes patient education on recognizing symptoms, understanding disease progression as well as when they should seek medical advice.
View Article and Find Full Text PDFObjectives: The aim of this study was to determine the association between different histological patterns and prognosis in patients with SSc and histologically proven muscle involvement.
Methods: A multicentre retrospective study was conducted of a cohort of scleroderma patients who had undergone muscle biopsy. The biopsies were reviewed in a coordinated manner to classify patients based on histological findings.
Article Synopsis
- Acid sphingomyelinase deficiency (ASMD) is a serious genetic disease that can affect people from childhood to adulthood, caused by problems with a gene called SMPD1.
- A study reviewed medical records from 27 hospitals in France to learn more about the health and survival of patients with ASMD from 1990 to 2020.
- The results showed that patients with type A usually did not live past early childhood, while those with type B lived longer, but there were still risks of early death from serious illnesses like neurodegeneration and cancer.
Article Synopsis
- - Relapsing polychondritis is an autoimmune disease primarily affecting cartilage, causing inflammation and deformation through periods of flare-ups and remission.
- - The condition severely impacts auricular and nasal cartilage, and can also involve the tracheobronchial and cardiac areas, making diagnosis complex due to overlapping symptoms with other inflammatory diseases.
- - Recent advancements in diagnostic methods and treatments, including immunosuppressants and targeted therapies, have significantly improved the prognosis for patients with this condition.
Background: Several rare surfactant-related gene (SRG) variants associated with interstitial lung disease are suspected to be associated with lung cancer, but data are missing. We aimed to study the epidemiology and phenotype of lung cancer in an international cohort of SRG variant carriers.
Methods: We conducted a cross-sectional study of all adults with SRG variants in the OrphaLung network and compared lung cancer risk with telomere-related gene (TRG) variant carriers.
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.
Sarcoidosis is an independent risk factor for venous thromboembolism (VTE). However, the characteristics and clinical evolution of sarcoidosis patients presenting a VTE (sarcoidosis/VTE group) in the course of their disease are not known. Consequently, if VTE occurrence is associated with a more severe disease is still pending.
View Article and Find Full Text PDFLow-cost air quality portable sensors and their potential use in respiratory health.
Int J Tuberc Lung Dis
November 2023
Air pollution is an environmental risk for the general population and for patients with various diseases, particularly respiratory diseases. Little data are available on personal exposure, but the recent emergence of low-cost air quality sensors (LCSs) should enable a better understanding of the health impacts of air pollution at the individual level. However, the reliability and accuracy of most sensors in the market have not been established, and a thorough understanding of their strengths and limitations is needed.
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- Recent studies show that using stented cryopreserved aortic allografts for tracheobronchial replacement is effective, especially for patients with severe tracheal cancer.
- The study analyzed 13 patients with extensive tracheal cancer from a larger registry, focusing on their surgical outcomes and complications.
- Findings indicate that the procedure had no 30-day mortality, a high rate of complete resection, and 84.6% of patients were alive at the last follow-up, although some experienced late complications.
Background: Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease, predisposing to an increased risk of infection. A complete picture of these infections is lacking.
Research Question: Describe the characteristics and clinical outcomes of patients diagnosed with aPAP, and to identify risk factors associated with opportunistic infections.
[Interventionnal bronchoscopy for the treatment of tracheobronchomalacia].
Rev Mal Respir
October 2023
Tracheobronchomalacia is usually characterized by more than 50% expiratory narrowing in diameter of the trachea and the bronchi. The expiratory collapse includes two entities: (1) the TBM related to the weakness of the cartilaginous rings, and (2) the Excessive Dynamic Airway Collapse (EDAC) due to the excessive bulging of the posterior membrane. Patients have nonspecific respiratory symptoms like dyspnea and cough.
View Article and Find Full Text PDFObjective: To investigate the association of air pollution exposure with the severity of interstitial lung disease (ILD) at diagnosis and ILD progression among patients with systemic sclerosis (SSc)-associated ILD.
Methods: We conducted a retrospective two-center study of patients with SSc-associated ILD diagnosed between 2006 and 2019. Exposure to the air pollutants particulate matter of up to 10 and 2.
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.
Background: Interstitial lung disease (ILD) is the leading cause of mortality in systemic sclerosis (SSc).
Objective: We performed an overview of the diagnostic approaches, follow-up and treatment strategies used in France for the management of SSc-associated ILD (SSc-ILD).
Design Structured Nationwide Online Surveymethods: A structured nationwide online survey was submitted to participants the French Medical Societies for Internal Medicine and Pneumology, and research groups on SSc-ILD from May 2018 to June 2020.