M BRUNO CRESTANI

Rhumatologue

📍 PARIS CEDEX 18 (75)HospitalierRPPS 10000527134

Enseignement

📊 Reconnaissance scientifique : 84/100📝 923 articles publiés🇫🇷 Publications académiques françaises (8)🎓 6 thèses dirigées

✨ Profil synthétique

IA · 24/05/2026

M. Bruno Crestani est un rhumatologue hospitalier à Paris, avec une production scientifique importante (h-index de 84 et 923 publications). Ses recherches portent principalement sur les maladies pulmonaires interstitielles, la sclérose systémique et l'hypertension pulmonaire. Il a également dirigé plusieurs thèses dans le domaine de la physiologie et de la physiopathologie.

Expertises présumées

Maladies pulmonaires interstitielles
Sclérose systémique
Hypertension pulmonaire
Fibrose pulmonaire idiopathique
Imagerie médicale
Physiopathologie pulmonaire
Génétique des maladies pulmonaires

Synthèse automatique à partir des sources publiques (HAL, OpenAlex, theses.fr, ClinicalTrials.gov, FAI²R, ANS). Pas une évaluation clinique. Le médecin peut corriger via son compte.

✨ Génération du profil synthétique IA en cours…

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Top publications · les plus citées

ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease developed by the task force for connective tissue disease-associated interstitial lung disease of the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR) Endorsed by the European Reference Network on rare respiratory diseases (ERN-LUNG)

Annals of the rheumatic diseases · 2026

📚 29 citations🎯 RCR 29.00🩺 Clinique🔓 Open Access

ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease

The European respiratory journal · 2026

📚 15 citations🎯 RCR 15.00🩺 Clinique🔓 Open Access📄 PDF gratuit ↗

Lire l'abstract Crossref ↓

Background Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs) and is associated with high morbidity and mortality. Clinical practice guidelines to standardise screening, diagnosis, treatment and follow-up for CTD-ILD are of high importance for optimised patient care. Methods A European Respiratory Society and European Alliance of Associations for Rheumatology task force committee, composed of pulmonologists, rheumatologists, pathologists, radiologists, methodologists and patient representatives, developed recommendations based on PICO (Patients, Intervention, Comparison, Outcomes) questions with grading of the evidence according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology and complementary narrative questions agreed on by both societies. For both PICO and narrative questions, the Evidence to Decision framework was used to formulate the recommendations. Results The task force committee concluded with recommendations for 25 PICO and 28 narrative questions, regarding ILD in the context of systemic sclerosis, rheumatoid arthritis (RA), idiopathic inflammatory myopathies, Sjögren disease (SjD), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). In four narrative questions, regarding screening and assessment of risk for ILD progression in MCTD, SjD and SLE and one PICO question regarding pirfenidone in CTD-ILD other than RA-ILD, the task force had insufficient evidence to support recommendations. Screening, diagnostic, monitoring and treatment algorithms were developed based on the recommendations and usual clinical practice. Conclusions We provide practical guidance by evidence-based recommendations to clinicians for each of the CTDs. In many cases there is low certainty or absence of evidence and we encourage further research to fill these gaps.

FGF21 Signaling Exerts Antifibrotic Properties during Pulmonary Fibrosis

American journal of respiratory and critical care medicine · 2025

📚 10 citations🎯 RCR 3.95

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Abstract Rationale Idiopathic pulmonary fibrosis (IPF) is a lethal disease with limited therapeutic options. FGF21 (fibroblast growth factor 21), an endocrine fibroblast growth factor that acts through the FGFR1 (fibroblast growth factor receptor 1)/KLB (β-Klotho) pathway, mitigates liver fibrosis. Objectives We hypothesized that FGF21 could exert antifibrotic properties in the lung. Methods The concentrations of FGF21 and KLB in the plasma of patients with IPF and control subjects were assessed. Pulmonary fibrosis development was assessed in Fgf21-deficient mice compared with wild-type littermates, at Day 14 (D14) after the intratracheal injection of bleomycin. We determined the effect of repeated subcutaneous injections of a PEGylated FGF21 analog at D7, D10, D14, and D17 after bleomycin on the development of pulmonary fibrosis. Mice were killed at D21. The effects of FGF21, alone or with KLB, on apoptosis in murine lung epithelial 15 cells and on the phenotype of human lung fibroblasts were assessed in vitro. Measurements and Main Results In the plasma of patients with IPF, FGF21 concentrations were increased, while KLB concentrations were decreased. Fgf21-deficient mice showed increased sensitivity to bleomycin in comparison with their wild-type littermates. Treatment with PEGylated FGF21 mitigated lung fibrogenesis, as evidenced by a lower injury score and decreased fibrosis markers and profibrotic mediator expression compared with the control group receiving the diluent. In murine lung epithelial 15 cells, stimulation with FGF21 and KLB inhibited apoptosis, through the decrease of BAX and BIM. Fibroblastic phenotype remained unaltered. Conclusions Our data indicate a possible antifibrotic effect of FGF21 in the lung achieved through the inhibition of alveolar type 2 cell apoptosis.

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Top publications · les plus citées

Publications scientifiques (50) — classées par pathologie

Transversal28

Revue générale5

Épidémiologie & registres4

Génétique4

Biothérapies non-anti-TNF2

Pharmacovigilance2

Recommandations2

Essai clinique1

Lupus1

Sjögren1

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