Davide Elia

Access to lymphangioleiomyomatosis care across Europe: an ERN-LUNG survey of clinical practice, and the case for a European registry and trial network

Donal O'Malley, Elisabeth Humbert-Dorfmüller, Joan Albert Barberà, Aernoud van Batenburg et autres

A European LAM Registry would enable standardised data collection, support clinical research and facilitate transnational collaboration, establishing an optimal platform for advancing care and developing a coordinated trial network https://bit.ly/4no2xlr.

Access to lymphangioleiomyomatosis care across Europe: an ERN-LUNG survey of clinical practice, and the case for a European registry and trial network

Donal O'Malley, Elisabeth Humbert-Dorfmüller, Joan Albert Barberà, Aernoud A. van Batenburg et autres

Extract Lymphangioleiomyomatosis (LAM) is a systemic, low grade, metastasizing neoplasm, that predominantly affects women of reproductive age, with an estimated prevalence of 20 cases per million females [1, 2]. LAM occurs either sporadically or in association with tuberous sclerosis complex (TSC), with …

ie, de, es, nl, dk, fr, it, be, gb, gr, ir, ge (code pays fourni par la source)

Characterising patients with pulmonary Langerhans’ cell histiocytosis and pulmonary hypertension

Carmel J. W. Stock, Manuel López‐Meseguer, Berta Laquente Sáez, Davide Elia et autres

The mechanisms for pulmonary Langerhans’ cell histiocytosis associated pulmonary hypertension (PLCH-PH) are incompletely understood and classified as group 5 PH. We aimed to describe characteristics and outcomes of patients with PLCH-PH. Retrospective cohort study from five expert centres. Adult patients with diagnostic …

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Efficacy and limits of Sirolimus therapy in patients with Lymphangioleiomyomatosis: a retrospective study

Sergio Alfonso Harari, Lucia Pacchetti, Davide Elia, Lu Fan et autres

Aim: Lymphangioleiomyomatosis (LAM) is a rare multysistemic disease characterized by cystic lung destruction, renal angiomyolipomas (AMLs), and lymphangioleiomyomas. Sirolimus stabilizes lung function in LAM, but its long-term effects remain unclear. This study evaluates the long-term effect of Sirolimus on respiratory function. Methods: …

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Novel treatment strategies for lymphangioleiomyomatosis: a narrative review

Davide Elia, Sergio Alfonso Harari, Lu Fan, Rémi Diesler et autres

Lymphangioleiomyomatosis (LAM) is a rare multisystemic disease primarily affecting women, manifested as cystic lung destruction, angiomyolipomas (AMLs) and lymphagioleiomyomas. The hallmark of LAM is the presence of abnormal perivascular epithelioid cells, referred to as LAM cells. LAM is classified into tuberous sclerosis-associated …

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Rare pulmonary diseases and pulmonary hypertension

Federico Tagariello, Davide Elia, Sergio Alfonso Harari

PURPOSE OF REVIEW: Pulmonary hypertension (PH) is a significant complication of various lung diseases, including rare conditions such as lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH). This review explores the pathophysiology, diagnostic challenges, and therapeutic strategies for managing PH in these …

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Anti‐neutrophil cytoplasmic antibodies associated interstitial pneumonia: A possible new clinical entity

Claudia Iannone, Maria Rosa Pellico, Antonella Caminati, Maurizio Zompatori et autres

BACKGROUND: Anti-neutrophil cytoplasmic antibodies (ANCA), a hallmark of systemic vasculitis (SV), have been reported in patients with idiopathic interstitial pneumonia (IIP). However, the clinical significance of ANCA in IIP remains unclear. METHODS: We retrospectively studied 101 IP patients diagnosed by pneumologists as …

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Nintedanib for patients with lymphangioleiomyomatosis: a phase 2, open-label, single-arm study

Sergio Alfonso Harari, Davide Elia, Antonella Caminati, Jens Geginat et autres

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TAPSE/sPAP ratio can be used to predict pulmonary hypertension in fibrosing interstitial lung disease

Andrea Sonaglioni, Antonella Caminati, Maurizio Zompatori, Roberto Cassandro et autres

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POS0314 ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODIES ASSOCIATED INTERSTITIAL PNEUMONIA: A NEW CLINICAL ENTITY?

M. R. Pellico, C. Iannone, Antonella Caminati, Lisa Tescaro et autres

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Symptomatic post COVID patients have impaired alveolar capillary membrane function and high VE/VCO2

Piergiuseppe Agostoni, Massimo Mapelli, Elisabetta Salvioni, Irene Mattavelli et autres

Abstract Background Post COVID-19 syndrome is characterized by several cardiorespiratory symptoms but the origin of patients’ reported symptomatology is still unclear. Methods Consecutive post COVID-19 patients were included. Patients underwent full clinical evaluation, symptoms dedicated questionnaires, blood tests, echocardiography, thoracic computer tomography …

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Identification of Lymphangioleiomyomatosis-associated Serum MicroRNAs

Riccardo Lorenzo Rossi, Davide Elia, Olga Torre, Roberto Cassandro et autres

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