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10. Fibrotic Hypersensitivity Pneumonitis: Diagnosis and Management

Author

Varone, Francesco, Iovene, Bruno, Sgalla, Giacomo, Calvello, Mariarosaria, Calabrese, Angelo, Larici, Anna Rita, and Richeldi, Luca

Subjects
Medical research, Medicine, Experimental, Bacterial pneumonia -- Diagnosis -- Care and treatment, Pneumonia -- Diagnosis -- Care and treatment, Health
Abstract

Fibrotic hypersensitivity pneumonitis is a complex interstitial lung disease that is not entirely understood. In its chronic and fibrotic form, hypersensitivity pneumonitis is one of the main mimickers of idiopathic pulmonary fibrosis (IPF). Distinguishing between these two conditions is challenging but is of particular clinical relevance. Two approved therapies are available for IPF, and a considerable number of clinical trials are now exploring newer pharmacological options. This impressive research effort is a consequence of new pathogenetic understanding, updated diagnostic criteria and a long history of pharmacological trials. Conversely, current knowledge gaps on pathogenesis of chronic hypersensitivity pneumonitis, coupled with lack of validated diagnostic criteria, make the management of this disease an unsolved clinical challenge. This also reflects the paucity of therapeutic clinical trials in this field. In this review, we describe the current evidence and the possible future options to approach this complex disease., Author(s): Francesco Varone [sup.1] , Bruno Iovene [sup.1] , Giacomo Sgalla [sup.1] , Mariarosaria Calvello [sup.1] , Angelo Calabrese [sup.1] , Anna Rita Larici [sup.1] [sup.2] , Luca Richeldi [sup.1] [...]

Published
2020
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12. A randomized trial of pamrevlumab in patients with COVID‐19 pneumonia

Author

Sgalla, Giacomo, primary, Leone, Paolo Maria, additional, Gualano, Gina, additional, Simonetti, Jacopo, additional, Comes, Alessia, additional, Verdirosi, Diana, additional, Di Gennaro, Francesco, additional, Larici, Anna Rita, additional, Ianniello, Stefania, additional, Cicchetti, Giuseppe, additional, Fusco, Nicoletta, additional, Pani, Marcello, additional, Palmieri, Fabrizio, additional, and Richeldi, Luca, additional

Published
2023
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13. GATA1-defective immune-megakaryocytes as possible drivers of idiopathic pulmonary fibrosis

Author

Gobbo, Francesca, primary, Zingariello, Maria, additional, Verachi, Paola, additional, Falchi, Mario, additional, Arciprete, Francesca, additional, Martelli, Fabrizio, additional, Peli, Angelo, additional, Mazzarini, Maria, additional, Vierstra, Jeff, additional, Mead-Harvey, Carolyn, additional, Dueck, Amylou C., additional, Sarli, Giuseppe, additional, Nava, Stefano, additional, Sgalla, Giacomo, additional, Richeldi, Luca, additional, and Migliaccio, Anna Rita, additional

Published
2023
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15. Experimental autotaxin inhibitors for the treatment of idiopathic pulmonary fibrosis

Author

Simonetti, Jacopo, Ficili, Marco, Sgalla, Giacomo, and Richeldi, Luca

Abstract

ABSTRACTIntroductionIdiopathic Pulmonary Fibrosis (IPF) is a progressive, irreversible, and fatal lung disease with unmet medical needs. Autotaxin (ATX) is an extracellular enzyme involved in the generation of lysophosphatidic acid (LPA). Preclinical and clinical data have suggested the ATX-LPAR signaling axis plays an important role in the pathogenesis and the progression of IPF.Areas CoveredThe aim of this review is to provide an update on the available evidence on autotaxin inhibitors in IPF and further details on the ongoing clinical studies involving these molecules.Expert OpinionThe development of autotaxin inhibitors as a potential therapy for idiopathic pulmonary fibrosis has gained attention due to evidence of their involvement in the disease. Preclinical and early-phase clinical studies have explored these inhibitors’ efficacy and safety, offering a novel approach in treating this disease. Combining autotaxin inhibitors with existing anti-fibrotic agents is considered for enhanced therapeutic effects. Large phase III trials assessed Ziritaxestat but yielded disappointing results, highlighting the importance of long-term observation and clinical outcomes in clinical research. Patient stratification and personalized medicine are crucial, as pulmonary fibrosis is a heterogeneous disease. Ongoing research and collaboration are essential for this advancement.

Published
2024
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20. Contributors

Author

Antin-Ozerkis, Danielle, primary, Behr, Jürgen, additional, Cerri, Stefania, additional, Danoff, Sonye K., additional, Evans, Janine, additional, Fell, Charlene D., additional, Garcia, Christine Kim, additional, Homer, Robert J., additional, Jones, Kirk D., additional, Kim, Dong Soon, additional, Lindell, Kathleen Oare, additional, Luppi, Fabrizio, additional, Matthay, Richard A., additional, Olson, Amy L., additional, Paoletti, Luca, additional, Puglisi, Silvia, additional, Richeldi, Luca, additional, Rubinowitz, Ami, additional, Ryu, Jay H., additional, Sgalla, Giacomo, additional, Shah, Aditi, additional, Silhan, Leann L., additional, Spagnolo, Paolo, additional, Swigris, Jeffrey J., additional, Urisman, Anatoly, additional, Vancheri, Carlo, additional, Vassallo, Robert, additional, Whelan, Timothy P.M., additional, and Yunt, Zulma X., additional

Published
2018
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28. Road Toward a New Model of Care for Idiopathic Pulmonary Fibrosis in the Lazio Region

Author

Di Bidino, Rossella, primary, Rogliani, Paola, additional, Sebastiani, Alfredo, additional, Ricci, Alberto, additional, Varone, Francesco, additional, Sgalla, Giacomo, additional, Iovene, Bruno, additional, Bruni, Teresa, additional, Flore, Maria Chiara, additional, D'Ascanio, Michela, additional, Cavalli, Francesco, additional, Savi, Daniela, additional, Di Michele, Loreta, additional, Cicchetti, Americo, additional, and Richeldi, Luca, additional

Published
2022
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31. Drug-Induced Interstitial Lung Disease in a patient treated with a combination of palbociclib and fulvestrant.

Author

Vittoria Licata, Maria Angela, Porro, Lucia Maria, Sgalla, Giacomo, and Richeldi, Luca

Subjects
INTERSTITIAL lung diseases, FULVESTRANT, DRUG side effects, ADULT respiratory distress syndrome, METASTATIC breast cancer
Abstract

A 59-year-old patient without a history of pulmonary disease presented with episodes of hemoptysis and acute respiratory failure after receiving fulvestrant and palbociclib for metastatic breast cancer. The High-Resolution chest CT demonstrated diffuse ground glass opacities, as well as diffuse smooth thickening of the interlobular septa and peribronchovascular interstitium, which are consistent with Drug-Induced Interstitial Lung Disease (DIILD). A few days of high-dose steroid therapy improved the patient's gas exchange from PaO2/FiO2 of 75 to 200. After hospital discharge, the oncologist resumed fulvestrant therapy, with no additional adverse events occurring during the subsequent follow-up. [ABSTRACT FROM AUTHOR]

Published
2023
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39. Residual respiratory impairment after COVID-19 pneumonia

Author

Lombardi, Francesco, Calabrese, Angelo, Iovene, Bruno, Pierandrei, Chiara, Lerede, Marialessia, Varone, Francesco, Richeldi, Luca, Sgalla, Giacomo, Landi, Francesco, Gremese, Elisa, Bernabei, Roberto, Fantoni, Massimo, Gasbarrini, Antonio, Settanni, Carlo Romano, Benvenuto, Francesca, Bramato, Giulia, Carfi', Angelo, Ciciarello, Francesca, Lo Monaco, Maria Rita, Martone, Anna Maria, Marzetti, Emanuele, Carmen, Napolitano, Pagano, Francesco Cosimo, Rocchi, Sara, Rota, Elisabetta, Salerno, Andrea, Tosato, Matteo, Tritto, Marcello, Calvani, Riccardo, Catalano, Lucio, Anna, Picca, Giulia, Savera, Tamburrini, Enrica, Borghetti, Alberto, Simona Di Gianbenedetto, Murri, Rita, Cingolani, Antonella, Ventura, Giulio, Taddei, Eleonora, Davide, Moschese, Arturo, Ciccullo, Stella, Leonardo, Addolorato, Giovanni, Franceschi, Francesco, Gertrude, Mingrone, Zocco, Maria Assunta, Mauirizio, Sanguinetti, Cattani Franchi, Paola, Marchetti, Simona, Alessandro, Bizzarro, Lauria, Alessandra, Rizzo, Stanislao, Savastano, Maria Cristina, Gambini, Gloria, Maria Grazia Cozzupoli, Culiersi, Carola, Passali, Giulio Cesare, Paludetti, Gaetano, Galli, Jacopo, Crudo, Fabrizio, Giovanni Di Cintio, Ylenia, Longobardi, Laura, Tricarico, Santantonio, Mariaconsiglia, Buonsenso, Danilo, Valentini, Piero, Davide, Pata, Davide, Sinatti, De Rose, Cristina, Aangelo, Calabrese, Sani, Gabriele, Delfina, Janiri, Giulia, Giuseppin, Marzia, Molinaro, Modica, Marco, Natale, Luigi, Larici, Anna Rita, Marano, Riccardo, Annamaria, Paglionico, Petricca, Luca, Laura, Gigante, Gerlando, Natalello, Fedele, Anna Laura, Lizzio, Marco Maria, Santoliquido, Angelo, Santoro, Luca, Nesci, Antonio, and Valentina, Popolla

Subjects
Male, Pulmonary and Respiratory Medicine, Percent Predicted Total Lung Capacity, Settore M-PSI/02 - PSICOBIOLOGIA E PSICOLOGIA FISIOLOGICA, Partial Pressure, Pneumonia, Viral, Walk Test, 030204 cardiovascular system & hematology, dyspnoea, Severity of Illness Index, Pulmonary function testing, Diseases of the respiratory system, 03 medical and health sciences, PFT, 0302 clinical medicine, Percent Predicted Residual Volume, DLCO, cough, Diffusing capacity, 6MWT, Humans, Medicine, Respiratory function, Lung volumes, Aged, COVID, Carbon Monoxide, Exercise Tolerance, RC705-779, SARS-CoV-2, business.industry, Research, Settore MED/09 - MEDICINA INTERNA, COVID-19, Middle Aged, Oxygen, Residual Volume, Dyspnea, 030228 respiratory system, Respiratory failure, Anesthesia, Pulmonary Diffusing Capacity, Female, ABG, Blood Gas Analysis, business
Abstract

Introduction The novel coronavirus SARS-Cov-2 can infect the respiratory tract causing a spectrum of disease varying from mild to fatal pneumonia, and known as COVID-19. Ongoing clinical research is assessing the potential for long-term respiratory sequelae in these patients. We assessed the respiratory function in a cohort of patients after recovering from SARS-Cov-2 infection, stratified according to PaO2/FiO2 (p/F) values. Method Approximately one month after hospital discharge, 86 COVID-19 patients underwent physical examination, arterial blood gas (ABG) analysis, pulmonary function tests (PFTs), and six-minute walk test (6MWT). Patients were also asked to quantify the severity of dyspnoea and cough before, during, and after hospitalization using a visual analogic scale (VAS). Seventy-six subjects with ABG during hospitalization were stratified in three groups according to their worst p/F values: above 300 (n = 38), between 200 and 300 (n = 30) and below 200 (n = 20). Results On PFTs, lung volumes were overall preserved yet, mean percent predicted residual volume was slightly reduced (74.8 ± 18.1%). Percent predicted diffusing capacity for carbon monoxide (DLCO) was also mildly reduced (77.2 ± 16.5%). Patients reported residual breathlessness at the time of the visit (VAS 19.8, p Conclusion Approximately one month after hospital discharge, patients with COVID-19 can have residual respiratory impairment, including lower exercise tolerance. The extent of this impairment seems to correlate with the severity of respiratory failure during hospitalization.

Published
2021

48. Mediastinal lymph node enlargement in idiopathic pulmonary fibrosis: relationships with disease progression and pulmonary function trends

Author

Sgalla, Giacomo, primary, Larici, Anna Rita, additional, Golfi, Nicoletta, additional, Calvello, Mariarosaria, additional, Farchione, Alessandra, additional, Ciello, Annemilia Del, additional, Varone, Francesco, additional, Iovene, Bruno, additional, Manfredi, Riccardo, additional, and Richeldi, Luca, additional

Published
2020
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