Search Results (5)

Background and Objectives: Usual interstitial pneumonia (UIP) is associated with progressive fibrosing interstitial lung diseases (PF-ILD) and poor survival in patients with fibrosing interstitial lung disease (FILD). We aimed to investigate the predictors of PF-ILD and survival in patients with FILD-UIP. Materials and Methods: This retrospective study was conducted at a single, tertiary hospital in China. Patients underwent routine follow-up visits every 3 to 6 months according to standard operating procedures (SOPs). Patients with FILD-UIP were further stratified using the proposed PF-ILD criteria. Results: This retrospective study enrolled 150 patients with FILD-UIP between October 2020 and June 2025, with 117 patients completing follow-up for more than 12 months. FILD-UIP was categorized as idiopathic pulmonary fibrosis (IPF) (n = 67) and non-IPF-UIP (n = 50), which included connective tissue disease-associated UIP (n = 29), hypersensitivity pneumonitis-associated UIP (n = 7), and interstitial pneumonia with autoimmune features-associated UIP (n = 14). During the follow-up period, 32 (47.8%) patients with IPF and 19 (38.0%) non-IPF-UIP experienced PF-ILD. Pulmonary hypertension (PH) and predicted percentage of forced vital capacity (FVC%pred) were dependent risk factors for PF-ILD in patients with FILD-UIP, non-IPF-UIP, and IPF. King’s Brief Interstitial Lung Disease (KBILD) is a dependent risk factor for PF-ILD in patients with FILD-UIP and IPF. PF-ILD is similarly associated with poor survival in patients with FILD-UIP, non-IPF-UIP, and IPF. Conclusions: Baseline disease severity is closely associated with the incidence of PF-ILD, with all forms of FILD-UIP at risk of PF-ILD and showing similar outcomes to IPF-UIP/PF-ILD. Full article

Tenascin-C (TNC) is an extracellular matrix (ECM) protein with key roles in various biological processes, such as embryonic development and tissue regeneration. However, its deregulated expression can contribute to pathological responses, promoting chronic inflammation, fibrosis, or tumor progression. It belongs to the tenascin family, a class of extracellular proteins that interfere with cellular events in both physiological and pathological contexts, interacting specifically with cells and other components of the ECM. TNC has emerged as a key player in the pathogenesis of chronic respiratory diseases (CRDs), including asthma, chronic obstructive pulmonary disease (COPD), lung cancer (LC), pulmonary hypertension (PH), and idiopathic pulmonary fibrosis (IPF). The influence of TNC on cellular responses, which is mediated by precise interactions with cellular receptors and ligands, triggers complex intracellular signaling cascades associated with the inflammatory response, fibrosis, and tumorigenesis in these CRDs. This review synthesizes recent evidence highlighting the multifaceted roles and underlying mechanisms of TNC in the context of these CRDs. Full article

Pulmonary hypertension (PH) associated with interstitial lung disease (ILD) (PH-ILD) significantly worsens clinical symptoms and survival, with no effective treatment available. This case report presents the successful treatment of PH-ILD with inhaled iloprost in a patient with idiopathic pulmonary fibrosis (IPF). The patient, a 68-year-old female, was diagnosed with IPF in 2018 and was maintained on pirfenidone. She experienced stable disease until March 2023, when she developed progressive exertional dyspnea, despite stability indicated by a computed tomography (CT) scan, without progression. Transthoracic echocardiography (TTE) and right heart catheterization (RHC) confirmed PH-ILD with a mean pulmonary artery pressure (mPAP) of 43 mmHg. Due to the ineffectiveness of sildenafil and a CT scan indicating stable IPF, a repeat RHC was performed, which showed a worsening of PH (mPAP 62 mmHg). Consequently, inhaled iloprost, at a dosage of 10 mcg every eight hours, was added to the existing antifibrotic agent. After two months, the patient experienced reduced exertional dyspnea and home oxygen requirements. By the seventh month, pulmonary function tests, the six-minute walk test, and RHC parameters (mPAP 37 mmHg) showed marked improvements. This case suggests that inhaled iloprost may be beneficial for managing PH-ILD. Further research is needed to confirm the efficacy of iloprost in PH-ILD treatment. Full article

Background: Pulmonary hypertension (PH) is a well-established complication in interstitial lung disease (ILD) patients. The aim of this study is to investigate the physiological and hemodynamic parameters that predict mortality in patients with ILD-PH. Methods: Consecutive ILD patients who underwent right heart catheterization (n = 340) were included. The information analyzed included demographics and physiological and hemodynamic parameters. Cox regression models were used to identify independent predictors of survival. Results: In total, 96 patients had PH and an additional 56 patients had severe PH. The overall survival of idiopathic pulmonary fibrosis (IPF) patients with PH was significantly worse than the survival of patients with other types of ILD with PH (p < 0.0001 by log-rank analysis). Patients with a reduced diffusing capacity of the lung for carbon monoxide (DLco) (<35% predicted), six-minute walk test final oxygen saturation by pulse oximetry (SpO₂) < 88% and pulmonary vascular resistance ≥4.5 Wood units in the ILD-PH cohort had significantly worse survival. IPF diagnosis, forced vital capacity, DLco, systolic pulmonary artery pressure and cardiac index were identified as independent predictors of survival among the ILD-PH cohort. Conclusions: Patients with ILD-PH have poor prognosis. Physiological and hemodynamic parameters were important factors independently associated with outcome. Full article

Background: Pulmonary hypertension (PH) is a common complication of idiopathic pulmonary fibrosis (IPF) that significantly contributes to morbidity and mortality. Macrophage migration inhibitory factor (MIF) is a critical factor in vascular remodeling of the pulmonary circulation. Objectives: We tested the effects of two small molecules targeting MIF on bleomycin (BLM)-induced collagen deposition, PH, and vascular remodeling in mouse lungs. Methods: We examined the distribution pattern of MIF, CD74, and CXCR4 in the lungs of patients with IPF-PH and the lungs of BLM-injected mice. Then, treatments were realized with (S,R)-3-(4-hydroxyphenyl)-4,5-dihydro-5-isoxazole acetic acid methyl ester (ISO-1) and N-(3-hydroxy-4-fluorobenzyl)-5 trifluoromethylbenzoxazol-2-thione 31 (20 mg/kg/day per os for 3 weeks) started 24 h after an intratracheal BLM administration. Results: More intense immunoreactivity was noted for MIF, CD74, and CXCR4 in lungs from IPF-PH patients and BLM-injected mice. Furthermore, we found that treatments of BLM-injected mice with ISO-1 or compound 31 attenuated lung collagen deposition and right ventricular systolic pressure increase. Additionally, reduced pulmonary inflammatory infiltration and pulmonary arterial muscularization were observed in the lungs of BLM-injected mice treated with ISO-1 or compound 31. Conclusions: Treatments with ISO-1 or compound 31 attenuates BLM-induced inflammation and fibrosis in lung, and prevents PH development in mice, suggesting that MIF is an important factor for IPF-PH development. Full article