Search Results (13)

Background: Hypersensitivity pneumonitis (HP) is an interstitial lung disease (ILD) caused by repeated exposure to inhaled antigens in susceptible subjects. High-resolution computed tomography (HRCT) of the lungs is the leading diagnostic method for ILDs, but in some cases HRCT findings are not sufficient to distinguish HP and other ILDs, particularly, fibrotic HP (fHP) and usual interstitial pneumonia (UIP). Objective: The aim of this study was to develop HRCT criteria to diagnose fHP in patients with a UIP-like pattern. Methods: In this retrospective study, we analyzed HRCT scans of patients with fHP and a UIP-like pattern who underwent lung biopsy, and patients with idiopathic pulmonary fibrosis (IPF) and a UIP pattern in HRCT. Results: We included 51 patients with confirmed fHP and 24 patients with IPF/UIP in the analysis. IPF/UIP patients were older, were prevalently males, and did not have any systemic autoimmune diseases or risk factors for other ILDs. fHP patients were younger, with an equal number of males and females, and were more likely to be exposed to environmental antigens. HRCT abnormalities in the fHP group predominated in the lower lung areas or were diffuse in axial scans, whereas IPF/UIP patients mostly demonstrated a diffuse craniocaudal distribution and subpleural axial predominance. Centrilobular nodules and mosaic attenuation were present significantly more often in the fHP group; honeycombing, traction bronchiectasis, and emphysema prevailed in IPF/UIP patients. In the logistic regression analysis, patients with fHP and IPF/UIP differed in the presence of centrilobular nodules, honeycombing, and in both craniocaudal and axial distributions of HRCT abnormalities. In the ROC analysis, the combination of centrilobular nodules, honeycombing, and diffuse axial and craniocaudal distributions can predict the diagnosis of fHP (AUC, 0.953 ± 0.022; 95%CI, 0.910–0.995; p < 0.001). Mosaic attenuation and reticulation did not change the probability of fHP. Conclusions: The most significant HRCT features of fHP compared to the UIP pattern were centrilobular nodules, honeycombing, and a diffuse axial and craniocaudal distribution of abnormal findings. Reticulation, mosaic attenuation, and GGO do not increase the probability of fHP. Full article

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a major impact on respiratory function, but also with possible underestimated effects on cognitive function. Although interest in cognitive impairment in chronic respiratory diseases, such as COPD, has increased, data on IPF remain limited and heterogeneous. Objective: This systematic review aimed to synthesize current evidence on cognitive impairment in IPF, identify the most affected domains, and evaluate the certainty of evidence using standardized methodological tools. Methods: A systematic review was conducted according to PRISMA 2020, with a registered PROSPERO protocol (CRD420251041866). Four databases (PubMed, Scopus, Web of Science, Cochrane Library) were searched for studies from 2014 to 2025. Methodological quality and certainty of evidence were appraised with the Joanna Briggs Institute (JBI) and GRADE frameworks. Results: Four studies met the inclusion criteria (two cross-sectional, one descriptive, one case–control). Across investigations, working and verbal memory emerged as the most consistently impaired domains, followed by processing speed and executive function, whereas visuospatial and language abilities were less frequently affected. Cognitive impairment was present even in mild IPF and became more pronounced with lower DLCO, shorter 6 min walk distance, greater desaturation, and obstructive sleep apnea. Certainty of evidence ranged from low to moderate due to small samples and heterogeneous testing. Conclusions: Cognitive dysfunction, particularly in memory, attention, and executive domains, is a frequent but under-recognized feature of IPF. Routine screening with brief, validated tools such as the MoCA may facilitate early detection and guide individualized rehabilitation. Full article

Background: Combined Pulmonary Fibrosis and Emphysema (CPFE) is a distinct syndrome characterized by upper-lobe emphysema and lower-lobe fibrosis, predominantly in older male smokers. Despite often preserved spirometric volumes, patients exhibit severely reduced diffusing capacity and high susceptibility to complications, including pulmonary hypertension (PH), acute exacerbations, and lung cancer, contributing to poor prognosis. Purpose: This review aims to synthesize current evidence on CPFE, focusing on clinical phenotype, functional impairment, differential diagnosis, complications, and emerging management strategies, highlighting distinctions from idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD). Methods: A narrative review of observational cohorts, retrospective series, and clinical studies examining CPFE patients was performed. Data on demographics, smoking history, symptomatology, pulmonary function, radiology, comorbidities, complications, and treatment approaches were extracted and integrated. Results: CPFE affects mainly males aged 65–70, with >90% reporting > 40 pack–years smoking history. Dyspnea is the cardinal symptom (>95%), often disproportionate to preserved FVC and TLC, accompanied by chronic cough in 30–70%. Exercise-induced desaturation is frequent, correlating with PH, observed in 47–90% of patients. Pulmonary function tests reveal preserved volumes, normal or near-normal FEV1/FVC, and severely reduced DLCO (35–45%), distinguishing CPFE from COPD and IPF. HRCT confirms the combined emphysematous and fibrotic pattern, critical for differential diagnosis. Acute exacerbations occur in 20–28% of cases, lung cancer in 22–46% (mostly squamous cell), and long-term oxygen therapy is required in >70%. Five-year survival is 35–55%, lower than emphysema alone and comparable or worse than IPF. Management focuses on smoking cessation, antifibrotics, oxygen therapy, and complication-specific treatments, and selected patients may undergo lung transplantation. Conclusions: CPFE is a clinically and functionally unique entity with a high burden of pulmonary and systemic complications. Accurate recognition using HRCT and DLCO, along with early intervention and tailored management, is essential to improve patient outcomes and guide prognostic stratification. Full article

Background/Objectives: Idiopathic pulmonary fibrosis (IPF) registries are established to enhance understanding of its natural history. Methods: Serbia (RS) participated in the EMPIRE (European Multi-Partner IPF Registry) from June 2015 to October 2022, involving four centers. The registry included patients over 18 diagnosed with IPF based on the 2011 international criteria. We aimed to gather key clinical, functional, and survival data, along with treatment information for IPF patients in RS, using a centralized electronic case report for consistency. Results: 188 RS patients participated (median age at diagnosis 65, 63.8% male, 51% smoking history, 56% radiological usual interstitial pneumonia (UIP) pattern). At the diagnosis, median forced vital capacity (FVC) was 73.7% and diffusion capacity for carbon monoxide (DL_CO) was 38%. At initiation of antifibrotic therapy, median FVC was 73.2% (71.5% for deceased, 75.8% for survivors (p = 0.455), and DL_CO was 33.8% (19.9% for deceased, and 35.6% for survivors (p = 0.046)). The median long-term survival from diagnosis was 29.4 months (95% CI: 22.6–36.2 months), and 9.4 months (95% CI: 5.9–12.9 months) from the initiation of therapy, with no difference in the duration of antifibrotic treatment between survivors and deceased (p = 0.598). Conclusions: The RS EMPIRE cohort represents a younger, less comorbid population with fewer smokers and more probable UIP, factors linked to a favorable prognosis. Nevertheless, survival was poorer than expected, mainly due to advanced disease severity at the time of antifibrotic initiation, as indicated by lower DL_CO. These findings highlight the importance of earlier diagnosis and treatment before significant physiological decline to improve outcomes. Full article

The accurate selection of the recipient is a crucial aspect in the field of lung transplantation (LTX), especially if patients were previously affected by oncological disease. The aim of this bicentric retrospective study was to evaluate short- and long-term outcomes in patients with previous oncological disease or unknown neoplasia found on native lungs submitted to LTX, compared to a control group. A total of 433 patients were included in the analysis, 31 with malignancies (Group 1) and 402 without neoplastic disease (Group 2). The two groups were compared in terms of short- and long-term outcomes. Patients in Group 1 were older (median age 58 years vs. 50 years, p = 0.039) and mostly affected by idiopathic pulmonary fibrosis (55% vs. 40% p = 0.002). Even though in Group 1 a lower rate of late post-operative complications was found (23% vs. 45%, p = 0.018), the median overall survival (OS) was lower compared to the control group (10 months vs. 29 months, p = 0.015). LTX represents a viable therapeutic option for patients with end-stage lung disease and a history of neoplastic disease. However, every case should be carefully debated in a multidisciplinary setting, considering oncological (histology, stage, and proper disease free-interval) and clinical factors (patient’s age and comorbidities). A scrupulous post-transplant follow-up is especially mandatory in those cases. Full article

Interstitial lung diseases (ILDs) constitute a group of more than 200 disorders, with idiopathic pulmonary fibrosis (IPF) being one of the most frequent. Telomere length (TL) shortening causes loss of function of the lung parenchyma. However, little is known about its role as a prognostic factor in ILD patients. With the aim of investigating the role of TL and telomerase activity in the prognosis of patients affected by ILDs, we analysed lung tissue samples from 61 patients. We measured relative TL and telomerase activity by conventional procedures. Both clinical and molecular parameters were associated with overall survival by the Kaplan–Meier method. Patients with IPF had poorer prognosis than patients with other ILDs (p = 0.034). When patients were classified according to TL, those with shortened telomeres reported lower overall survival (p = 0.085); differences reached statistical significance after excluding ILD patients who developed cancer (p = 0.021). In a Cox regression analysis, TL behaved as a risk-modifying variable for death associated with rheumatic disease (RD) co-occurrence (p = 0.029). Also, in patients without cancer, ferritin was significantly increased in cases with RD and IPF co-occurrence (p = 0.032). In relation to telomerase activity, no significant differences were detected. In conclusion, TL in lung tissue emerges as a prognostic factor in ILD patients. Specifically, in cases with RD and IPF co-occurrence, TL can be considered as a risk-modifying variable for death. Full article

Pirfenidone and nintedanib are the only two drugs approved for the treatment of idiopathic pulmonary fibrosis (IPF). Both proved to be safe and well-tolerated in clinical trials, but real-world data and direct comparisons are scarce. This real-life study explored the safety profile of pirfenidone and nintedanib with a prolonged follow-up. We retrospectively collected clinical status, adverse events (AEs), and treatment changes from IPF patients who had started an antifibrotic treatment at our centre from December 2011 to December 2020, including 192 patients treated with pirfenidone and 89 with nintedanib. The majority of patients in both groups experienced one or more AEs during the follow-up. A higher proportion of AEs in the nintedanib group were effectively treated with behavioural modifications or additional medications compared with the pirfenidone group (52.5% vs. 40.6%, p = 0.04). Overall, a difference in the impact of AEs due to nintedanib versus pirfenidone resulted in a lower permanent discontinuation of therapy (8.3% vs. 18.3%, p = 0.02), with the latter being associated with a higher risk of drug discontinuation at 48 months after initiation (OR = 2.52, p = 0.03). Our study confirms the safety profile of antifibrotic drugs in IPF but highlights that AEs due to nintedanib are usually easier to manage and lead to fewer cases of permanent discontinuation of therapy. Full article

Background: The coupling of the right ventricle (RV) to the pulmonary circulation is an indicator of RV performance that can be non-invasively estimated by echocardiography. There are no data about its use in patients affected by fibrotic interstitial lung diseases (f-ILD). Methods: Fifty f-ILD patients, including 27 cases with idiopathic pulmonary fibrosis (IPF) (M = 37; mean age 67 ± 7 years), were studied with standard and speckle-tracking echocardiography and compared with 30 age-matched healthy volunteers. The mean patient follow-up was 70 ± 4 months. Results: Fibrotic ILD patients had a larger right ventricle (RV) and worse diastolic function because the RV global longitudinal strain (GLS) was significantly lower and the systolic pulmonary artery pressure (sPAP) estimates were higher in comparison with those of controls. Conversely, tricuspid annular systolic excursion (TAPSE) did not differ between controls and patients. Median values of TAPSE/sPAP and RV GLS/sPAP were significantly reduced in f-ILD patients (p < 0.0001). Patients with an RV GLS/sPAP below the median value had a shorter survival time (61 vs. 74 months, p = 0.01); this parameter was an independent predictor of a worse outcome. Conclusion: Low estimates of RV GLS/sPAP are predictive of worse outcomes in f-ILD patients. RV coupling seems to be a promising surrogate biomarker of RV performance to discriminate the patient phenotype with significant management and prognosis implications. Full article

(1) Background: We explored, for the first time, the contribution of angiogenic T cells (TAng) in interstitial lung disease associated to autoimmune disease (AD-ILD⁺) as potential biomarkers of the disease, evaluating their role in the underlying vasculopathy and lung fibrosis. Additionally, the relationship of TAng with clinical manifestations and cellular and molecular endothelial dysfunction-related biomarkers was assessed. (2) Methods: We included 57 AD-ILD⁺ patients (21 with rheumatoid arthritis (RA)-ILD⁺, 21 with systemic sclerosis (SSc)-ILD⁺ and 15 with other AD-ILD⁺) and three comparative groups: 45 AD-ILD⁻ patients (25 RA-ILD⁻ and 20 SSc-ILD⁻); 21 idiopathic pulmonary fibrosis (IPF) patients; 21 healthy controls (HC). TAng were considered as CD3⁺CD184⁺CD31⁺ by flow cytometry. (3) Results: A similar TAng frequency was found between AD-ILD⁺ and IPF, being in both cases lower than that observed in AD-ILD⁻ and HC. A lower TAng frequency was associated with negative Scl-70 status and lower FEV1/FVC ratio in SSc-ILD⁺, as well as with men in RA-ILD⁺ and non-specific interstitial pneumonia radiological pattern in other AD-ILD⁺. No relationship between TAng and endothelial progenitor cells, endothelial cells and vascular endothelial growth factor gene expression and protein levels was disclosed. (4) Conclusions: Our findings suggest TAng as potential biomarkers for the early diagnosis of ILD in AD. Full article

The pathogenesis of pleuroparenchymal fibroelastosis (PPFE), a rare interstitial lung disease, remains unclear. Based on previous reports and our experience, we hypothesized that alveolar epithelial denudation (AED) was involved in the pathogenesis of PPFE. This multicenter retrospective study investigated the percentage of AED and the features of the denudated areas in 26 PPFE cases, 30 idiopathic pulmonary fibrosis (IPF) cases, and 29 controls. PPFE patients had lower forced vital capacities and higher residual volume/total lung capacities in pulmonary function tests compared to IPF and control patients. Histopathologically, subpleural fibroelastosis was observed in PPFE, and AED was observed in 12.01% of cases in the subpleural or interlobular septa regardless of fibroelastosis. The percentage of AED in the PPFE group was significantly higher than that in the IPF group (6.84%; p = 0.03) and the normal group (1.19%; p < 0.001). In the IPF group, the percentage of AED and the presence of PPFE-like lesions in the upper lobes were examined radiologically, but no correlation was found. We showed that AED frequently occurred in PPFE. AED was less frequent in IPF, which, in combination with imaging data, suggests that PPFE may have a different pathogenesis from IPF. Full article

Several databases of epidemiologic studies in patients with idiopathic pulmonary fibrosis (IPF) have been analyzed in the Western community. However, few studies have been reported in Asia. The objective of this study was to investigate the epidemiology of IPF in Taiwan. We collected and analyzed patients with IPF from the Taiwan National Health Insurance Research Database from 2001 to 2011. We estimated the annual incidence and cumulative prevalence of IPF and mean survival time of patients and determined the causes of death. The annual incidence rates of IPF remained stable after 2005, ranging from 0.7 to 1.3 cases per 100,000 people per year, whereas the cumulative prevalence rates increased steadily from 3.1 to 6.4 cases per 100,000 people per year during 2006–2011 based on a narrow case definition. Men older than 75 years had higher incidence compared with other age groups. The mean survival after diagnosis was 6.9 years. Old age, male sex, and respiratory hospitalization were associated with shorter survival time after diagnosis. Both the incidence and prevalence rates of IPF were lower in Taiwanese patients than Western ones. Moreover, the survival time was higher in the Asian population compared with the Western population. These results may suggest the heterogeneity of the IPF definition in different study populations and geographic locations. Full article

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fibrotic interstitial lung disease of unknown origin with a characteristic imaging and histologic pattern called usual interstitial pneumonia (UIP). The diagnosis of IPF is a complex procedure that requires the support of various specialists, who must integrate clinical, radiological, and histological data. The multidisciplinary team (MDT) has become the new gold standard to diagnose and manage the disease, increasing the accuracy and agreement of the diagnosis between different centers. It is mandatory to exclude nonspecific interstitial pneumonia or other diseases that can cause the UIP pattern, particularly drugs or exposure diseases, including chronic hypersensitivity pneumonitis or systemic autoimmune disease. The role of the MDT is also to decide who could need a biopsy or to review patient diagnoses at regular intervals in those with additional information or unexpected evolution. This review provides updated information to achieve a proper IPF diagnosis. Full article

The aim of this study was to present the diagnostic problems in pts treated for invasive aspergillosis (IA) in the Illrd Clinic of the Institute of Tuberculosis in the years 1993–2005. The material consisted of clinical documentation of 18 pts. 15 out of 18 pts (83.4%) died. In all those cases autopsy examination was done. In 13 pts IA was the main and in another 2 only the accessory cause of death. AU pts were treated with corticosteroids and/or cytostatic drugs because of lung cancer (13 pts), haematologic disorders (1 pts), Wegener's granulomatosis (3 pts) and idiopathic pulmonary fibrosis (1 pts). In 13 out of 18 pts granulocytopenia was revealed (on an average from 0.008 × 109/L to 0.95 × 109/L) one month before death. In 13 pts IA was limited to the lungs, in 5 others there were also fungal lesions in brain, kidneys, liver, spleen, pleura, pericardium and heart. Pts with disseminated fonn ofIA had significantly lower granulocyte count and were treated with higher doses of corticosteroids than others. Immunosupressive drugs and granulocytopenia can be regarded as predisposing factors. IA was diagnosed before death only in 5 out of 18 pts. This was mainly caused by the lack of the symptoms of in­fection (4 pts) and negative results ofmycological (6 pts) and serological examination (8 pts). We should underline that mycological examination was only done in 11 pts and serological examination of blood for fungal antigen only in 6 pts. We conclude that mycological infection ought to be searched in all pts treated with high doses of corticosteroids and those with prolonged granulocytopenia, especially if they have the symptoms of infection which does not respond to antibiotic therapy. Full article