Search Results (222)

Background and Objectives: Idiopathic pulmonary fibrosis (IPF) is a progressive, unpredictable, fatal interstitial lung disease. Antifibrotic therapy with pirfenidone or nintedanib slows functional decline, yet comparative real-world evidence remains limited. Materials and Methods: This retrospective, single-center, comparative cohort study included 76 IPF patients treated at the Clinic for Pulmonology at the University Clinical Center of Serbia (February 2019–February 2025). Diagnosis of IPF was made according to the guidelines of the American Thoracic Society and the European Respiratory Society. Demographic features, comorbidities, forced vital capacity (FVC), diffusion capacity for carbon monoxide (DLCO), high-resolution computerized tomography (HRCT) patterns, 6-min walk test distance (6MWTD), echocardiography, and survival outcomes were analyzed. Disease progression was defined as a ≥10% decline in FVC and/or DLCO after 12 months. Results: Of the 76 patients, 31 received nintedanib and 45 pirfenidone. Baseline characteristics, comorbidities, and HRCT patterns were comparable between groups. Mean annual decline in FVC was −1.74% with pirfenidone and −2.38% with nintedanib, without a statistical difference. DLCO declined by −4.25% and −6.29%, respectively, with similar downward trends over time in both groups. Progression was recorded in 35 (46.1%) patients, of whom 18 (58.06%) were in the nintedanib group and 17 (37.77%) in the pirfenidone group, with no difference between therapies (p = 0.81). Definite and probable usual interstitial pneumonia (UIP) were evenly represented on HRCT, although progression correlated significantly with the probable UIP pattern (p = 0.006). 6MWTD decreased in both groups over 12 months, again without treatment-related differences (p = 0.566). During up to 6 years of follow-up, overall survival was 4.18 years, with no significant difference between the nintedanib (4.55 years) and pirfenidone (3.81 years) groups (p = 0.159). No association was found between disease stage (FVC or DLCO) and progression. Conclusions: This study demonstrates that pirfenidone and nintedanib are equally effective in the management of IPF in real-world settings. The absence of significant differences in functional decline, progression rates, and survival indicates that treatment choices should be guided by individual clinical profiles rather than efficacy alone, reinforcing antifibrotic therapy as the primary approach to alter the course of IPF. Importantly, disease progression was strongly associated with a probable UIP pattern on HRCT, supporting current guidelines suggesting that probable UIP has a natural history and prognosis similar to those of definite UIP. Full article

Background/Objectives: Respiratory dysfunction in Parkinson’s disease (PD) is frequently underrecognized, particularly when resting oxygen saturation is preserved. Dynamic stress testing, however, may reveal exercise-induced oxygen desaturation, reflecting a latent functional respiratory impairment. The relationship between exertional oxygen desaturation and cognitive performance in PD remains insufficiently explored. Objective: To investigate the association between exercise-induced oxygen desaturation and global cognitive performance in patients with PD, and to explore the contribution of pulmonary gas exchange impairment assessed by diffusing capacity of the lung for carbon monoxide (DLCO). Methods: This prospective, cross-sectional, single-center observational study with consecutive enrollment included 50 patients with idiopathic Parkinson’s disease undergoing multidisciplinary respiratory evaluation following neurological assessment. Participants underwent cognitive evaluation using the Romanian version of the Montreal Cognitive Assessment (MoCA), pulmonary function testing including DLCO and total lung capacity (TLC), and a supervised 6-min walk test (6MWT) with continuous pulse oximetry. Exercise-induced oxygen desaturation was defined as a decrease in SpO₂ of ≥4% from baseline. Correlation analyses and multivariable regression models were applied. Results: Exercise-induced oxygen desaturation was frequent, with 60% of patients exhibiting a ≥4% decrease in SpO₂ during the 6MWT. Greater desaturation was significantly associated with lower MoCA scores (Spearman’s r = −0.383, p = 0.006). No significant associations were found between exertional desaturation and resting pulmonary function parameters, including DLCO and TLC. In multivariable analysis, lower MoCA score and levodopa–carbidopa intestinal gel treatment independently predicted greater oxygen desaturation during exercise. Conclusions: Exercise-induced oxygen desaturation is common in patients with PD despite preserved resting oxygenation and is associated with poorer cognitive performance. These findings suggest that exertional desaturation may reflect a dynamic functional impairment and may be associated with increased physiological vulnerability. Functional exercise testing with oxygen saturation monitoring may provide complementary information beyond resting pulmonary assessments. Full article

Background and Objectives: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease characterized by alveolar-capillary membrane remodeling and impaired gas diffusion. The diffusing capacity of the lung for nitric oxide (DLNO) has been proposed as a physiological parameter reflecting membrane diffusing capacity and pulmonary vascular involvement, potentially providing complementary information to diffusing capacity of the lung for carbon monoxide (DLCO). This study aimed to evaluate the role of DLNO in the functional assessment of patients with IPF and its correlation with clinical and echocardiographic outcomes. Materials and Methods: This observational, retrospective study included 35 consecutive IPF patients receiving antifibrotic therapy between February and December 2023. All participants underwent plethysmography, combined single-breath DLNO and DLCO testing, six-minute walk test (6MWT), mMRC dyspnea scale assessment, and echocardiography for the estimation of a higher probability of pulmonary hypertension (PH). Results: DLNO was significantly lower in males compared to females (49.3 ± 16.7% vs. 74.6 ± 16.1%, p < 0.001), with a reduced DLNO/DLCO ratio in men. DLNO correlated with oxygen therapy requirement (p = 0.010) and lower oxygen saturation during the 6MWT (p = 0.021). Patients with higher echocardiographic probability of PH showed markedly reduced DLNO values (17.6 ± 7.6%, p = 0.016) and higher FVC/DLNO ratios (2.31 ± 0.85 vs. 1.65 ± 0.64, p = 0.023), together with lower DLCO levels (p = 0.037). Conclusions: DLNO may complement DLCO in the evaluation of gas exchange and alveolar-capillary dysfunction in IPF. Although preliminary, these findings support the potential clinical utility of DLNO as an adjunct parameter in the functional characterization of IPF. Further multicenter studies are warranted to confirm these results. Full article

Background: Post-COVID-19 syndrome (PCS) is defined as the persistence or development of new symptoms 3 months after the initial infection with the SARS-CoV-2 virus, these clinical aspects being most often associated with functional respiratory changes, as well as imagistic modifications. This study aimed to evaluate longitudinal changes in pulmonary function among patients with PCS, in relation to the severity of the acute COVID-19 episode and the time elapsed since infection. Methods: A retrospective, observational study was conducted at the Clinical Hospital of Pulmonary Diseases Iași, Romania, between January 2021 and December 2022, including 97 adult patients with confirmed PCS. Demographic, clinical, and functional data were collected from medical records. Pulmonary function tests (PFTs) were performed according to ATS/ERS standards, assessing Forced Vital Capacity (FVC), Forced Expiratory Volume in the First Second (FEV₁), FEV₁/FVC ratio (Tiffeneau Index), Maximal Expiratory Flow at 50% and 25% of FVC (MEF50, MEF25), Diffusing Capacity of the Lung for Carbon Monoxide (adjusted for haemoglobin) (DLCO), Carbon Monoxide Transfer Coefficient (KCO), Alveolar Volume (AV), Total Lung Capacity (TLC) and Residual Volume (RV). Patients were grouped by time elapsed since infection (1–3, 4–7, 9–12, and up to 22 months). Statistical analyses included the Mann–Whitney U test, Spearman’s correlation, ROC curve analysis, and Principal Component Analysis (PCA). Results: A progressive improvement in FVC was observed up to 9–18 months post-infection (p < 0.05), while FEV₁ remained stable, suggesting a predominantly restrictive ventilatory pattern. Patients with moderate acute COVID-19 presented significantly lower FVC%, FEV₁%, DLCO%, and KCO% values compared with those with mild disease (p < 0.05). Diffusion abnormalities (DLCO and KCO) persisted beyond 12 months, indicating lasting alveolar-capillary impairment. ROC analysis identified TLC (AUC = 0.857), AV (AUC = 0.855), and KCO (AUC = 0.805) as the most discriminative parameters for residual dysfunction. PCA revealed three major functional domains—airflow limitation, diffusion capacity, and lung volume—explaining up to 70% of total variance. Conclusions: We are facing the emergence of a new phenomenon, namely a secondary post-COVID-19 pandemic of patients confronting with persistent post-COVID-19 symptoms who present with functional respiratory changes and who require careful monitoring in dynamics, personalized treatments and a multidisciplinary approach. Full article

Chronic obstructive pulmonary disease (COPD) and tuberculosis (TB) increasingly co-occur in low- and middle-income countries and aging populations. Prior pulmonary TB is a robust, smoking-independent determinant of COPD and is linked to persistent systemic inflammation, endothelial dysfunction, dyslipidemia, and hypercoagulability axes that also amplify cardiovascular disease (CVD) risk. We conducted a targeted narrative non-systematic review (2005–2025) of PubMed/MEDLINE, Embase, Scopus, and Web of Science, selecting studies for clinical relevance across epidemiology, clinical phenotypes, pathobiology, biomarkers, risk scores, sleep-disordered breathing, and management. No quantitative synthesis or formal risk-of-bias assessment was performed. Accordingly, findings should be interpreted as a qualitative synthesis rather than pooled estimates. Prior TB is associated with a distinctive COPD phenotype characterized by mixed obstructive–restrictive defects, reduced diffusing capacity (DLCO), radiographic sequelae, and higher exacerbation/hospitalization burden. Mechanistic insights: Convergent mechanisms chronic immune activation, endothelial injury, prothrombotic remodeling, molecular mimicry, and epigenetic reprogramming provide biologic plausibility for excess CVD, venous thromboembolism, and pulmonary hypertension. Multimarker panels spanning inflammation, endothelial injury, myocardial strain/fibrosis, and coagulation offer incremental prognostic value beyond clinical variables. While QRISK4 now includes COPD, it does not explicitly model prior TB or COPD-TB outcomes, but data specific to post-TB cohorts remain limited. Clinical implications: In resource-constrained settings, pragmatic screening, prioritized PAP access, guideline-concordant pharmacotherapy, and task-shifting are feasible adaptations. A history of TB is a clinically meaningful modifier of cardiopulmonary risk in COPD. An integrated, multimodal assessment history, targeted biomarkers, spirometry/lung volumes, DLCO, 6 min walk test, and focused imaging should guide individualized care while TB-aware prediction models and implementation studies are developed and validated in high-burden settings. Full article

Background: Serum C–C motif chemokine ligand 18 (seCCL18) in systemic sclerosis (SSc) has been primarily associated with progressive interstitial lung disease (SSc-ILD) and mortality. However, its relationship with non-pulmonary organ involvement, disease activity, and long-term outcome has not been comprehensively evaluated. We therefore examined the clinical relevance of seCCL18 in a single-center SSc cohort. Methods: A total of 151 patients with SSc (83 diffuse cutaneous (dcSSc), 68 limited cutaneous SSc (lcSSc); median (IQR) disease duration: 9 (4;16) years) and 47 age- and sex-matched healthy controls (HCs) were enrolled. Serum CCL18 concentrations were measured by enzyme-linked immunosorbent assay. Elevated seCCL18 was defined as >130 ng/mL (mean + 2 SD of the healthy control group). Organ involvement and disease activity (EUSTAR Activity Index, EUSTAR-AI) were assessed at baseline, while survival was analysed longitudinally. Results: Patients with SSc had significantly higher seCCL18 levels than HCs (mean ± SD: 99.9 ± 43.2 vs. 75.0 ± 27.5 ng/mL, p < 0.01). Elevated seCCL18 was associated with SSc-ILD (81.1% vs. 60.5%, p = 0.022), reduced forced vital capacity (FVC < 70%: 16.2% vs. 3.5%, p = 0.006), and reduced diffusing capacity for carbon monoxide (DLCO < 70%: 80.6% vs. 54.4%, p = 0.005). Higher seCCL18 levels were observed in patients with myocardial disease (104.8 ± 41.8 vs. 83.8 ± 44.2 ng/mL, p = 0.008), left ventricular diastolic dysfunction (107.1 ± 40.5 vs. 84.5 ± 45.0 ng/mL, p < 0.001), and oesophageal involvement (110.7 ± 38.3 vs. 93.3 ± 43.1 ng/mL, p = 0.009). SeCCL18 levels above the cut-off were more frequently associated with tendon friction rubs (51.4% vs. 27.4%, p = 0.007), active disease (EUSTAR-AI ≥ 2.5: 73% vs. 44%, p = 0.002), and elevated inflammatory markers (CRP > 5 mg/L: 51.4% vs. 19.3%, p < 0.001; ESR > 28 mm/h: 37.8% vs. 18.4%, p = 0.015). During a median follow-up of 87 months, 22 patients (15%) died. Elevated baseline seCCL18 predicted poorer survival in univariate analysis (log-rank p = 0.013) and remained an independent predictor of mortality in multivariable Cox regression (HR 1.789; 95% CI 1.133–2.824; p = 0.013), together with declining DLCO and reduced six-minute walk test performance. Conclusions: Elevated seCCL18 may identify patients with systemic sclerosis who exhibit a more severe multisystem phenotype, including cardiopulmonary, gastrointestinal, and musculoskeletal involvement, increased inflammatory activity, and reduced long-term survival. These findings suggest that seCCL18 may have some clinical utility as a prognostic biomarker reflecting widespread disease involvement beyond the lungs, even in patients with long-standing disease; however, the lack of an established cut-off value requires further validation in prospective, multicentre studies. Full article

Background/Objective: Pulmonary impairments have been identified as some of the most complex and debilitating post-acute sequelae of SARS-CoV-2 infection (PASC) or long COVID. This study identified and characterised the specific forms of pulmonary impairments detected using pulmonary function tests (PFT), chest X-rays (CXR), and computed tomography (CT) scans in patients with long COVID symptoms. Methods: We conducted a single-centre retrospective study to evaluate 60 patients with long COVID who underwent PFT, CXR, and CT scans. Pulmonary function in long COVID patients was assessed using defined thresholds for key test parameters, enabling categorisation into normal, restrictive, obstructive, and mixed lung-function patterns. We applied exact binomial (Clopper–Pearson) 95% confidence intervals to calculate the proportions of patients falling below the defined thresholds. We also assessed the relationships among spirometric indices, lung volumes, and diffusion capacity (DLCO) using scatter plots and corresponding linear regressions. The findings from the CXRs and CT scans were categorised, and their prevalence was calculated. Results: A total of 60 patients with long COVID symptoms (mean age 60 ± 13 years; 57% female) were evaluated. The cohort was ethnically diverse and predominantly non-smokers, with a mean BMI of 32.4 ± 6.3 kg/m². PFT revealed that most patients had preserved spirometry, with mean Forced Expiratory Volume in 1 Second (FEV1) and Forced Vital Capacity (FVC) above 90% predicted. However, a significant proportion exhibited reductions in lung volumes, with total lung capacity (TLC) decreasing in 35%, and diffusion capacity (DLCO/TLCO) decreasing in 75%. Lung function pattern analysis showed 88% of patients had normal function, while 12% displayed a restrictive pattern; no obstructive or mixed patterns were observed. Radiographic assessment revealed that 58% of chest X-rays were normal, whereas CT scans showed ground-glass opacities (GGO) in 65% of patients and fibrotic changes in 55%, along with findings such as atelectasis, air trapping, and bronchial wall thickening. Conclusions: Spirometry alone is insufficient to detect impairment of gas exchange or underlying histopathological changes in patients with long COVID. Our findings show that, despite normal spirometry results, many patients exhibit significant diffusion impairment, fibrotic alterations, and ground-glass opacities, indicating persistent lung and microvascular damage. These results underscore the importance of comprehensive assessment using multiple diagnostic tools to identify and manage chronic pulmonary dysfunction in long COVID. Full article

Objectives: To develop and validate a transthoracic lung ultrasound (TLUS)-integrated clinical nomogram for predicting pulmonary arterial hypertension (PAH) in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD). Methods: This multicenter retrospective study included 550 patients with CTD-ILD from the Second Affiliated Hospital of Fujian Medical University and 169 external cases from the Xijing Hospital, Fourth Military Medical University. Patients were randomly divided into a training cohort (n = 385) and an internal validation cohort (n = 165); the external dataset served as a testing cohort. Demographic, physiological, laboratory, pulmonary function, and TLUS data were collected. Univariate and multivariate logistic regression analyses identified independent predictors of PAH, which were used to construct a nomogram model. Discrimination was assessed using receiver operating characteristic (ROC) curves and area under the curve (AUC) values. Calibration, decision curve analysis (DCA), and clinical impact curves (CIC) were performed to evaluate model accuracy and clinical utility. Results: Five independent predictors were identified: respiratory rate, diffusing capacity of the lung for carbon monoxide (DLCO% predicted), TLUS score, red blood cell (RBC) count, and brain natriuretic peptide (BNP). The model achieved excellent discrimination with AUCs of 0.952 (95% confidence interval [CI]: 0.927–0.977) in the training cohort, 0.935 (95% CI: 0.885–0.985) in the validation cohort, and 0.874 (95% CI: 0.806–0.942) in the testing cohort, outperforming individual predictors. Calibration plots showed close agreement between predicted and observed probabilities, while DCA and CIC confirmed strong clinical benefit and applicability across all thresholds. Conclusions: This TLUS-integrated nomogram provides a noninvasive and reliable tool for individualized PAH risk assessment in CTD-ILD patients. By combining ultrasound findings with physiological and laboratory markers, the model enables accurate detection of high-risk cases and may assist clinicians in optimizing surveillance and management strategies. Full article

Background/Objectives: Systemic sclerosis (SSc) is a rare and severe autoimmune disease with limited treatment options. Autologous hematopoietic stem cell transplantation (HSCT) and mesenchymal stem cell transplantation (MSCT) have emerged as promising therapeutic strategies, especially for patients with refractory or rapidly progressive forms of the disease. However, no comparative synthesis has yet evaluated the clinical outcomes, safety, and applicability of these two distinct stem-cell-based interventions. This systematic review aimed to perform a comparative qualitative synthesis of clinical outcomes, safety profiles, and evidence quality for HSCT and MSCT in patients with systemic sclerosis, focusing on survival, skin fibrosis, pulmonary function, and adverse events. Methods: A comprehensive search was conducted in PubMed, ScienceDirect, Cochrane Library, and Google Scholar for the period between 2015 and May 2025. Studies were included if they reported on adult patients with a confirmed diagnosis of SSc treated with either autologous HSCT or MSCT and provided clinical outcome data. Risk of bias was assessed using the Newcastle-Ottawa Scale. Due to heterogeneity across studies, results were synthesized qualitatively. Results: Eleven studies met the inclusion criteria, comprising 504 patients (316 HSCT, 188 MSCT). HSCT showed consistent improvement in survival (1-, 5-, and 10-year), reduction in modified Rodnan skin scores (mRSS), and s ilization or improvement in pulmonary function (DLCO, FVC), albeit with a higher incidence of serious adverse events, including transplant-related mortality (up to 10%) and infectious complications. MSCT demonstrated favorable effects on skin fibrosis and lung involvement with a significantly lower toxicity profile. However, long-term survival data and methodological robustness were limited were more limited. HSCT was supported by multiple randomized controlled trials and international guidelines, while MSCT remains under clinical investigation with promising but still preliminary evidence. Conclusions: Both HSCT and MSCT demonstrate potential clinical benefits in systemic sclerosis, but they differ substantially in evidence strength and risk profiles. HSCT provides the most robust evidence for long-term disease modification in carefully selected patients, whereas MSCT represents a promising and safer investigational option, particularly for patients ineligible for intensive therapy. Further well-designed comparative studies are required to define their optimal clinical roles. Full article

Background: The prevalence of pulmonary hypertension (PH) in patients who are positive for myositis-specific antibody (MSA) and myositis-associated antibody (MAA) remains unclear. Methods: We conducted a retrospective study of patients with an age of 18 years or older diagnosed with myositis interstitial lung disease (ILD) at our university’s ILD clinic between 2019 and 2022. Echocardiographic PH was defined by tricuspid regurgitation velocity (TRV) ≥ 2.9 m/s on transthoracic echocardiography (TTE) consistent with intermediate probability of PH using 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. We grouped patients based on low probability of PH vs. intermediate to high probability of PH. We examined 6 min walk test (6MWT) data, pulmonary function tests (PFTs), all-cause mortality, and rate of lung transplantation. We also evaluated patients who were on immunosuppression vs. those not on immunosuppression. Results: The intermediate to high probability of PH group had a higher prevalence of dermato-specific antibodies (14.2% vs. 34.5%, p = 0.048). Specifically, MDA-5 was found to be more prevalent in patients with intermediate to high probability of PH (7.1% vs. 24.1%, p = 0.040). There was no difference in 6MWT parameters between groups (363.2 ± 115.6 m vs. 294.9 ± 147.5 m, p = 0.108). FVC and DLCO were lower in patients with intermediate to high probability of PH (71.3 ± 22.4 L vs. 58.8 ± 16.7 L, p = 0.037; 56.3 ± 21.8 mL/min/mmHg vs. 36.9 ± 15.5 mL/min/mmHg, p = 0.003). The all-cause mortality and rate of lung transplantation was higher in the intermediate to high probability of PH group (5.4% vs. 20.7%, p = 0.041, 0% vs. 6.9%, p = 0.049). There was no difference in all-cause mortality between patients who were on immunosuppression vs. those who were not on immunosuppression in patients with intermediate to high probability of PH (33.3% vs. 7.1%; p = 0.169). Conclusions: Patients with MSA/MAA may have an increased risk of PH with reduced lung function, higher mortality, and greater rate of lung transplantation. Our study further elucidates the growing body of evidence that dermato-specific antibodies, such as MDA-5 are associated with an increased risk of PH. Further research is needed to investigate the role of PH and immunosuppression in these patients. Full article

Background and Objectives: Fibrosing interstitial lung diseases (ILDs) may predispose to neurocognitive impairment through chronic hypoxemia and systemic inflammation, yet data integrating pulmonary physiology, disease severity, and cognition are limited. We aimed to compare global cognitive performance between adults with fibrosing ILD and contemporaneous non-ILD clinic comparators, explore differences across ILD subtypes, and identify physiologic and clinical predictors of low MMSE scores. Materials and Methods: In this single-center cross-sectional study, 45 adults with fibrosing ILD and 32 non-ILD participants from university-affiliated pulmonology clinics completed the Mini-Mental State Examination (MMSE) and standardized lung function testing (including diffusing capacity, DLCO%). Comorbidity (Charlson index), inflammatory markers (C-reactive protein), and GAP (Gender–Age–Physiology) severity were recorded. Associations with MMSE and MMSE < 24 were examined using correlations and multivariable logistic regression. Results: Mean MMSE was lower in ILD than in non-ILD participants (23.9 ± 3.6 vs. 26.8 ± 2.8; p < 0.001), and MMSE < 24 occurred in 33.3% versus 12.5%, respectively. Within ILD, the usual interstitial pneumonia (UIP) pattern showed the lowest MMSE scores. DLCO% and total lung capacity correlated positively with MMSE (r = 0.44 and r = 0.34, respectively). In multivariable models, ILD diagnosis remained associated with MMSE < 24 (odds ratio [OR] 2.72, 95% CI 1.14–6.48), and each 10-percentage-point decrement in DLCO% increased the odds of MMSE < 24 (OR 1.42, 95% CI 1.11–1.92). GAP ≥ 4 was also associated with impaired cognition (OR 2.91, 95% CI 1.13–7.57). Conclusions: Fibrosing ILD, particularly with reduced diffusing capacity and higher GAP stage, is associated with lower MMSE scores and a higher frequency of values below a conventional impairment threshold. Prospective studies incorporating comprehensive neuropsychological testing are needed to determine whether and how neurocognitive assessment should be integrated into routine ILD care. Full article

Background/Objectives: Despite the complementary insights provided by body plethysmography and impulse oscillometry, direct comparisons across obstructive and restrictive lung diseases remain limited. The aim of this study was to evaluate correlations between plethysmographic and oscillometric parameters, with a particular focus on hyperinflation and small airway dysfunction. Methods: We retrospectively analyzed 69 adult patients (35 obstructive, 34 restrictive) hospitalized in the Pulmonology Department of Mureș Clinical Hospital. All patients underwent body plethysmography (sRaw, Rtot, FRC, RV, TLC, RV/TLC) and impulse oscillometry (R5, R20, R5-20, X5, Fres, AX). Non-parametric tests were used to compare groups, and associations were assessed using Spearman’s correlation. Results: Higher airway resistance (sRaw 124 vs. 62, p < 0.001; R5 0.55 vs. 0.38, p < 0.01) and greater hyperinflation (RV 124 vs. 99, p < 0.001) were observed in patients with obstructed airways. Impulse oscillometry reactance markers (X5, Fres, AX) significantly differentiated obstructive from restrictive pathophysiology (p < 0.02). In the obstructive group, sRaw correlated with R5 (p = 0.01) and R5-20 (r = 0.58, p < 0.001), while AX correlated with RV (r = 0.59, p < 0.001). Restrictive patients revealed negative correlations between AX and static volumes (RV, TLC, RV/TLC; all p < 0.05). DLCO was higher in obstructive patients (75 vs. 62, p = 0.01). Conclusions: Our study demonstrates that body plethysmography and impulse oscillometry provide complementary information on respiratory mechanics and that the results obtained with the two methods correlate significantly, especially in obstructive diseases. Full article

Background: Interstitial lung diseases (ILDs) often progress quickly and are associated with a poor prognosis. New noninvasive biomarkers to assist in the classification and prognostication of ILD are needed. Exhaled nitric oxide (FeNO), Cavity nitric oxide (CaNO), and carbon monoxide (eCO) are biomarkers of airway inflammation, widely used in respiratory inflammatory diseases such as asthma and chronic obstructive pulmonary disease (COPD). However, their value in ILD remains unclear. Objective: To evaluate the potential diagnostic and prognostic value of FeNO, CaNO, and eCO in ILD, and explore their integration into clinical practice. Methods: A total of 237 patients were recruited for the study, including 14 with idiopathic pulmonary fibrosis (IPF), 46 with interstitial pneumonia with autoimmune features (IPAF), 19 with mixed connective tissue disease–associated ILD (MCTD-ILD), 65 with polymyositis/dermatomyositis-associated ILD (PM/DM-ILD), 17 with rheumatoid arthritis-associated ILD (RA-ILD), 7 with systemic lupus erythematosus-associated ILD (SLE-ILD), 19 with Sjögren’s syndrome-associated ILD (SS-ILD), and 50 with systemic sclerosis-associated ILD (SSc-ILD). Multiple-flow FeNO and eCO analyses were performed in this population. The associations of these biomarkers with pulmonary function, acute exacerbations, and radiologic fibrosis classification were evaluated. Results: Patients with IPF exhibited significantly higher levels of FeNO at 50 mL/s (FeNO50) compared to those with connective tissue disease-associated ILD (CTD-ILD) and IPAF. Both CaNO and eCO were negatively correlated with pulmonary function parameters, particularly forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCO). Receiver operating characteristic (ROC) curve analysis indicated that CaNO is a reliable biomarker for acute exacerbation, with an area under the ROC curve (AUC) of 0.8887, and a cutoff value of 6.35. Additionally, CaNO > 6.35 was associated with a relative risk (RR) of 12.87 for acute exacerbation (AE) compared to CaNO ≤ 6.35. Moreover, both CaNO and eCO levels were significantly higher in the fibrotic ILD group compared to the non-fibrotic group, with ROC analysis indicating AUCs of 0.7173 for CaNO and 0.6875 for eCO. Conclusions: FeNO, CaNO, and eCO can provide strong support for the early diagnosis and monitoring of ILD, especially with CaNO playing a crucial role in predicting acute exacerbations. Integrating these biomarkers into clinical practice can help doctors more accurately assess the progression of ILD and develop personalized treatment plans, ultimately improving the prognosis of ILD patients. Future research is needed to validate the effectiveness of these biomarkers in clinical management, facilitating their integration as standard tools for clinical monitoring. 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/Objectives: In clinical trials designed to evaluate efficacy and safety of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF), inclusion criteria were age ≤ 80 years, FVC ≥ 50%pred and DLco ≥ 35%pred. The outcomes in patients progressing beyond these criteria are not yet fully investigated. This study aims to evaluate the effectiveness of pirfenidone in patients who, during treatment, progress beyond the criteria adopted in clinical trials. Methods: This observational retrospective single-centre study included patients younger than 81 years and with mild-to-moderate IPF who had initiated pirfenidone from December 2011 to October 2023. We compared the monthly decline in absolute FVC and %DLco before and after the progression beyond one or more inclusion criteria used in clinical trials. Results: A total of 174 patients were included in the study, with a mean follow-up of 39.2 months (SD ± 29.7 months, range 2–152 months). Seventy-six of them remained within all criteria (control group), 72 passed one criterion, 25 two criteria and 1 all criteria. There was no difference in the trend of FVC and %DLco between the control group and the groups that passed one or two criteria. The intra-individual trend after passing one criterion was similar to that shown before, for both FVC and %DLco. The intra-individual trend also appeared to be preserved when analyzing a small group of patients who had passed any two criteria. Conclusions: Neither attaining advanced age nor the development of severe functional impairment appeared to limit the effectiveness of pirfenidone. Full article