Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is one of the leading causes of morbidity and mortality in SSc, affecting up to three-quarters of patients. The disease course is highly heterogeneous, ranging from indolent, nonprogressive forms to rapidly progressive pulmonary fibrosis (PPF). Identifying patients at risk for progression remains a key clinical challenge. Routine screening with high-resolution computed tomography at diagnosis and regular pulmonary function test monitoring are essential for early detection and timely management. Recent efforts have sought to harmonize the definition of PPF across fibrosing ILDs, recognizing shared clinical and pathophysiological mechanisms beyond idiopathic pulmonary fibrosis. Approximately 20% to 30% of patients with SSc-ILD develop PPF, yet reliable predictors of progression remain elusive. Advances in imaging, functional assessment, and molecular biomarkers hold promise for improving risk stratification. Therapeutic approaches have evolved from conventional immunosuppression toward combination and antifibrotic strategies. Mycophenolate mofetil remains the preferred first-line therapy, whereas nintedanib is the only antifibrotic agent approved for both SSc-ILD and PPF, having demonstrated efficacy in slowing lung function decline. Combination therapy, particularly mycophenolate mofetil with nintedanib or biologics such as rituximab and tocilizumab, is gaining traction in clinical practice despite limited comparative data. Emerging antifibrotic and anti-inflammatory agents offer new therapeutic prospects. This review summarizes current understanding of PPF in SSc-ILD, focusing on its evolving definition, prognostic determinants, and treatment landscape, and highlights unmet needs for personalized, evidence-based management.

Severe asthma represents a major clinical challenge, accounting for disproportionate healthcare costs and morbidity despite affecting only 3–5% of patients with asthma. Traditional computed tomography (CT) can quantify bronchial wall thickening and mucus plugging, but concerns about cumulative radiation exposure limit its use for longitudinal monitoring, particularly in patients requiring frequent assessment. Recent advances in magnetic resonance imaging (MRI), specifically T2-weighted sequences combined with ultrashort echo time (UTE) imaging, have enabled quantitative assessment of bronchial wall signal intensity without ionizing radiation. Pilot studies demonstrate that bronchial wall T2-weighted MRI signal intensity (BrWall_T2-MIS) is significantly elevated in severe versus non-severe asthma, correlates with airflow obstruction and type-2 inflammatory markers, and has shown preliminary ability to predict exacerbation risk. Importantly, this biomarker may capture different pathophysiological information than CT-derived wall thickness measurements, potentially reflecting active inflammation rather than fixed remodelling. Although evidence remains preliminary and limited to single-centre studies with small sample sizes, BrWall_T2-MIS represents a promising investigational tool that could enhance phenotyping and therapeutic monitoring in the era of targeted biological therapies for severe asthma.

CDK4/6 inhibitors, including ribociclib, may cause organising pneumonia that closely mimics lung cancer on CT and FDG-PET. Histological confirmation is essential in discordant cases. https://bit.ly/48nGZ3t.

Cardiovascular diseases, such as hypertension, coronary artery diseases (CAD), arrhythmias, and chronic heart failure (CHF), often require treatment with beta-blockers. These conditions frequently coexist with chronic obstructive pulmonary disease (COPD), which can complicate therapeutic decisions. Indeed, patients with moderate-to-severe COPD are frequently not given these agents out of concern for possible bronchoconstriction arising from blockade of β2-adrenoceptors in the airways. Observational studies, and meta-analyses support the cardiovascular benefits of β-blockade in people with COPD and cardiovascular diseases. Recent trials evaluating cardioselective β-blockade suggest that cardioselective beta-blockers such as bisoprolol, metoprolol and nebivolol are safe and likely beneficial in those patients that would benefit from their intake due to presence of cardiac comorbid diseases. The aim of this systematic review is to summarise the recent trials and indications for use of cardioselective β-blockers in patients with COPD.

Although guidelines recommend a multidisciplinary team (MDT) approach for the diagnosis and management of systemic autoimmune rheumatic disease-associated interstitial lung disease (SARD-ILD), they lack guidance on MDT composition and function. This Delphi consensus project aimed to define a shared MDT model for managing patients with SARD-ILD. A questionnaire was circulated to an expert panel of 77 Italian pulmonologists, rheumatologists, immunologists, and internal medicine specialists, with statements rated over two voting rounds using a 5-point Likert scale. Response rates were 73

Interstitial lung abnormalities (ILAs) are increasingly recognized as incidental findings on chest computed tomography and may represent an early stage in the spectrum of interstitial lung disease (ILD). However, their clinical significance and optimal management remain debated. While some ILAs may progress to pulmonary fibrosis and functional impairment, many remain stable, and the criteria for initiating diagnostic evaluation, determining monitoring intensity, and starting therapeutic interventions are not clearly established. Current guidelines emphasize risk stratification and longitudinal surveillance, yet significant uncertainties persist regarding which individuals may benefit from early treatment rather than conservative follow-up. This narrative review synthesizes the existing evidence on ILA epidemiology, natural history, and proposed management approaches. We analyse data supporting both early intervention—particularly when ILAs evolve into clinically meaningful ILD—and watchful waiting strategies to avoid overtreatment in asymptomatic and stable cases. The review highlights persistent knowledge gaps, including the lack of consensus on defining progression, determining optimal follow-up intervals, and identifying clear indications for pharmacologic therapy. Overall, ILAs represent a heterogeneous and still poorly defined entity. By critically examining current evidence and ongoing debates, this review aims to guide clinicians in navigating the “to treat or not to treat” dilemma and to outline key priorities for future research.

Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease in which systemic corticosteroids remain first-line therapy despite limited evidence, especially in fibrotic forms. This study aimed to identify clinical, radiological, and biological features associated with functional response to steroids. We retrospectively analyzed 43 consecutive patients with HP treated with systemic corticosteroids and followed for at least 6 months. Patients were classified according to changes in forced vital capacity (FVC) as responders (≥5% increase), non-responders (≥5% decrease), or indifferent (±5%). Eighteen patients (42%) were responders, 15 (35%) indifferent, and 10 (23%) non-responders. Non-responders showed a consistently worse functional trajectory. A fibrosing high-resolution computed tomography pattern and baseline consolidations were more frequent in this group, as were precipitating antibodies against P. notatum and A. fumigatus. Conversely, bronchoalveolar lavage lymphocytosis >20% was more common among responders. Baseline FVC% and relative diffusing capacity of the lung for carbon monoxide were higher in non-responders, whereas demographic characteristics, smoking history, antigen exposure, comorbidities and autoantibody positivity did not differ significantly across groups. Fewer than half of patients experienced functional improvement after steroid therapy. Radiological fibrosis, consolidations, and specific precipitating antibodies were associated with lack of response, whereas bronchoalveolar lavage lymphocytosis predicted improvement. These findings highlight the heterogeneity of HP and may help identify patients unlikely to benefit from corticosteroids, supporting earlier consideration of alternative therapeutic strategies.

Acute exacerbations (AEs) occur both in patients with idiopathic pulmonary fibrosis (IPF) and non-IPF fibrotic interstitial lung disease (fILD). These events confer high morbidity and mortality, with a lack of proven effective therapeutic interventions. The objective of this state-of-the-art document is to summarize latest evidence since the 2016 international working group report on AE-IPF, expanding it across the spectrum of all fILDs. A comprehensive literature review on the epidemiology, associated and risk factors, prognosis, and management of AE-fILD is summarized. In addition to revising the AE definition and diagnostic criteria for broad application across different fILDs, a conceptual framework for acute respiratory worsening (ARW) has been proposed to encompass a variety of acute respiratory deteriorations, both related and unrelated to AE. This allows structured evaluation in both clinical and research settings. The proposed revised definition for AE-fILD is an acute respiratory event characterized by increased respiratory symptoms or signs and associated with radiologic or histologic features consistent with diffuse alveolar damage (with or without superimposed organizing pneumonia) in a patient with known or newly diagnosed fILD. On the other hand, ARW refers to a heterogeneous group of clinical events with acute symptom worsening not attributable to DAD in patients with fILD, such as pulmonary edema, bronchitis, and pneumonia, although severe pneumonia can trigger AE-fILD. Additionally, we discuss considerations for inclusion of AE as a clinical trial endpoint, as well as research priorities for advancing knowledge on the pathogenic mechanisms, event prediction, risk stratification, and development of drugs and supportive treatments.

Pulmonary fibrosis comprises a heterogeneous group of interstitial lung diseases (ILDs) with diverse aetiologies but often convergent clinical behaviour. Idiopathic pulmonary fibrosis (IPF) represents the prototypical fibrotic ILD; however, a substantial proportion of patients with non-IPF fibrotic ILDs develop a progressive pulmonary fibrosis (PPF) phenotype characterised by irreversible functional decline, worsening symptoms, increased healthcare utilisation, and excess mortality. Although traditionally classified according to underlying cause, accumulating epidemiological, clinical, and biological evidence indicates that once progression emerges, fibrotic ILDs share common trajectories that transcend etiologic boundaries. Across IPF and non-IPF PPF, longitudinal decline in forced vital capacity represents the dominant marker of disease activity and prognosis, with similar rates of deterioration and comparable mortality risk. Shared genetic susceptibility factors—particularly variants affecting telomere maintenance and epithelial integrity—together with convergent fibrotic pathways suggest a common biological vulnerability that may influence disease behaviour beyond the original diagnosis. Clinically, patients with PPF exhibit symptom burden, quality-of-life impairment, risk of acute exacerbations, and need for advanced supportive care that largely overlap with those observed in IPF. Randomised clinical trials further reinforce this convergence, demonstrating that antifibrotic therapies attenuate lung function decline across IPF and PPF populations. Overall, current evidence supports the view that PPF might represent a clinical syndrome characterised by shared disease trajectories, common clinical needs, and comparable responses to antifibrotic therapy. • Progressive pulmonary fibrosis may represent a clinical syndrome that transcends the underlying aetiology of interstitial lung disease, reflecting shared biological pathways that drive disease progression. • Shared genetic susceptibility factors, although present only in a subset of patients, together with convergent biological pathways—including epithelial injury, aberrant repair responses, fibroblast activation and extracellular matrix deposition—support the concept that diverse fibrotic ILDs may ultimately converge toward common mechanisms driving progressive fibrosis • Functional decline, prognosis, and healthcare needs in progressive pulmonary fibrosis closely resemble those observed in idiopathic pulmonary fibrosis, leading to overlapping clinical management and supportive care requirements. • Randomized clinical trials increasingly support a phenotype-driven therapeutic strategy, with antifibrotic agents demonstrating efficacy across different progressive fibrotic lung diseases. • A syndromic approach to patients with progressive pulmonary fibrosis emphasizes early identification of a progressive phenotype, enabling timely treatment decisions, holistic management, and a shift from aetiology-centred to behaviour-centred care.

BACKGROUND:Idiopathic pulmonary fibrosis (IPF) predominantly affects older adults. Frailty is increasingly recognized as an important feature of IPF, although its prognostic value remains uncertain. This study aimed to assess the prevalence of frailty and concordance among validated instruments, characterize the principal domains of vulnerability identified through comprehensive geriatric assessment (CGA), and evaluate the prognostic value of integrating frailty with indices of respiratory disease severity in older adults with IPF. METHODS:We conducted a prospective, single-center study of patients aged 65 years or older with confirmed IPF. Frailty was assessed using the Fried frailty phenotype (FFP), a CGA-derived Frailty Index (CGA-FI), and the Clinical Frailty Scale (CFS). The primary outcome was a composite of all-cause mortality or acute exacerbation during follow-up. Cox proportional hazards models and bootstrap-derived concordance indices were used to evaluate prognostic associations and model discrimination. RESULTS:Among the 140 patients included, the prevalence of frailty ranged from 16.4% according to the CGA-FI to 22.3% according to the FFP, whereas 19.3% were classified as frail according to the CFS. During a median follow-up of 465 days (IQR, 221-1076 days), 35 patients (25.0%) experienced the composite outcome. Frailty assessed using the CFS (CFS ≥5: HR, 3.94; 95%CI, 1.81-8.58) and a higher Gender-Age-Physiology (GAP) stage (GAP II-III: HR, 3.11; 95%CI, 1.39-7.00) were independently associated with the composite outcome. The integrated GAP-CFS model showed good discrimination, with a bootstrap-derived Harrell C index of 0.72 (95%CI, 0.62-0.81). Patients who experienced events also had higher Strength, Assistance in Walking, Rising From a Chair, Climbing Stairs, and Falls (SARC-F) scores and greater functional impairment. CONCLUSIONS:Frailty is common and clinically relevant in IPF. Integrating frailty measures with pulmonary indices improves prognostic stratification and supports the use of the CFS and CGA to guide personalized, multidimensional care.

BACKGROUND:Coexistence of interstitial lung disease (ILD), particularly idiopathic pulmonary fibrosis (IPF), and lung cancer poses major diagnostic and therapeutic challenges, yet clinical management remains heterogeneous. The project aims to describe current Italian practices for integrated management of ILD with concomitant lung cancer.  Methods: ICARO (Interstiziopatia e Cancro del polmone: AppRoccio al management clinico integratO) is a national cross-sectional clinician survey conducted in Italy on behalf of the Italian Respiratory Society from  November 2024 to March 2025. A 12-item multiple-choice questionnaire assessed diagnostic strategies, treatment preferences, and perceived toxicity risks. Invitations were sent to X physicians, among which 38 ansewered (35 specialists and senior 3 registrars (age range: 28-68 years).  Results: An ILD multidisciplinary team was available in 26/38 (71.1%) centres. Diagnostic procedures for lung cancer in ILD patients were reported as performed "always/often" by 14/38 (36.8%), with the main concern being ILD progression after procedures (31/38 - 81.6%). Most respondents continued antifibrotic therapy during systemic cancer treatment (28/38- 73.7%). Combined chemotherapy plus immune checkpoint inhibitors was perceived as the highest-risk regimen by 19/38 physicians (50%), and 20/38 (52.6%) were hesitant to offer neoadjuvant immunotherapy in stage II-IIIa NSCLC. Severe toxicity from radiotherapy was reported as frequent by 8/38 (21.1%).  Conclusions: Italian clinicians report substantial variability in diagnostic and therapeutic strategies for lung cancer in ILDs, driven mainly by concern for ILD progression and treatment-related pulmonary toxicity. Although limited, this study unveils an urgent need for further prospective studies to better define the safety and efficacy of combined therapeutic approaches and to establish evidence-based guidelines to support clinical decision-making.

Interstitial lung disease (ILD) is the most severe extra-articular manifestation of rheumatoid arthritis (RA), representing one the most frequent causes of death for patients with RA. The treatment of RA-ILD is still debated and challenging for both rheumatologist and pulmonologist. Ideally, it should aim to control the underlying joint disease activity, to prevent ILD, or to reduce the progression of lung damage, in particular fibrotic changes. Disease-modifying antirheumatic drugs (DMARDS) are used in daily practice for the treatment of joint involvement but are not demonstrated to be effective in ILD, although good control of the systemic disease might improve patients’ prognosis. However, immunosuppressants, usually suggested for the treatment of ILD related to autoimmune rheumatic diseases, often have low efficacy in regard to inflammatory joint manifestations of RA. Finally, the awareness of potential pulmonary toxicity related to disease-modifying antirheumatic drugs further complicates this scenario. Therefore, a multidisciplinary discussion, including at least a rheumatologist, pulmonologist, pathologist, and thoracic radiologist is generally requested to decide the best therapeutic strategy for an individual patient. In this paper, we will review the current available options for the treatment of RA-ILD, focusing on their possible use according to the current knowledge on pathogenesis and clinical evolution of RA-ILD.

Background:Ulcerative colitis (UC) is a chronic inflammatory bowel disease characterized by extraintestinal manifestations in nearly half of patients. Although pulmonary involvement has traditionally been considered rare, with a reported prevalence of less than 1%, recent studies indicate that subclinical airway abnormalities may occur in 40-60% of cases. Tracheobronchial stenosis is an uncommon but clinically significant extraintestinal manifestation that causes considerable diagnostic and therapeutic challenges. Case series:This report presents three female patients with UC who developed significant bronchial stenosis, predominantly affecting the left main bronchus. Notably, a temporal dissociation between intestinal and respiratory disease activity was observed; in two cases, airway symptoms developed years after the initial UC diagnosis, and in one case, symptoms appeared following total colectomy during intestinal disease remission. Histopathological examination consistently revealed chronic lymphoplasmacytic infiltrates and mucosal edema. Management:Therapeutic responses varied considerably, ranging from active inflammation to irreversible fibrotic remodeling. One patient, unresponsive to systemic corticosteroids, required rigid bronchoscopy with balloon dilatation to restore airway patency. Another patient experienced rapid improvement with high-dose corticosteroids administered during a UC flare, while the third demonstrated partial improvement with persistent cicatricial stenosis. Conclusion:Early identification of tracheobronchial involvement in UC is important to prevent irreversible pulmonary damage. Given the rarity of this manifestation and the absence of standardized treatment protocols, multidisciplinary management incorporating medical therapy and interventional bronchoscopy is necessary to individualize patient care.

NTM pulmonary disease (NTM-PD) is frequently associated with low body mass index and weight loss, yet comprehensive nutritional evaluation at diagnosis remains limited. We conducted a multicenter observational study across eight Italian referral centers to assess nutritional status and physical performance in newly diagnosed NTM-PD patients. Assessments included anthropometry, nutritional and physical activity questionnaires, bioelectrical impedance analysis, handgrip strength, gait speed, and pulmonary function tests in order to identify nutritional phenotypes and sarcopenia according to EWGSOP2 definition. 69 patients (77

Introduction: Sarcoidosis is a systemic granulomatous disease characterized by heterogeneous clinical presentation and variable organ involvement. Beyond pulmonary manifestations, extrapulmonary disease and associated comorbidities substantially contribute to disease burden and clinical complexity. Although sex-related differences in sarcoidosis phenotype have been reported, data on sex-specific patterns of systemic comorbidities remain limited. Methods: We retrospectively analyzed 1,313 patients with sarcoidosis followed at five Italian referral centers. Systemic comorbidities, disease phenotype, and treatment exposure were systematically recorded and analyzed according to biological sex. Organ involvement was assessed using the WASOG Sarcoidosis Organ Assessment Instrument. Results: Female patients showed a significantly higher prevalence of autoimmune, bone, and psychiatric comorbidities, as well as a greater burden of extrapulmonary sarcoidosis, particularly cutaneous and ocular involvement. Extrapulmonary disease was associated with increased systemic comorbidity burden. Malignancy occurred within the broader context of systemic comorbidities, was more frequent among female patients, and was not associated with treatment exposure. Conclusion: Sarcoidosis is characterized by sex-specific patterns of systemic comorbidities and disease expression that extend beyond pulmonary involvement. These findings support the relevance of sex-aware clinical assessment and highlight the need for prospective studies to better define comorbidity trajectories and inform personalized management strategies.

A strong rationale supports the combination of an immunocheckpoint inhibitor (ICI) and an antiangiogenic agent in different tumor types such as endometrial cancer (EC) and renal cell carcinoma (RCC). Accordingly, the combination of pembrolizumab plus lenvatinib demonstrated the efficacy in pretreated advanced EC and metastatic RCC, and to optimize drugs administration a more in-depth knowledge of adverse events management for the combination is needed. We analyzed the most common toxicities for appropriate and proactive management to maximize the efficacy of the tyrosine kinase inhibitor (TKI)-ICI combination and a summary of the most relevant recommendations on the management of organ specific disorders was produced. A multidisciplinary approach is suggested, involving collaboration between oncologists and organ specialists to promptly prevent/address possible toxicities associated with the treatment.