INTRODUCTION:Sex-related differences in interstitial lung disease (ILD) phenotypes are well recognized, but it remains unclear whether sex itself independently influences outcomes in non-idiopathic pulmonary fibrosis (non-IPF) ILD once comorbidities, lung function, and treatment are considered. METHODS:In the prospective INSIGHTS-ILD registry (data cut 17 September 2025), we compared men and women with non-IPF ILD using descriptive analyses and Cox models with prespecified adjustment steps: model A (age, comorbidity count, and smoking), model B (A + forced vital capacity [FVC] and diffusing capacity of the lung for carbon monoxide [DLCO]), and model C (B + antifibrotic therapy). Prespecified subgroup analyses included age strata (≤55 and >55 years) and ILD entities. Longitudinal FVC and DLCO trajectories were assessed over 24 months. RESULTS:Among 883 patients (483 men and 400 women), exposures and disease entities differed significantly by sex: men reported more occupational/environmental exposures and had higher rates of fibrotic idiopathic interstitial pneumonia, whereas women more frequently had autoimmune-related ILD and a family history of ILD. Men had a higher comorbidity burden and more often received antifibrotic therapy at baseline. Survival was shorter in men (HR: 1.51; 95% CI: 1.03-2.21), but this association disappeared after adjustment in model A (HR: 1.04; 95% CI: 0.65-1.68), model B (HR: 1.03; 95% CI: 0.61-1.74), and model C (HR: 1.04; 95% CI: 0.62-1.77). Progression-free survival and transplant-free survival showed no consistent sex-related differences. Longitudinal FVC and DLCO declines were modest and largely parallel in both sexes, with no significant between-group differences. Findings were similar across age groups and ILD entities. CONCLUSION:Men and women with non-IPF ILD differ in exposures, phenotypes, and comorbidities, but after accounting for these factors, sex is not an independent predictor of survival or functional progression. Risk assessment should therefore primarily be based on objective disease characteristics rather than sex alone.

Background Interstitial lung diseases (ILD) represent an interdisciplinary clinical challenge and are not uncommonly associated with rheumatological diseases. Interstitial lung disease multidisciplinary meetings (ILD-MDM) provide a structured platform for interdisciplinary case discussions and decision making. Despite their great importance in patient care, data on the prevalence, structure and function of ILD-MDM in Germany are lacking. Objective The aim of the study was to assess the current status of ILD-MDM in German hospitals to gain insights into their composition, processes and potential for optimization. Material and methods A web-based survey was conducted via SurveyMonkey under the auspices of the German Society for Rheumatology and Clinical Immunology (DGRh) and in collaboration with the German Respiratory Society (DGP) and the German Radiological Society (DRG). A standardized questionnaire captured information on the participating specialist disciplines, organizational structures as well as the content and challenges of local ILD-MDM. The analysis was conducted descriptively. Results A total of 125 physicians from 15 federal states in Germany participated. Pulmonologists (93.6 %), radiologists (86.4 %), rheumatologists (59.2 %) and pathologists (57.6 %) are the most commonly represented members of ILD-MDM. The majority of ILD-MDMs are conducted either in person (50 %) or in a hybrid format (31.5 %) and are held on a weekly basis (41.1 %). Of all patient cases discussed, two thirds receive a definitive diagnosis and treatment recommendation. Conclusion The findings reveal a high acceptance and prevalence of ILD-MDM in Germany but also highlight potential areas for improvement, particularly regarding interdisciplinary participation, technical infrastructure and standardization.

Background Interstitial lung diseases (ILDs) comprise a group of more than 200 different subtypes. They vary widely in terms of incidence, prognosis and treatment, yet real-life data from Germany are sparse.Methods The prospective Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases (EXCITING)-ILD registry included patients with all different ILD subtypes from different healthcare settings. Follow-up ranged from 36 months to 5 years. Data were analysed descriptively. Baseline characteristics, diagnostic and treatment information are presented as absolute numbers and percentages. The Wilcoxon signed-rank sum test was used to quantify differences between groups. Line plots and bar plots were used for graphical presentation.Results A total of 601 patients (60.7% men, mean age 64.3 years) from 32 centres were included in the EXCITING-ILD registry. The most common subtypes were sarcoidosis with 26.6% (n=160) and idiopathic pulmonary fibrosis (IPF) with 25.3% (n=152). Pulmonary hypertension was present in 8.7% of patients (n=52), with high incidences in connective tissue disease-associated ILD (16.3%) and pneumoconiosis (27.3%). The mean forced vital capacity was 76.4% predicted, and the mean DLCO-SB (diffusing capacity for carbon monoxide) was 54.1% predicted. The mean time to diagnosis was 38.8 months (SD 64.4) and was significantly shorter when the diagnosis was made after multidisciplinary discussion (31.6 vs 49.2 months, p<0.001). The frequency of surgical lung biopsies decreased over time in the registry, whereas the proportion of cryobiopsies showed a notable increase. In IPF, the number of patients treated with antifibrotics increased from 35.2% before 2015 to 48.4% in 2019.Conclusion The EXCITING-ILD registry describes the frequency of ILD subtypes, ILD-related impairments, selected comorbidities and diagnostic and treatment patterns in a representative German population.

Background This study aims to report real-life data on the characteristics and treatment patterns of patients with fibrosing interstitial lung disease (ILD; except idiopathic pulmonary fibrosis) across multiple specialised centres in Germany. Eligibility criteria included ILD affecting >10% of lung parenchyma on high-resolution computed tomography, a single breath diffusion capacity for carbon monoxide ( D LCO ) ≤80% predicted and active treatment of lung disease. Methods As of the interim analysis cut-off, 655 patients (mean± sd age 65.9±11.7 years, 54.5% male) were included. The most common ILD subtypes were fibrosing hypersensitivity pneumonitis (31.2%), fibrosing ILD (22.0%), rheumatoid arthritis and connective tissue disease ILDs (13.0%) and unclassifiable fibrosing ILD (13.0%). Results Lung function metrics included total lung capacity at 68.3±17.6% predicted, forced vital capacity at 69.8±19.8% predicted, forced expiratory volume in 1 s at 73.7±19.5% predicted and D LCO at 33.8±15.6% predicted. Current treatments included oral steroids (62.6%), antifibrotic therapy (50.7%), azathioprine (14.4%), methotrexate (10.2%) and mycophenolate mofetil (11.1%). Patients on antifibrotic therapy were typically older at diagnosis and registry inclusion, more often male, had more comorbidities, a lower 6-min walk distance and reduced lung function metrics compared with those not on antifibrotic therapy. Notably, 27.3% of the patients on antifibrotic therapy did not meet progression criteria (INBUILD), whereas 40.1% of patients not receiving antifibrotic therapy did meet those criteria. Conclusion The patient characteristics observed align with those observed in randomised controlled trials and other noninterventional studies. Patients on antifibrotic therapy generally had more severe disease profiles.

Interstitielle Lungenerkrankungen (ILD) stellen eine interdisziplinäre klinische Herausforderung dar und sind nicht selten mit rheumatologischen Erkrankungen assoziiert. ILD-Boards bieten eine strukturierte Plattform zur interdisziplinären Fallbesprechung und Entscheidungsfindung. Trotz ihrer hohen Bedeutung in der Patientenversorgung fehlt es an Daten zur Verbreitung, Struktur und Arbeitsweise von ILD-Boards in Deutschland. Ziel der Studie war die Erfassung des Status quo von ILD-Boards an deutschen Kliniken sowie deren Zusammensetzung, Abläufe und Optimierungspotenziale. Es erfolgte eine webbasierte Umfrage via SurveyMonkey unter der Schirmherrschaft der Deutschen Gesellschaft für Rheumatologie und Klinische Immunologie (DGRh) und in Zusammenarbeit mit der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin (DGP) sowie der Deutschen Röntgengesellschaft (DRG). Ein standardisierter Fragebogen erfasste Informationen zu beteiligten Fachdisziplinen, organisatorischen Strukturen sowie Inhalten und Herausforderungen der lokalen ILD-Boards. Die Auswertung erfolgte deskriptiv. An der Studie nahmen insgesamt 125 Ärzte aus 15 Bundesländern teil. Pneumologen (93,6

Abstract Background Interstitial lung diseases (ILD) comprise a heterogeneous group of mainly chronic lung diseases with different disease trajectories. Progression (PF-ILD) occurs in up to 50% of patients and is associated with increased mortality. Methods The EXCITING-ILD (Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases) registry was analysed for disease trajectories in different ILD. The course of disease was classified as significant (absolute forced vital capacity FVC decline > 10%) or moderate progression (FVC decline 5–10%), stable disease (FVC decline or increase < 5%) or improvement (FVC increase ≥ 5%) during time in registry. A second definition for PF-ILD included absolute decline in FVC % predicted ≥ 10% within 24 months or ≥ 1 respiratory-related hospitalisation. Risk factors for progression were determined by Cox proportional-hazard models and by logistic regression with forward selection. Kaplan-Meier curves were utilised to estimate survival time and time to progression. Results Within the EXCITING-ILD registry 28.5% of the patients died (n = 171), mainly due to ILD (n = 71, 41.5%). Median survival time from date of diagnosis on was 15.5 years (range 0.1 to 34.4 years). From 601 included patients, progression was detected in 50.6% of the patients (n = 304) with shortest median time to progression in idiopathic NSIP (iNSIP; median 14.6 months) and idiopathic pulmonary fibrosis (IPF; median 18.9 months). Reasons for the determination as PF-ILD were mainly deterioration in lung function (PFT; 57.8%) and respiratory hospitalisations (40.6%). In multivariate analyses reduced baseline FVC together with age were significant predictors for progression (OR = 1.00, p < 0.001). Higher GAP indices were a significant risk factor for a shorter survival time (GAP stage III vs. I HR = 9.06, p < 0.001). A significant shorter survival time was found in IPF compared to sarcoidosis (HR = 0.04, p < 0.001), CTD-ILD (HR = 0.33, p < 0.001), and HP (HR = 0.30, p < 0.001). Patients with at least one reported ILD exacerbation as a reason for hospitalisation had a median survival time of 7.3 years (range 0.1 to 34.4 years) compared to 19.6 years (range 0.3 to 19.6 years) in patients without exacerbations (HR = 0.39, p < 0.001). Conclusion Disease progression is common in all ILD and associated with increased mortality. Most important risk factors for progression are impaired baseline forced vital capacity and higher age, as well as acute exacerbations and respiratory hospitalisations for mortality. Early detection of progression remains challenging, further clinical criteria in addition to PFT might be helpful.

Die Sektion 7 "Klinische Pneumologie" hat aktuell 1527 Mitglieder:innen. 37% der Mitglieder der Sektion 7 sind Frauen. Die meisten Mitglieder:innen gehören der Altersgruppe 35–45 Jahre an. Die Mitglieder:innen stammen aus allen Bereichen der Pneumologie, wie Universitätskliniken, Fachkliniken, Versorgungshäusern und vielen Praxen. Das Spektrum der in der Sektion 7 vertretenen Interessen und Themen ist daher breit gefächert. Vor dem Hintergrund der bereits jetzt existenten inhaltlichen Schwerpunkte der Sektion sollen im Rahmen der geplanten Reform der Sektionsstruktur der DGP und der vorgesehenen Einrichtung von Assemblies aus der Sektion 7 "Klinische Pneumologie" zwei Sektionen entstehen, nämlich die Sektion "COPD" und die Sektion "Interstitielle und seltene Lungenerkrankungen". Diese Sektionen sollen zukünftig mit anderen Sektionen in der Assembly A (Klinische Pneumologie) zusammengeschlossen werden.

Hintergrund Interstitielle Lungenerkrankungen (ILD) sind eine heterogene Gruppe von überwiegend chronischen Lungenerkrankungen. Bei bis zu 50% der Patienten kommt es zu einer Progression.

Background Interstitial lung diseases (ILD) comprise a heterogeneous group of mainly chronic lung diseases with more than 200 entities and relevant differences in disease course and prognosis. Little data is available on hospitalisation patterns in ILD. Methods The EXCITING-ILD (Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases) registry was analysed for hospitalisations. Reasons for hospitalisation were classified as all cause, ILD-related and respiratory hospitalisations, and patients were analysed for frequency of hospitalisations, time to first non-elective hospitalisation, mortality and progression-free survival. Additionally, the risk for hospitalisation according to GAP index and ILD subtype was calculated by Cox proportional-hazard models as well as influencing factors on prediction of hospitalisation by logistic regression with forward selection. Results In total, 601 patients were included. 1210 hospitalisations were recorded during the 6 months prior to registry inclusion until the last study visit. 800 (66.1%) were ILD-related, 59.3% of admissions were registered in the first year after inclusion. Mortality was associated with all cause, ILD-related and respiratory-related hospitalisation. Risk factors for hospitalisation were advanced disease (GAP Index stages II and III) and CTD (connective tissue disease)-ILDs. All cause hospitalisations were associated with pulmonary hypertension (OR 2.53, p = 0.005). ILD-related hospitalisations were associated with unclassifiable ILD and concomitant emphysema (OR = 2.133, p = 0.001) as well as with other granulomatous ILDs and a positive smoking status (OR = 3.082, p = 0.005). Conclusion Our results represent a crucial contribution in understanding predisposing factors for hospitalisation in ILD and its major impact on mortality. Further studies to characterize the most vulnerable patient group as well as approaches to prevent hospitalisations are warranted.

Das INSIGHTS-ILD Register (DRKS00027389, BfArM 7562) dokumentiert die Charakteristika und den Behandlungsverlauf von Patienten mit fibrosierender interstitieller Lungenerkrankung (fILD). Eingeschlossen werden Patienten mit fILD (keine IPF), einer DLCO≤ 80% des Sollwertes, einem Parenchymbefall der Lunge>10% im HRCT und die bereits eine antiinflammatorische, immunmodulatorische und/oder antifibrotische Therapie erhalten.

The evaluation of a patient with interstitial lung disease (ILD) includes assessment of clinical, radiological, and often histopathological data. As there were no specific recommendations to guide the evaluation of patients under the suspicion of an ILD within the German practice landscape, this position statement from an interdisciplinary panel of ILD experts provides guidance related to the diagnostic modalities which should be used in the evaluation of ILD. This includes clinical assessment rheumatological evaluation, radiological examinations, histopathologic sampling and the need for a final discussion in a multidisciplinary team.

Zusammenfassung Die Beurteilung von Patienten mit v. a. einer interstitiellen Lungenerkrankung (ILD) umfasst die Beurteilung klinischer, radiologischer und oft histopathologischer Daten. Da bislang noch keine dezidierten Empfehlungen für die Evaluation bei Verdacht auf eine ILD in Deutschland existierten, war es Ziel dieses interdisziplinären Konsensusstatements, eine praktische Orientierungshilfe für den klinischen Alltag in Bezug auf die interdisziplinäre Diagnostik der ILDs zu geben. Dazu gehören die umfassende klinisch-pneumologische und in vielen Fällen auch eine rheumatologische Beurteilung, radiologische Diagnostik sowie Probenentnahme zur histopathologischen Evaluation sowie die abschließende Diskussion im multidisziplinären Team.

Interstitial lung diseases (ILDs) are a large group of pulmonary disorders characterized histologically by the cardinal involvement of the pulmonary interstitium. The prototype of ILDs is idiopathic pulmonary fibrosis (IPF), an incurable disease characterized by progressive distortion and loss of normal lung architecture through unchecked collagen deposition. Acute exacerbations are dramatic events during the clinical course of ILDs, associated with high morbidity and mortality. Infections, microaspiration, and advanced lung disease might be involved in the pathogenesis of acute exacerbations. Despite clinical scores, the prediction of the onset and outcome of acute exacerbations is still inaccurate. Biomarkers are necessary to characterize acute exacerbations better. We review the evidence for alveolar epithelial cell, fibropoliferation, and immunity molecules as potential biomarkers for acute exacerbations of interstitial lung disease.

Hintergrund ILDs fassen über 200 verschiedene Erkrankungen mit Unterschieden in Verlauf und Prognose zusammen. Es liegen nur wenige Daten über Hospitalisierungsmuster bei ILDs vor.

The high incidence of thrombotic events suggests a possible role of the contact system pathway in COVID-19 pathology. In this study, we determined the altered levels of factor XII (FXII) and its activation products in critically ill patients with COVID-19 in comparison with patients with severe acute respiratory distress syndrome related to the influenza virus (acute respiratory distress syndrome [ARDS]-influenza). Compatible with those data, we found rapid consumption of FXII in COVID-19 but not in ARDS-influenza plasma. Interestingly, the lag phase in fibrin formation, triggered by the FXII activator kaolin, was not prolonged in COVID-19, as opposed to that in ARDS-influenza. Confocal and electron microscopy showed that increased FXII activation rate, in conjunction with elevated fibrinogen levels, triggered formation of fibrinolysis-resistant, compact clots with thin fibers and small pores in COVID-19. Accordingly, clot lysis was markedly impaired in COVID-19 as opposed to that in ARDS-influenza. Dysregulated fibrinolytic system, as evidenced by elevated levels of thrombin-activatable fibrinolysis inhibitor, tissue-plasminogen activator, and plasminogen activator inhibitor-1 in COVID-19 potentiated this effect. Analysis of lung tissue sections revealed widespread extra- and intravascular compact fibrin deposits in patients with COVID-19. A compact fibrin network structure and dysregulated fibrinolysis may collectively contribute to a high incidence of thrombotic events in COVID-19.