Die Aktualisierung der American Thoracic Society/European Respiratory Society(ATS/ERS)-Klassifikation der interstitiellen Pneumonien von 2025 erweitert deren Einteilung über rein idiopathische Entitäten hinaus und umfasst nun auch Krankheitsbilder mit identifizierbaren Auslösern [1]. Zentraler Bestandteil dieser Aktualisierung ist die terminologische Neuausrichtung der Klassifikation zu einem deskriptiven, primär morphologisch orientierten Konzept mit dem Ziel der klaren Unterscheidung von radiologischen und histologischen Mustern auf der einen und von klinisch definierten Erkrankungen auf der anderen Seite. Die akute interstitielle Pneumonie (AIP) wird nun als idiopathische diffuse alveoläre Schädigung (DAD) bezeichnet, die desquamative interstitielle Pneumonie (DIP) als Alveolarmakrophagenpneumonie (AMP). Das Muster der bronchiolozentrischen interstitiellen Pneumonie (BIP) wird als eigenständiges morphologisches Muster eingeführt, der Begriff der Hypersensitivitätspneumonitis (HP) soll künftig ausschließlich der multidisziplinären Diagnose vorbehalten sein. Darüber hinaus bietet die aktualisierte Klassifikation ein biologisch begründetes und klinisch anwendbares System zur Kategorisierung in interstitielle und alveoläre Füllmuster und die auch prognostisch relevante Aufteilung in fibrotische und nichtfibrotische Verläufe. Hervorzuheben ist zudem eine stärkere Betonung der transparenten Angabe der diagnostischen Sicherheit im interdisziplinären Board für interstitielle Lungenerkrankungen (ILD-Board), inklusive der Vergabe von „provisorischen“ Diagnosen bzw. Verwendung des Begriffs der „unklassifizierbaren ILD“ bei niedriger und sehr niedriger diagnostischer Sicherheit. Die aktuelle Klassifikation kann helfen, den diagnostischen Ablauf und den Entscheidungsprozess im ILD-Board zu standardisieren und transparenter zu gestalten und dadurch auch die klinische Versorgung zu optimieren. Es ist zu erwarten, dass dieses Update die Grundlage für künftige Forschung und neue Erkenntnisse im Bereich der ILD sein wird. Daneben soll jedoch nicht unerwähnt bleiben, dass die neue Klassifikation hinsichtlich der Einführung des Musters der BIP und der provisorischen Entität der „idiopathischen BIP“ von einigen ExpertInnen kritisch beurteilt wird. Befürchtet wird konkret, dass durch die stärkere Betonung eines morphologischen Musters und die terminologische Abgrenzung zur HP die diagnostische Aufmerksamkeit für potenziell auslösende Expositionen abgeschwächt werden könnte.

Background/Objectives: Immunotherapy has emerged as an important field of research in non-small-cell lung cancer (NSCLC) and has demonstrated promising results in clinical practice. In recent years, multiple studies have been conducted, increasing the amount of available data. Therefore, the aim of this systematic review is to assess the combination of perioperative immunotherapy with chemotherapy compared to chemotherapy only in patients with resectable NSCLC in terms of survival, pathological response, and adverse events. Methods: The clinical databases PubMed, Cochrane Library, ClinicalTrials.gov, and the World Health Organization International Clinical Trials Registry Platform (WHO ICTRP) were systematically searched, up to March 2026. A two-step selection process served as the screening for eligibility, in which the assessment was based on pre-defined inclusion and exclusion criteria. This process was visualized via a PRISMA diagram. For each included study, the risk of bias was assessed with the help of the Cochrane Risk of Bias 2.0 tool and the Newcastle Ottawa Scale. A narrative synthesis was performed due to heterogeneity. Data were extracted into tables. Results: A total of 16 studies, involving 4646 patients in total, met the eligibility criteria, and their data on study population, intervention, comparison, and outcome were extracted into tabular form. Survival and pathological response rates are continuously higher in patients treated with immunochemotherapy. Findings on adverse events differed across the individual studies, though the results indicate an increased risk of treatment-related adverse events (TRAEs) in patients undergoing the combined treatment approach. Discussion/Conclusions: Chemoimmunotherapy leads to superior clinical outcomes in terms of survival and pathological response rates, though the trend towards a higher incidence and severity of TRAEs warrants further research. The interpretation of findings is limited by differences in study characteristics, mechanism of conduct, and endpoints between the individual studies.

The update of the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of interstitial pneumonias published in 2025 extends the oprior classification system beyond merely idiopathic entities, now also encompassing entities with identifiable triggers [1]. The core component of this update is the terminological reorientation of the classification towards a descriptive, primarily morphologically oriented concept with the aim of a clear differentiation of radiological and histological patterns from clinically defined diseases. Acute interstitial pneumonia (AIP) is now designated as idiopathic diffuse alveolar damage (DAD), and desquamative interstitial pneumonia (DIP) as alveolar macrophage pneumonia (AMP). The pattern of bronchiolocentric interstitial pneumonia (BIP) has been introduced as an independent morphological pattern, reserving the term hypersensitivity pneumonitis (HP) exclusively for multidisciplinary diagnosis in the future. Furthermore, the updated classification provides a biologically founded and clinically applicable categorization system of interstitial and alveolar filling patterns and the prognostically relevant separation into fibrotic and nonfibrotic phenotypes. Also, there is a stronger emphasis on the transparent reporting of the diagnostic confidence by interstitial lung disease (ILD) boards, including the use of "provisional" diagnoses or of the term "unclassifiable ILD" in cases of low and very low diagnostic certainty. The current classification can help to standardize the diagnostic course and to make the decision-making process in the ILD board more transparent. It is to be expected that this update will be the foundation for future research and for new knowledge in the field of ILD. In addition, it should however not be ignored that the new classification has been critisized by some experts with respect to the introduction of the pattern of BIP and the provisional entity of "idiopathic BIP". It is feared that this change in terminology emphasizing the morphological pattern rather than causative factors could lead to reduced attention concerning potential trigger exposures in the diagnostic process.

Interstitial lung abnormalities (ILA) are findings detected on computed tomography (CT) that potentially reflect early stages of interstitial lung disease (ILD). Their prevalence ranges between 3-10% in the general population, with higher rates observed in older individuals and smokers. ILA include bilateral and nonhypostasis-related ground-glass opacities, reticular abnormalities, traction bronchiectasis, lung architectural distortion and honeycombing, affecting more than 5% of a lung zone. The risk of progression to ILD varies between 20-80%, depending on the ILA subtype and associated risk factors. Clinical progression and risk factors include advanced age, nicotine exposure, inhaled noxious substances, thoracic surgical procedures, pneumotoxic treatment and abnormal pulmonary function parameters. Radiologically, fibrotic ILA with subpleural and basal predominance as well as larger extent of lung involvement are significantly associated with increased risk of progression. The clinical management is based on a structured evaluation including high-resolution CT, lung function diagnostics and risk stratification. In the absence of signs of advanced fibrotic changes, individualized follow-up intervals ranging from 6-36 months are recommended, depending on the patient's risk profile. This position paper provides practical recommendations for managing ILA, in line with current international guidelines, while considering new evidence on genetic risk factors, imaging features associated with progression and clinical predictors. The aim is an early identification of high-risk patients and avoidance of unnecessary diagnostic or therapeutic interventions.

Interstitielle Lungenanomalien (ILA) sind in der Computertomographie (CT) detektierte Befunde, die potenziell frühe Stadien interstitieller Lungenerkrankungen (ILD) widerspiegeln. Ihre Prävalenz variiert zwischen 3 und 10

Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is a major driver of premature mortality in patients with rheumatoid arthritis (RA). Detection of RA-ILD is crucial but requires awareness among the treating physicians. To date, however, there is no international recommendation concerning screening for ILD in RA patients. After a systematic literature review, the modified Delphi technique in combination with the nominal group technique was used to provide a Delphi consensus statement elaborated by an expert panel of pneumonologists, rheumatologists, and a radiologist. Based on the available evidence, several clusters of questions were defined and discussed until consent was reached. A screening algorithm for ILD in patients with RA based on clinical signs, respiratory symptoms, and risk factors has been developed. Further, the recommendations address diagnostic tools for RA-ILD and the follow-up of RA patients qualifying for ILD screening.

Consolidation immunotherapy with immune checkpoint Inhibitor (ICI) Durvalumab is an effective treatment for inoperable stage III non-small cell lung cancer (NSCLC) patients with a PD-L1 expression ≥ 1

PURPOSEPalliative radiotherapy (PRT) toward the end of life (EOL) in advanced tumor disease is contentious. Although EOL RT can alleviate cancer-related symptoms, relief typically occurs weeks to months after treatment, potentially compromising the quality of life of patients during the final stages. This study aims to assess factors influencing the decision-making process regarding EOL RT.MATERIALS AND METHODSThis retrospective study of a real-world cohort included 684 consecutive patients with a diagnosis of a solid tumor who died between 2017 and 2021. In these patients, factors potentially influencing the administration of EOL RT were analyzed.RESULTSOf the 684 patients, 164 received PRT, with 60 (36.6%) receiving EOL RT within the last 30 days of life. The median time from the last RT session to death was 55 days. Significant factors influencing EOL RT administration were age ≤65 years (odds ratio [OR], 1.75 [95% CI, 1.02 to 3.01]), Union for International Cancer Control stage IV (OR, 2.77 [95% CI, 1.41 to 5.46]), lung cancer (OR, 2.16 [95% CI, 1.00 to 4.68]), palliative care referral (OR, 1.80 [95% CI, 0.98 to 3.30]), systemic anticancer treatment ≤30 days before death (OR, 1.87 [95% CI, 1.05 to 3.33]), and Eastern Cooperative Oncology Group performance status ≥2 (OR, 3.73 [95% CI, 1.88 to 7.40]). Furthermore, RT near the EOL was more likely administered at multiple sites (OR, 2.08 [95% CI, 1.00 to 4.29]) and with ≤5 fractions (OR, 2.37 [95% CI, 1.23 to 4.57]), while being associated with lower response rates (OR, 0.43 [95% CI, 0.21 to 0.86]) and increased therapy discontinuation (OR, 4.40 [95% CI, 1.45 to 13.37]).CONCLUSIONThese findings highlight varying RT patterns influenced by specific factors, demonstrating the complexity of EOL treatment decisions in advanced cancer care. Identifying key factors for personalized, patient-centered EOL RT decisions warrants further investigation.

Background Skeletal morbidity in patients with cancer has a major impact on the quality of life, and preserving bone health while improving outcomes is an important goal of modern antitumor treatment strategies. Despite their widespread use in early disease stages, the effects of immune checkpoint inhibitors (ICIs) on the skeleton are still poorly defined. Here, we initiated a comprehensive investigation of the impact of ICIs on bone health by longitudinal assessment of bone turnover markers in patients with cancer and by validation in a novel bioengineered 3D model of bone remodeling.Methods An exploratory longitudinal study was conducted to assess serum markers of bone resorption (C-terminal telopeptide, CTX) and formation (procollagen type I N-terminal propeptide, PINP, and osteocalcin, OCN) before each ICI application (programmed cell death 1 (PD1) inhibitor or programmed death-ligand 1 (PD-L1) inhibitor) for 6 months or until disease progression in patients with advanced cancer and no evidence of bone metastases. To validate the in vivo results, we evaluated osteoclast (OC) and osteoblast (OB) differentiation on treatment with ICIs. In addition, their effect on bone remodeling was assessed by immunohistochemistry, confocal microscopy, and proteomics analysis in a dynamic 3D bone model.Results During the first month of treatment, CTX levels decreased sharply but transiently. In contrast, we observed a delayed increase of serum levels of PINP and OCN after 4 months of therapy. In vitro, ICIs impaired the maturation of preosteoclasts by inhibiting STAT3/NFATc1 signaling but not JNK, ERK, and AKT while lacking any direct effect on osteogenesis. However, using our bioengineered 3D bone model, which enables the simultaneous differentiation of OB and OC precursor cells, we confirmed the uncoupling of the OC/OB activity on exposure to ICIs by demonstrating impaired OC maturation along with increased OB differentiation.Conclusion Our study indicates that the inhibition of the PD1/PD-L1 signaling axis interferes with bone turnover and may exert a protective effect on bone by indirectly promoting osteogenesis.

In many cases sarcoidosis is a multisystemic disease that requires interdisciplinary medical cooperation in the diagnostics, treatment and medical care during follow-up. Due to the often chronic course, it is of utmost importance to include patients with their priorities and wishes at an early stage and extensively in disease management and to establish a shared decision making whenever possible. In the process of writing this joint position paper, the expert group on interstitial and orphan lung diseases of the Austrian Society for Pulmonology and the working group on rheumatological lung disorders of the Austrian Society for Rheumatology and Rehabilitation sought to include patient advocacy groups as well as experts for rare organ manifestations of sarcoidosis. This position paper is not only meant to reflect current scientific and clinical standards but should also focus the national expertise and by networking and exchange to be a first step to strengthen cooperation between stakeholders to ultimately improve care for patients with sarcoidosis.

In unserem Fallbericht stellen wir einen 79-jährigen Mann vor, der sich mit Uveitis, Arthritis, Perichondritis, Fieber und Vaskulitis der kleinen Gefäße vorstellte. Die Laborwerte zeigten anhaltend erhöhte Entzündungsmarker. Im Lauf eines Jahrs besserten sich seine Symptome und Entzündungsmarker nur unter Glukokortikoidbehandlung, und trotzdem er eine interstitielle Lungenerkrankung entwickelte. In der Brust-CT zeigten sich parenchymale Trübungen. Eine bronchoalveoläre Lavage und eine Lungenbiopsie zeigten eine neutrophile Alveolitis und eine parenchymatöse Entzündung. Nachdem Ergebnisse ausblieben, wurde die Möglichkeit einer VEXAS-Erkrankung in Betracht gezogen und später durch molekulare Tests bestätigt. VEXAS ist eine kürzlich beschriebene entzündliche Erkrankung, die durch Mutationen im UBA1-Gen verursacht wird. Die Symptome sind vielfältig und umfassen Fieber, Knorpelentzündung, Lungenentzündung, Vaskulitis und makrozytäre Anämie. Ein charakteristisches Merkmal sind zytoplasmatische Einschlüsse in myeloischen und erythroiden Vorläuferzellen im Knochenmark. Hier berichten wir über unseren ersten Fall von VEXAS Syndrom.

Background Systemic anticancer treatment (SACT) for advanced cancer patients with limited prognosis before death is associated with high toxicity and reduced quality of life. Guidelines discourage this approach as low-value care. However, a significant number of patients continue to receive SACT in the last 30 days of life. Materials and methods A retrospective study was carried out at the University Hospital Krems, encompassing the analysis of patients who were diagnosed with a solid tumor and died between 2017 and 2021, with a particular focus on the use of end-of-life (EOL) SACT. Results A total of 685 patients were included in the study. SACT was applied in 342 (49.9%) patients, of whom 143 (41.8%, total population: 20.9%) patients received SACT within the last 30 days of life. Median time from last SACT to death was 44.5 days. The analysis of potential factors impacting the administration of EOL SACT revealed the following significant findings: type of SACT [P < 0.001, targeted therapy odds ratio (OR) 5.09, 95% confidence interval (CI) 2.26-11.48; chemotherapy/targeted therapy OR 3.60, 95% CI 1.47-8.82; immune checkpoint inhibitor OR 2.32, 95% CI 1.37-3.92], no referral to palliative care (PC) (P = 0.009, OR 1.86, 95% CI 1.16-2.96), no admission to PC ward (P < 0.001, OR 2.70, 95% CI 1.67-4.35), and poor Eastern Cooperative Oncology Group (ECOG) performance status (≥2, P < 0.001, OR 3.35, 95% CI 1.93-5.83). Conclusion The timing of SACT near the EOL is significantly influenced by several factors, including the type of SACT, referral to PC services, admission to PC unit, and ECOG performance status. These findings underscore the complexity of treatment decisions in advanced cancer care and highlight the need for personalized, patient-centered approaches that consider both clinical and patient-related factors to optimize care at the EOL.

Die Sarkoidose ist in vielen Fällen eine Multisystemerkrankung, die eine interdisziplinäre medizinische Zusammenarbeit in Diagnostik, Therapie und in der medizinischen Betreuung im Verlauf erfordert. Aufgrund des oft chronischen Verlaufes ist es besonders wichtig, Patientinnen und Patienten mit ihren Prioritäten und Wünschen frühzeitig und umfassend in die medizinische Betreuung einzubinden und, wenn möglich, ein „shared decision making“ zu etablieren. Beim Verfassen dieses gemeinsamen Positionspapieres war es der Expertengruppe für interstitielle Lungenerkrankungen und „orphan diseases“ der Österreichischen Gesellschaft für Pneumologie sowie der Arbeitsgruppe Rheuma und Lunge der Österreichischen Gesellschaft für Rheumatologie und Rehabilitation ein besonderes Anliegen, sowohl PatientInnenvertreter als auch ExpertInnen für seltenere Organmanifestationen der Sarkoidose einzubeziehen. Dieses Positionspapier soll nicht nur ein Spiegel der aktuellen klinischen und wissenschaftlichen Praxis sein, sondern auch die nationale Expertise bündeln und durch Vernetzung und Austausch ein erster Schritt zu einer Stärkung der Betreuungsstruktur von PatientInnen mit Sarkoidose sein.

Abstract Background: Precision cancer medicine aims to identify the right drug for the right patient, enriching for patients more likely to respond to a particular treatment. This paradigm is gaining importance during the early clinical lifecycle of a new potential drug to improve patient-centric trial designs, drive clinical success and eventually increase approval rates - enlarging the therapeutic arsenal available for oncology patients. To optimize the chance of success with our A2AR-selective antagonist, EXS-21546 (546; NCT04727138, discovered in collaboration with Evotec), we have identified an adenosine-induced immunosuppression biomarker signature (adenosine burden score or ABS) for clinical trial patient selection that also correlates with checkpoint inhibitor (CI) response prediction in ex vivo primary models. Here we present transcriptional and functional data mapping adenosine burden at the single cell level, and investigate subsequent modulation through antagonism of A2AR with 546, combination effects with CI, to prioritize patients for 546+CI therapy. Methods: By leveraging disease-relevant primary human tissues together with matched single cell and bulk transcriptomics, we assess adenosine-induced anticancer immune suppression and show initial biological confirmation of patient selection methodology and combination therapy effects with a translatable high content imaging platform (Kornauth et al 2021 & Snidjer et al 2017). Results: The ABS detects adenosine rich microenvironments with greater specificity and sensitivity than other published signatures. Validating the ABS in TGCA, we found the ABS anti-correlates with a validated predictor of anti-PD-1 therapy success (TIS, Damotte et al 2019), unraveling that high-adenosine/ABS cases are among patients least likely to respond to immunotherapy (low TIS). A2AR antagonism with 546 demonstrated a reduction of the adenosine burden, and restored the CI response potential as addressed by the ABS and TIS, respectively. Further immune reactivation was seen with antagonism of adenosine signaling by ‘546/CI combination ex vivo in primary tissues pre-selected with our ABS signature. Conclusions: Combining deep learning of single cell functional and multi-omics profiling data of disease relevant primary model systems, we model the association of the immune response potential to A2AR antagonism in cancer to define a biomarker signature to predict patients likely to benefit from A2AR antagonism and CI. This will be confirmed and validated retrospectively in an ongoing clinical study of 546 in two cancer indications. Citation Format: Isabella Alt, Robert Sehlke, Anna Lobley, Claudia Baumgaertler, Maja Stulic, Klaus Hackner, Lucia Dzurillova, Edgar Petru, Laudia Hadjari, Judith Lafleur, Josef Singer, Nikolaus Krall, Jozef Šufliarsky, Lukas Hefler, Thorsten Füreder, Christina Taubert, Andrew Payne, Christophe Boudesco, Gregory Ian Vladimer. Identification of transcript adenosine fingerprint to enrich for A2AR and PD-1 inhibition responders [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 2151.