Bronchiectasis is a chronic, often progressive respiratory disease characterized by irreversible dilation of the bronchi. It is etiologically heterogeneous and frequently associated with a significant symptom burden, multiple complications, and reduced quality of life. In recent years, the global prevalence of bronchiectasis has increased markedly, placing a substantial economic burden on healthcare systems. These consensus-based guidelines are the first German-language guidelines focused on the management of bronchiectasis in adults. They underscore the critical role of thoracic imaging - particularly computed tomography - in diagnosing and distinguishing bronchiectasis and highlight the importance of identifying the underlying etiology in guiding treatment decisions. The guidelines provide comprehensive recommendations for both pharmacological and non-pharmacological treatment strategies. Non-drug interventions include smoking cessation, physiotherapy, physical training, pulmonary rehabilitation, noninvasive ventilation, thoracic surgery, and lung transplantation. Pharmacological therapies emphasize the long-term use of mucolytics, bronchodilators, anti-inflammatory agents, and antibiotics. In addition, the guidelines address the management of upper airway involvement, common comorbidities, and acute exacerbations. They also cover sociomedical issues, disability rights, and the role of patient education and self-management in optimizing care. Special life stages - such as transition from pediatric to adult care, family planning, pregnancy, parenthood, and palliative care - are also considered. The overarching goal was to promote comprehensive, consensus-driven, and patient-centered care that accounts for individual risks and needs.

Dear Editors, Lung transplantation (LuTX) is an established and effective therapeutic option for patients with end-stage lung disease [1]. Over the past decades, advances in surgical techniques, perioperative management, immunosuppressive strategies, and long-term follow-up care have resulted in significant improvements in survival rates, health-related quality of life, and functional capacity after lung transplantation [2]. Consequently, many recipients are able to resume a broad range of physical activities, including high-intensity and endurance sports. Early publications on this topic are available from other solid organ transplant recipients [3]. In this context, participation in high-altitude mountaineering has been documented in carefully selected liver transplant recipients under close medical supervision [4]. In 2015 a transplanted patient reached the highest mountain peak (6.189m, Island Peak, Nepal) ever [5]. Also, lung transplant recipients are able to adapt to altitude and capable of performing prolonged exercise at high altitude after slow ascent [6,7]. In 2017 eight lung transplanted patients successfully summited Mount Kilimanjaro (5.895 m, Tanzania) under guidance of the Vienna lung transplant team [7,8]. Available evidence suggests that transplanted lungs retain the capacity to physiologically adapt to hypobaric hypoxia and can sustain prolonged physical exertion at high altitude, provided that ascent is gradual and appropriate acclimatization is ensured [7]. Eleven lung transplant recipients reached the summit of Mount Jebel Toubkal (4.167 m, Morocco) in 2019 without any adverse events, despite poorer cardiopulmonary performance compared to healthy volunteers [9]. In addition, they show stable immunosuppressive drug trough levels and Torque Teno virus loads indicate good tolerance relative to physical stress (Mühlbacher, 2026, under review).As part of an international medical expedition under guidance of the Vienna lung transplant team and the respective national team leaders, nine transplanted patients (8 patients after lung transplantation, one patient after liver transplantation) were included to climb Mount Aconcagua (6.961m, Argentina) in January 2026. The expedition was supported by an accompanying team of physicians and professional guides. The actual tour planning was carried out by a professional expedition provider (Furtenbach Adventures GmbH, Rum, Austria) in cooperation with a local expedition provider (Grajales Expeditions, Los Penitentes, Mendoza Province), both of whom have many years of experience in planning and safely conducting expeditions. The selection of possible candidates was based on lung function and spiroergometry and was done in accordance with the included transplant centers in Austria, Switzerland, Croatia, Denmark and the USA.Hypoxic conditioning (HC) applied at home as a pre-acclimatization strategy prior to high-altitude exposure may facilitate high-altitude ascents with a reduced risk of developing acute mountain sickness (AMS) [10,11]. However, standardized protocols remain insufficiently defined, and robust scientific data are limited, although pre-acclimatization appears to be a key determinant in the success of rapid ascent expeditions [12]. It is currently unknown how this form of pre-acclimatization affects patients after lung transplantation. To maximize participant safety during the expedition, however, all participants completed a structured home-based HC program comprising at least 200 hours of exposure prior to departure [10]. Participants completed mandatory safety and first aid training prior to the expedition; high flow oxygen systems (Summit Elite System, Summit Oxygen International Ltd) and carbon oxygen cylinders (4L, working pressure 300 bar; Armotech, Czech Republic) where available throughout the expedition for safety reasons.The expedition to Mount Aconcagua (6.962 m, Argentina), followed a structured 19day schedule organized by experienced professional providers. Accordingly, the ascent followed a standard acclimatization protocol via the normal route: approach to Plaza de Mulas Base Camp (BC) (4.350 m) over four days, followed by progressive establishment of higher camps at Plaza Canadá (5.050 m), Nido de Cóndores (5.560 m), and Camp Cólera (6.080 m). The summit attempt (6.961 m) was performed from High Camp Cólera (Figure 1A). Additional days were reserved for weather contingency and descent. Physiological monitoring, including heart rate and peripheral oxygen saturation, was performed using wearable devices provided within the framework of the project. In addition, the Lake Louise Acute Mountain Sickness (AMS) score was assessed daily based on self-reported symptoms documented by the mountaineers [13].On January 24, a lung transplant recipient successfully reached the summit of Mount Aconcagua (6,962 m, Argentina) together with the accompanying expedition team, without the use of supplemental oxygen. The ascent and descent were completed without adverse clinical events. In particular, no signs or symptoms consistent with high-altitude pulmonary edema (HAPE) or high-altitude cerebral edema (HACE) were observed during high-altitude exposure.This 51-year-old male lung transplant recipient (BMI 18.3 kg/m²), transplanted in 2002 for cystic fibrosis, resided at 407 m above sea level. Relevant comorbidities included diabetes mellitus and chronic kidney disease (creatinine: 2.57 mg/dl, November 2025); maintenance immunosuppression consisted of once-daily 0.75mg extended-release tacrolimus in combination with everolimus 0.5mg twice daily. He had prior high-altitude exposure, including Mount Kilimanjaro (5,895 m, Tanzania) [7], without any history of AMS, HAPE, or HACE. Baseline functional assessment demonstrated a maximal oxygen uptake (VO₂max) of 30.9 ml•kg⁻¹•min⁻¹ (90% predicted; maximal workload 140 W) and an FEV₁ of 2.7 L (86% predicted), indicating preserved exercise capacity and stable graft function prior to the expedition. As part of the pre-acclimatization strategy, the lung transplant recipient completed 311 hours of HC over 36 days (Figure 1B). Following HC, hemoglobin levels remained stable (from 13.7 to 13.8 g/dl). As simulated altitude increased during the preacclimatization phase, the participant experienced only mild symptoms, which was accompanied by a corresponding elevation in AMS scores (maximum AMS-Score of 2, Figure 1B). On the mountain he reported only mild to moderate symptoms of AMS (maximum AMS-Score of 5), and no instances of HAPE or HACE occurred at any point during the expedition (Figure 1C Resting SpO2 values progressively declined with increasing altitude from of 89% at BC to 75% at Camp 3. In contrast, heart rate remained relatively stable throughout the stay on the mountain, with average values ranging from 90 bpm at BC to 95 bpm at Camp 3. In summary, selected patients after lung transplantation are able to tolerate and physiologically adapt to high-altitude exposure when preceded by normobaric hypoxic preacclimatization, without experiencing severe high-altitude-associated complications.Moreover, one lung transplant recipient successfully summited Mount Aconcagua without supplemental oxygen, which, to our knowledge, constitutes the highest reported altitude reached following lung transplantation.Ethical approval was granted by the Ethics Committee of the Medical University of Vienna (2105/2025). The study was performed in accordance with national regulations and institutional guidelines. All participants provided written informed consent before participation.

Background Hypersensitivity pneumonitis (HP) is characterized by a complex immunological reaction of the lung that may lead to chronic interstitial lung disease (ILD). Pulmonary hypertension (PH) may aggravate the clinical picture. We aimed to assess the prevalence of PH in two independent cohorts including patients with moderate and severe HP. Patients and Methods We retrospectively analyzed the data of HP patients with severe ILD undergoing lung transplantation (cohort-1) and invited HP patients with mild disease participating at pulmonary rehabilitation for a prospective screening for PH (cohort-2). The presence of PH was assessed by both echocardiography and right heart catheterization (RHC). Results In cohort 1 (n = 55, median age 57 [95% CI 52-60] years, 59% male, forced vital capacity [FVC] 37 [35-45] % predicted), echocardiographic systolic pulmonary arterial pressure (sPAP) was available in 31 patients and showed elevated values (53 [45-63] mmHg). Sixteen patients (29%) had a high probability of pulmonary hypertension (PH; sPAP > 50 mmHg). Based on clinical assessment, a non-invasive evaluation using echocardiography was performed in accordance with current guideline recommendations, followed by invasive assessment with right heart catheterization (RHC) in selected patients. RHC was performed in 21 patients, revealing a mean pulmonary arterial pressure (mPAP) of 28 (24-33) mmHg and a pulmonary vascular resistance (PVR) of 2.8 (2.2-3.4) Wood units; PH was confirmed in 15 patients (71% of those undergoing RHC, 27% of the total cohort). In cohort 2 (n = 96, median age 62 [60-65] years, 66% male, FVC 98 [95-104] % predicted), echocardiographic screening was available in 88 patients and showed upper-normal sPAP values (29 [28-31] mmHg). One patient (1%) had a high probability of PH. Following clinical assessment and guideline-recommended non-invasive screening, RHC was performed in 13 patients with suspected PH. Hemodynamic measurements showed an mPAP of 19 (16-21) mmHg and a PVR of 1.9 (1.7-2.8) Wood units; PH was confirmed in 5 patients (38% of those undergoing RHC, 5% of the total cohort). All five patients had a plausible alternative explanation for their PH. Conclusion PH is a frequent complication in patients with HP and severe ILD. In HP patients with mild disease, PH is uncommon and alternative causes should be considered.

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

BACKGROUND:Solid organ transplant (SOT) recipients face elevated tuberculosis risk, yet optimal prevention strategies in low- to medium-incidence regions remain unclear. METHODS:We conducted a multicenter retrospective cohort study of adult SOT recipients transplanted between 2007 and 2012 at 15 European centers, with follow-up through 2018. The primary outcome was microbiologically confirmed post-transplant tuberculosis. Incidence rates were calculated per 100,000 person-years; standardized incidence ratios (SIRs) used World Health Organization country-specific background rates. Cox models assessed risk factors. RESULTS:Among 5805 patients (median age 51; 62.7% male; 73.9% renal transplants), 33.8% were tested for tuberculosis infection and 10.3% received tuberculosis preventive therapy (TPT). Over 33,785 person-years, 23 patients (0.4%) developed tuberculosis (68.0/100,000 person-years). Highest incidence occurred in patients with positive screening but no TPT (233.8/100,000). Incidence was higher in Southern vs. Central Europe (251.9 vs. 28.7/100,000), with pooled SIRs of 12.8 and 3.1, respectively. Tuberculosis risk was elevated among Southern European recipients (HR 22.9) and those with migration history (HR 2.7). CONCLUSION:Tuberculosis risk is increased in European SOT recipients. Regionally adapted prevention strategies, including targeted screening in low-incidence areas and universal screening in higher-incidence regions, are warranted.

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.

ZusammenfassungDie Bronchiektasen-Erkrankung ist eine ätiologisch heterogene, chronische und oftmals progredient verlaufende Atemwegs- und Lungenerkrankung, die durch eine irreversible Erweiterung der Bronchien gekennzeichnet ist. Sie geht häufig mit einer erheblichen Symptomlast, multiplen Komplikationen sowie einer eingeschränkten Lebensqualität einher. Seit mehreren Jahren ist weltweit eine deutliche Zunahme der Prävalenz der Bronchiektasen-Erkrankung mit einer relevanten ökonomischen Belastung der Gesundheitssysteme zu beobachten. Die vorliegende konsensusbasierte Leitlinie ist die erste deutschsprachige Leitlinie, die das Management der Bronchiektasen-Erkrankung bei Erwachsenen behandelt. Die Leitlinie betont die Wichtigkeit der thorakalen Bildgebung mittels CT zur Diagnose und Differenzierung der Bronchiektasen sowie die Bedeutung der Ätiologie zur Festlegung der Therapieansätze. Es werden sowohl nicht-medikamentöse als auch medikamentöse Therapien ausführlich erörtert. Zu den nicht-medikamentösen Maßnahmen gehören Raucherentwöhnung, Physiotherapie, körperliches Training, Rehabilitation, nichtinvasive Beatmung, Thoraxchirurgie und Lungentransplantation. Bei den medikamentösen Therapien wird besonders auf die langfristige Anwendung von Mukolytika, Bronchodilatatoren, antiinflammatorischen Medikamenten und Antibiotika eingegangen. Darüber hinaus geht die Leitlinie auf die Herausforderungen und Strategien bei der Behandlung einer oberen Atemwegsbeteiligung, von Komorbiditäten und Exazerbationen sowie die sozialmedizinischen Aspekte und das Schwerbehindertenrecht ein. Zudem wird die Bedeutung der Patientenaufklärung und des Selbstmanagements hervorgehoben. Abschließend werden spezielle Lebensphasen wie Transition, Kinderwunsch, Schwangerschaft und Elternschaft sowie Palliativmedizin behandelt. Die Leitlinie zielt darauf ab, eine umfassende, konsensusbasierte und patientenzentrierte Versorgung zu gewährleisten, wobei individuelle Risiken und Bedürfnisse berücksichtigt werden.

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.

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.

Die systemische Sklerose (SSc) ist eine schwerwiegende autoimmune Bindegewebserkrankung, die jedes Organ betreffen kann. Die Haupttodesursache der SSc ist jedoch bedingt durch pulmonale Komplikationen, die sich entweder als interstitielle (ILD) oder als vaskuläre Lungenerkrankung (pulmonale Hypertonie, PH) manifestieren können. Die Diagnostik ist mehrstufig und erfolgt in der Regel über funktionelle und bildmorphologische Diagnostik. Die definitive Abklärung sowie Behandlung beider Komplikationen sollten im Expertenzentrum erfolgen.

Bronchiectasis is an etiologically heterogeneous, chronic, and often progressive respiratory disease characterized by irreversible bronchial dilation. It is frequently associated with significant symptom burden, multiple complications, and reduced quality of life. For several years, there has been a marked global increase in the prevalence of bronchiectasis, which is linked to a substantial economic burden on healthcare systems. This consensus-based guideline is the first German-language guideline addressing the management of bronchiectasis in adults. The guideline emphasizes the importance of thoracic imaging using CT for diagnosis and differentiation of bronchiectasis and highlights the significance of etiology in determining treatment approaches. Both non-drug and drug treatments are comprehensively covered. Non-pharmacological measures include smoking cessation, physiotherapy, physical training, rehabilitation, non-invasive ventilation, thoracic surgery, and lung transplantation. Pharmacological treatments focus on the long-term use of mucolytics, bronchodilators, anti-inflammatory medications, and antibiotics. Additionally, the guideline covers the challenges and strategies for managing upper airway involvement, comorbidities, and exacerbations, as well as socio-medical aspects and disability rights. The importance of patient education and self-management is also emphasized. Finally, the guideline addresses special life stages such as transition, family planning, pregnancy and parenthood, and palliative care. The aim is to ensure comprehensive, consensus-based, and patient-centered care, taking into account individual risks and needs.

Background: Systemic sclerosis (SSc) is a multiorgan disease, that frequently affects the skin and involves the lung in up to 70 percent of cases with a 10 years mortality rate in SSc up to 50 percent. B cell-depleting therapy with rituximab (RTX) seems to be effective in the treatment of SSc, but data from randomized controlled trials (RCTs) are missing and there is no consensus on frequency and dosage of RTX in SSc. [1-3] Objectives: We aimed to assess the long-term efficacy and safety of quarterly administered RTX in SSc. Methods: Retrospective analyses of 40 consecutive systemic sclerosis patients suffering from interstitial lung disease and progredient skin involvement treated with rituximab twice within 14 days every three months from 2010-2020. Of RTX-treated patients 42.5 % were treated with RTX monotherapy and 47.5 with RTX in combination with other immunosuppressants. The patients' fulfilled the LeRoy and the ACR/EULAR Criteria for SSc. (4) Modified Rodnan skin score (mRSS), lung function test results, laboratory values including serum immunoglobulin (IgG, IgA, IgM) concentrations.[5] The clinical parameters are included in the EScSGAI and have been documented over time.[6] (Figure 1) Results: In total 40 SSc patients received RTX as described above over a median time of 3.9 years (1-10 years). A significant improvement in median mRSS (baseline: 19, month 24: 16, p<0.001), as well as a stable predicted forced vital capacity (FVC) were observed from baseline to month 24. There were no new or unexpected safety signals especially regarding treatment-related infectious adverse events. Immunoglobuline concentrations stayed within the normal range and specific antibodies to pneumococcal polysaccharides were preserved despite of long term B cell depleting therapy. During the observation period of up to ten years none of the patients died. (A) European Scleroderma Study Group Activity Index, (B) modified Rodnan Skin Score (mRss), (C) forced vital capacity (FVC) in percent predicted, and (D) diffusing lung capacity of carbonmonoxide (DLCO) in percent during RTX treatment. The number of patients treated is indicated by n, the duration of treatment on the x-axis by months. Median + 95%CI Significance was calculated using paired Wilcoxon Test. Conclusion: Conclusion: SSc can be effectively and safely treated by 3-monthly, low-dose RTX. Controlled, randomized studies are needed to validate the advantage of continuous B cell depletion by 3-monthly low dose RTX application compared to other application intervals. REFERENCES: [1] Katsumoto TR, Whitfield ML, Connolly MK. The pathogenesis of systemic sclerosis. Annu Rev Pathol. 2011 Feb 28;6:509–37. [2] Moazedi-Fuerst FC, Kielhauser SM, Brickmann K, Hermann J, Lutfi A, Meilinger M, et al. Rituximab for systemic sclerosis: arrest of pulmonary disease progression in five cases. Results of a lower dosage and shorter interval regimen. Scand J Rheumatol [Internet]. 2014 Jan [cited 2015 Feb 25];43(3):2. [3] Maher TM, Tudor VA, Saunders P, Gibbons MA, Fletcher S V, Denton CP, et al. Rituximab versus intravenous cyclophosphamide in patients with connective tissue disease-associated interstitial lung disease in the UK (RECITAL): a double-blind, double-dummy, randomised, controlled, phase 2b trial. Lancet Respir Med. 2022 Jan; [4] van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum [Internet]. 2013 Nov [cited 2016 Mar 23];65(11):2737–47. [5] Venhoff N, Effelsberg NM, Salzer U, Warnatz K, Peter HH, Lebrecht D, et al. Impact of rituximab on immunoglobulin concentrations and B cell numbers after cyclophosphamide treatment in patients with ANCA-associated vasculitides. PLoS One. 2012 May 21;7(5). [6] Valentini G, Bencivelli W, Bombardieri S, D'Angelo S, Della Rossa A, Silman AJ, et al. European Scleroderma Study Group to define disease activity criteria for systemic sclerosis. III. Assessment of the construct validity of the preliminary activity criteria. Ann Rheum Dis [Internet]. 2003 Sep [cited 2018 Oct 10];62(9):901–3. Acknowledgements: NIL. Disclosure of Interests: None declared.

Abstract Background Chronic pulmonary aspergillosis (CPA) can complicate underlying pulmonary diseases, and clinical management of CPA is challenging. Guidelines support clinicians but due to the complexity of the disease they can be difficult to adhere to. Objectives To map current guideline recommendations for the clinical management of CPA into a scoring tool to facilitate and quantify guideline adherence in clinical practice. Methods Recommendations for diagnosis, treatment and follow-up of CPA presented in the current ESCMID/ERS/ECMM and CPAnet guidance documents were assembled and weighed on the basis of their strength of recommendation and level of evidence. Results Twenty-seven recommendations were identified, resulting in a total maximum EQUAL CPA Score of 51. For diagnostics (ScoreMax = 27), a strong emphasis on expert consultation, culture, direct microscopy, histopathology, serology and imaging was reflected in respective points, whereas molecular techniques and susceptibility testing count into the diagnostics score to a lesser extent. Ten treatment recommendations (ScoreMax = 14), including antifungal therapy, therapeutic drug monitoring and treatment duration, were identified. Surgery, where indicated, adds three points. For refractory disease or intolerance of first-line antifungal treatment, optimal second-line treatment added another two points. During follow-up (ScoreMax = 10), response assessment via imaging gave three points, while culture and serology added two points each to the ScoreMax. Conclusion The EQUAL CPA Score intents to be used as a comprehensive tool for measuring guideline adherence. If adherence to current guidelines is associated with clinical outcome, this will be assessed in future studies.

"Reply to: Targeting Pulmonary Hypertension Caused by Pulmonary Fibrosis: A Promising NKT Cell-based Therapy." American Journal of Respiratory and Critical Care Medicine, 0(ja), pp. –

Zusammenfassung Die Lungenbeteiligung ist die häufigste Todesursache bei Patienten mit systemischer Sklerose (SSc). Da eine Lungenbeteiligung häufig asymptomatisch ist, wird derzeit empfohlen, bei allen Patienten mit einer neu diagnostizierten SSc eine Thorax-CT durchzuführen. Uneinigkeit herrscht derzeit darüber, wie SSc-Patienten, bei denen zum Diagnosezeitpunkt keine Lungenbeteiligung gefunden wurde, weiterverfolgt werden sollen. Basierend auf einem Konsensus österreichischer Rheumatologen, Pneumologen und Radiologen, wird empfohlen, bei asymptomatischen PatientInnen mit einer negativen CT zum Zeitpunkt der Erstdiagnose jährlich transthorakale Ultraschalluntersuchungen sowie Lungenfunktionsuntersuchungen alle 6 bis 12 Monate durchzuführen. Bei Vorliegen eines positiven Lungenultraschallbefundes wird eine ergänzende CT zur weiterführenden Abklärung empfohlen. Aufgrund der Datenlage werden bei PatientInnen mit einem höheren Risiko, definiert durch entsprechende Risikofaktoren, jährliche CT-Verlaufskontrollen empfohlen.

Background Although the number of lung transplantations (LTx) performed worldwide for coronavirus disease 2019 (COVID-19)-induced acute respiratory distress syndrome (ARDS) is still low, there is general agreement that this treatment can save a subgroup of the most severely ill patients with irreversible lung damage. However, the true proportion of patients eligible for LTx, the overall outcome and the impact of LTx on the pandemic are unknown. Methods A retrospective analysis was performed using a nationwide registry of hospitalised patients with confirmed severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) infection admitted between 1 January 2020 and 30 May 2021 in Austria. Patients referred to one of the two Austrian LTx centres were analysed, and grouped into patients accepted and rejected for LTx. Detailed outcome analysis was performed for all patients who received a LTx for post-COVID-19 ARDS and compared with patients who underwent LTx for other indications. Results Between 1 January 2020 and 30 May 2021, 39 485 patients were hospitalised for COVID-19 in Austria. 2323 required mechanical ventilation and 183 received extracorporeal membrane oxygenation (ECMO) support. 106 patients with severe COVID-19 ARDS were referred for LTx. Of these, 19 (18%) underwent LTx. 30-day mortality after LTx was 0% for COVID-19 ARDS transplant recipients. At a median follow-up of 134 (47–450) days, 14 out of 19 patients were alive. Conclusions Early referral of ECMO patients to a LTx centre is pivotal in order to select patients eligible for LTx. Transplantation offers excellent midterm outcomes and should be incorporated in the treatment algorithm of post-COVID-19 ARDS.

The Austrian Society of Pneumology (ASP) launched a first statement on severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in May 2020, at a time when in Austria 285 people had died from this disease and vaccinations were not available. Lockdown and social distancing were the only available measures to prevent more infections and the breakdown of the health system. Meanwhile, in Austria over 13,000 patients have died in association with a SARS-CoV‑2 infection and coronavirus disease 2019 (COVID-19) was among the most common causes of death; however, SARS-CoV‑2 has been mutating all the time and currently, most patients have been affected by the delta variant where the vaccination is very effective but the omicron variant is rapidly rising and becoming predominant. Particularly in children and young adults, where the vaccination rate is low, the omicron variant is expected to spread very fast. This poses a particular threat to unvaccinated people who are at elevated risk of severe COVID-19 disease but also to people with an active vaccination. There are few publications that comprehensively addressed the special issues with SARS-CoV‑2 infection in patients with chronic lung diseases. These were the reasons for this updated statement. Pulmonologists care for many patients with an elevated risk of death in case of COVID-19 but also for patients that might be at an elevated risk of vaccination reactions or vaccination failure. In addition, lung function tests, bronchoscopy, respiratory physiotherapy and training therapy may put both patients and health professionals at an increased risk of infection. The working circles of the ASP have provided statements concerning these risks and how to avoid risks for the patients.