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.

BackgroundChronic rhinosinusitis (CRS) contributes to morbidity in cystic fibrosis (CF), and sinus surgery serves as second-line treatment. Magnetic resonance imaging (MRI) was recently shown to differentiate CF-related CRS (CF-CRS) manifestations, and to monitor therapy response, but has not been used to investigate the effects of sinus surgery on CF-CRS.ObjectiveThe aim of this study was to systematically study the effects of sinus surgery on CF-CRS.MethodsTwenty controls with CF (median age 15 years, range 9-33 years) who had not undergone sinus surgery with annual MRI examinations were age-matched to the surgery group. The surgery group comprised 10 individuals with CF (median age 15 years, range 8-32 years) who underwent endoscopic sinus surgery between 2010 and 2018 and underwent MRI in median 2.5 months before (MRI1) and at least one (MRI2) or two (MRI3) annual MRIs after surgery. The median time difference between sinus surgery and MRI2 was 14 months, and between MRI2 and MRI3 it was 13 months. All patients were modulator-naïve. The established CRS-MRI score was used including sinus dimension measurement.ResultsIn controls, the median maxillary sinus width was stable from MRI1 through MRI3 (range 23.0-24.5 mm; P > .999). In the surgery group, the median maxillary sinus width decreased from MRI1 to MRI2 (-5.5 mm, P < .01), and remained stable from MRI2 to MRI3 (+3.0 mm; P = .544). The prevalence of maxillary sinus deformation decreased from MRI1 to MRI2 (-35%; P < .05) and was stable from MRI2 to MRI3 (+19%; P = .295). The CRS-MRI sum score was stable from MRI1 through MRI3 in controls (median 28, 23 and 34 at MRI1-2-3), and in the surgery group (36, 35 and 39, respectively) (P = .743-.999).ConclusionSinus surgery improves maxillary sinus width and deformation. The CRS-MRI score could not detect further benefits of surgery on CF-CRS. MRI supports the evaluation of sinus surgery in the era of modulator treatment strategies as some patients still suffer from CF-CRS despite optimized modulator treatment and there is a need to identify patients that still might profit from sinus surgery.

BACKGROUND:MRI revealed a high prevalence of lung abnormalities in children with primary ciliary dyskinesia (PCD). However, longitudinal imaging data on the onset and progression of PCD are lacking. RESEARCH QUESTION:When do abnormalities in lung morphologic features and perfusion in patients with PCD first emerge, and how do they progress longitudinally from infancy through adulthood as assessed by MRI? STUDY DESIGN AND METHODS:One hundred eighty-nine MRI examinations (mean [SD] examinations per patient, 2.4 [1.7] examinations; range, 1-10 examinations) from 75 patients with PCD (mean [SD] age, 17.7 [15.0] years; range, 0-65 years) were included. MRI was assessed using the chest MRI scoring system by 2 independent readers. Spirometry was performed at the time of MRI, and the Bronchiectasis Severity Index (BSI) was calculated in adult patients. RESULTS:Bronchiectasis or wall thickening, mucus plugging, consolidation, and perfusion abnormalities were highly prevalent at infancy (100%, 80%, 60%, and 50%, respectively). Prevalence of mucus plugging, consolidation, and perfusion abnormalities increased at adulthood (97%, 80%, and 97%, respectively; P < .05). The mean (SD) MRI global score was 14.5 (4.5) at infancy, showed stability from preschool age through school age and adolescence (18.4 [6.7], 19.8 [8.2], and 18.5 [9.6]; P = .386-.529, respectively), and an elevation in adulthood (26.7 [7.2]; P < .001). MRI morphologic features, perfusion, and global score correlated weakly with age (r = 0.20-0.38; P < .05) and showed moderate to strong correlations with FEV1 % predicted (r = -0.44 to -0.71; P < .001) and BSI (r = 0.44-0.49; P < .001). INTERPRETATION:Our results show an early onset of lung disease in PCD already in infancy and a progressive increase in the prevalence and extent of changes in lung morphologic features and perfusion at adulthood. These results support the use of MRI as an end point in clinical trials in patients with PCD.

RATIONALE:A cross-sectional study employing magnetic resonance imaging (MRI) of paranasal sinuses recently showed a high prevalence and extent of chronic rhinosinusitis (CRS) in children with primary ciliary dyskinesia (PCD). However, longitudinal imaging data on CRS onset and progression are lacking. OBJECTIVE:To longitudinally evaluate CRS in PCD from infancy through adolescence with MRI. METHODS:22 children with PCD (median baseline age 8.5 year, range 0.0-18.0 year) underwent a median of 3 (range 1-9) annual standardized paranasal sinus MRI examinations. MRI were assessed by 2 independent readers using the previously evaluated CRS-MRI score, including assessment of the prevalence and dominance (defined as the most prominent abnormality within a sinus) of each abnormality. RESULTS:At infancy (0 year), 83%-100% of paranasal sinuses and mastoid cells were opacified. Mucosal swelling was the most prevalent (83%-100%) and in most sinuses the dominant abnormality (50%-100%). In maxillary sinuses, also polyps (75%) and mucopyoceles (13%) were prevalent. At preschool age (1-5 year), the prevalence of opacified sphenoid sinus increased (83% vs. 100%, P < .01), while the prevalence of opacification was stable for the other sinuses as well as for mastoid cells (P = .567-0.999 vs. infancy). Mucosal swelling remained the most prevalent (100%) and dominant abnormality (47%-100%). In maxillary sinuses, the prevalence and subscore of mucopyoceles increased (81% vs. 13% and 2.0 ± 1.6 vs. 4.1 ± 2.1, respectively; P < .05), while polyps were stable (70% vs. 75%, P > .999 vs. infancy). At school age (≥6 year), almost all maxillary, sphenoid and ethmoid sinuses (91%-100%), and 87% of mastoid cells were opacified (P = .122-.999 vs. preschool age). Especially in maxillary sinuses, the prevalence of mucopyoceles, polyps and sinus deformation decreased (60% vs. 81%, 40% vs. 70%, and 51% vs. 78%, respectively; P < .05 vs. preschool age). The CRS-MRI sum score averaged 27.8 ± 4.9 at infancy, was stable at preschool age (31.9 ± 5.6, P > .999) and decreased at school age to 25.6 ± 8.3 (P < .01). CONCLUSIONS:Longitudinal paranasal sinus MRI detects high prevalence and extent of paranasal sinuses abnormalities from infancy. Our data support its role for comprehensive non-invasive monitoring of CRS in children with PCD.

MRI detects abnormal lung perfusion in patients with cystic fibrosis (CF). However, little is known about the contribution of bronchial arteries to lung perfusion in CF. We hypothesized that delayed perfusion can be detected by dynamic contrast-enhanced (DCE-)MRI and that bronchial artery dilatation (BAD) is associated with changes in lung perfusion. Morpho-functional MRI was prospectively acquired in 75 patients with CF (18.7 ± 7.6 years, range 6–39 years). Lungs and perfusion defects were segmented automatically to quantify perfusion defects in percent (QDP). Pulmonary blood flow (PBF), mean transit time (MTT), and perfusion delay were calculated for the whole lung, inside normally perfused and perfusion defect areas. Chest MRI score and BAD were assessed visually. QDP and PBF correlated with MRI global score (r = 0.58 and −0.53, p < 0.001). In normally perfused lung, PBF was higher (161.2 ± 77.9 mL/100 mL/min vs. 57.5 ± 26.4 mL/100 mL/min, p < 0.001), and MTT (5.4 ± 1.7 s vs. 6.9 ± 2.3 s, p < 0.001) and perfusion delay were shorter than in perfusion defect areas (4.6 ± 5.3 s vs. 13.4 ± 16.2 s, p < 0.001). 48 (64.0

This multicenter trial was conducted to evaluate MRI for the longitudinal management of incidental pulmonary nodules in heavy smokers. 239 participants (63.9 ± 8.4 years, 43–82 years) at risk of or with COPD GOLDI-IV from 16 centers prospectively underwent two rounds of same-day low-dose computed tomography (LDCT1 2) and MRI1 2 at an interval of three years in the nationwide COSYCONET trial. All exams were independently assessed for incidental pulmonary nodules in a standardized fashion by two blinded readers, incl. axis measurements and Lung-RADS categorization, with consensual LDCT results serving as the standard of reference. A change in diameter ≥ 2 mm was rated as progress. 11 patients underwent surgery for suspicious nodules after the first round. Two hundred twenty-four of two hundred forty nodules (93.3

Rationale: The progression of lung changes in cystic fibrosis (CF) from infancy through adolescence remains poorly understood as a result of limited longitudinal imaging data. Objectives: To assess changes in lung morphology and perfusion in children with CF through the pediatric age range by longitudinal chest magnetic resonance imaging (MRI). Methods: 1,112 annual chest MRI scans were performed in 226 patients with CF aged 0-18 years. MRI was assessed using a validated MRI scoring system. Results: The MRI global score continuously increased from 5.5 ± 4.6 at infancy (0 yr) to 17.9 ± 8.5 at adolescence (12-18 yr), and the MRI morphology score increased from 5.0 ± 3.9 to 12.3 ± 6.1 (P < 0.001). Bronchiectasis/wall thickening prevalence increased from 89.1% at infancy to approximately 100% at preschool age (1-5 yr), and the subscore increased from 3.1 ± 1.9 at infancy to 6.6 ± 2.1 at adolescence (P < 0.001). Mucus plugging prevalence increased from 55.4% at infancy to 83.0% at adolescence, and the subscore increased from 1.2 ± 1.6 to 3.7 ± 2.6 in the same period (P < 0.001). Perfusion abnormalities were found in 44.4% at infancy, and increased to approximately 90% at preschool age (P < 0.001). The MRI perfusion score increased from 1.1 ± 1.6 at infancy to 5.6 ± 3.0 at adolescence (P < 0.001). Chronic Pseudomonas aeruginosa infection was associated with higher MRI scores at school age (6-11 yr; P < 0.05-0.001). Conclusions: This is the first study to assess longitudinal changes in lung morphology and perfusion in CF throughout the pediatric age range, providing percentiles as age-specific references for lung disease severity. Our data may facilitate the use of MRI as an endpoint in clinical trials in children with CF. Clinical trial registered with www.clinicaltrials.gov (NCT00760071 and NCT02270476).

There are well-documented differences in idiopathic pulmonary fibrosis (IPF) between sexes. The sex-specific prevalence of interstitial lung disease (ILD) subtypes in patients who require a full diagnostic work-up, including transbronchial cryobiopsy (TCB), after initial multidisciplinary discussion (MDD) is still unknown. Retrospective analysis of sex dispareties in patients with ILD who received an interdisciplinary indication for lung biopsy and underwent bronchoalveolar lavage, TCB and, if necessary, surgical lung biopsy at our ILD centre in Heidelberg between 11/17 and 12/21. The analysis included clinical parameters, visual assessment of computed tomography (CT), automated histogram analyses of lung density by validated software and final MDD-ILD classifications. A total of 402 patients (248 men, 154 women; mean age 68 ± 12 years) were analysed. Smoking behaviour was similar between the sexes, but women were more exposed to environmental factors, whereas men were more exposed to occupational factors. Women had higher rates of thyroid disease (29.9% vs. 12.5%; p < 0.001) and extrathoracic malignancies (16.2% vs. 9.3%; p = 0.041), but lower rates of coronary heart disease (7.1% vs. 19.8%; p < 0.001), stroke (1.3% vs. 6.5%; p = 0.014) and sleep apnoea (5.8% vs. 17.7%; p < 0.001). There were no sex differences regarding CT lung density. On visual inspection, women were less likely to have reticular opacities (65% vs. 76%; p = 0.017) and features of usual interstitial pneumonia (17% vs. 34%; p < 0.001). Among final diagnoses, hypersensitivity pneumonitis was more common in women (34.4%) compared to men (21.8%; p = 0.007). In contrast, IPF was more common in men (22.6%) than in women (7.1%; p < 0.001), and unclassifiable interstitial lung disease was also more frequent in men (21.8%) compared to women (6.5%; p < 0.001). This study highlights significant sex-based differences in the prevalence and characteristics of ILD requiring comprehensive diagnostic work-up. These findings underscore the importance of considering sex-specific factors in the diagnosis and management of ILD.

Zystische und noduläre Lungenkrankheiten umfassen ein breites Spektrum von Erkrankungen mit unterschiedlichsten Ätiologien und klinisch-radiologischem Erscheinungsbild. Dabei ist ihre Unterscheidung für das Patientenmanagement von entscheidender Bedeutung, kann jedoch aufgrund von Krankheiten, die Merkmale beider Kategorien und überlappende radiologische Muster aufweisen, komplex sein. Detaillierte Beschreibung bildgebender Merkmale von zystischen und nodulären Lungenkrankheiten in der hochauflösenden Computertomographie (CT), vorrangig anhand ihrer Ätiologie, um eine präzisere Differenzialdiagnose dieser Erkrankungen zu ermöglichen. Narratives Review anhand aktueller Literatur zu dem Thema aus klinisch-radiologischer Sicht. Diese Arbeit kategorisiert systematisch die Differenzialdiagnosen zystischer und nodulärer Lungenerkrankungen und bietet Einblicke in ihre radiologischen Muster und Ätiologien. Sie unterstreicht hierbei die Bedeutung der CT in der Diagnostik dieser Erkrankungen und zeigt die Bedeutung von multidisziplinären Boards, die Fachwissen aus der Radiologie, Pneumologie, Rheumatologie und Pathologie vereinen. Die sichere Differenzialdiagnose zystischer und nodulärer Lungenkrankheiten, insbesondere allein anhand ihrer radiologischen Merkmale, bleibt aufgrund ihrer Überlappungen und dynamischen Natur schwierig. Multidisziplinäre Boards sollten klinischer Standard zur präzisen differenzialdiagnostischen Aufarbeitung dieser Erkrankungen sein, da sie die Anamnese, die Symptomatik, die radiologischen Befunde und, sofern nötig, die histopathologischen Untersuchungen vereinen und somit einen robusteren Rahmen für Diagnose und Management bieten.

Background/Objectives: Contrast-enhanced computed tomography (CT) is the standard radiologic examination for evaluating the extent of mediastinal tumors. If tumor infiltration into the large central thoracic vessels, the pericardium, or the myocardium is suspected, cine magnetic resonance imaging (cine-MRI) can provide additional valuable information. Methods: We conducted a retrospective study of patients with mediastinal tumors who were staged with CT, cine-MRI, and a T1-weighted turbo spin echo (T1TSE) prior to surgical resection. Imaging was re-evaluated regarding tumor infiltration into the pericardium, myocardium, superior vena cava, aorta, pulmonary arteries, and atria and compared with intraoperative findings and postoperative histopathological reports (gold standard). Unclear CT findings were further investigated. Results: Forty-seven patients (29 female and 18 male patients; median age: 58 years) met the inclusion criteria. Cine-MRI was able to predict infiltration of the aorta in 86%, pulmonary arteries in 85%, and atria in 80% of unclear CT cases. Aortic tumor infiltration in unclear CT cases was significantly more often correctly diagnosed with cine-MRI than with T1TSE sequence. Conclusions: Additional cine-MRI is of crucial benefit in unclear CT cases. We recommend performing cine-MRI if infiltration into the large central vessels and atria is suspected. T1TSE sequence is of very limited additional value.

Rationale: Clinical trials show that lumacaftor/ivacaftor (LUM/IVA) treatment has the potential to modify early cystic fibrosis (CF) disease progression in children as young as 2 years of age. Objectives: To assess the long-term impact of LUM/IVA treatment on CF disease progression in children aged 2-5 years. Methods: This phase 2 trial had two parts: part 1, a 48-week, randomized, double-blind, placebo-controlled study of LUM/IVA in children aged 2-5 years (previously reported) was followed by a 48-week open-label treatment period in which all children received LUM/IVA (part 2; reported here). Endpoints assessed in part 2 included absolute changes from baseline in chest magnetic resonance imaging (MRI) global score at Week 96; weight-for-age, stature-for-age, and body mass index (BMI)-for-age z-scores at Week 96; lung clearance index based on lung volume turnover required to reach 2.5% of starting N2 concentration (LCI2.5) through Week 96; chest MRI morphological score, chest MRI perfusion score, weight, stature, BMI, and microbiology cultures (oropharyngeal swabs) at Week 96; sweat chloride, amount of immunoreactive trypsinogen, fecal elastase-1 concentration, and fecal calprotectin through Week 96; and number of pulmonary exacerbations, time to first pulmonary exacerbation, and number of CF-related hospitalizations. Results: Forty-nine children received one or more doses of LUM/IVA in the open-label period (33 in the LUM/IVA to LUM/IVA group and 16 in the placebo to LUM/IVA group), with a mean exposure of 47.1 (standard deviation [SD], 5.2) weeks. The mean absolute change in MRI global score (negative value indicates improvement) from baseline at Week 96 was -2.7 (SD, 7.0; 95% confidence interval [CI], -5.2 to -0.1) in the LUM/IVA to LUM/IVA group and -5.6 (SD, 6.9; 95% CI, -9.2 to -1.9) in the placebo to LUM/IVA group. Improvements in LCI2.5, sweat chloride concentration, and markers of pancreatic function and intestinal inflammation were also observed in both groups. Growth parameters remained stable in both groups. The majority of children had adverse events considered mild (38.8%) or moderate (40.8%). Two (4.1%) children discontinued LUM/IVA treatment because of adverse events (distal intestinal obstruction syndrome [n = 1] and alanine aminotransferase increase [n = 1]). Conclusions: These findings confirm the potential for early LUM/IVA treatment to alter the trajectory of CF disease progression, including CF lung disease, in children as young as 2 years of age. Clinical trial registered with ClinicalTrials.gov (NCT03625466).

Hintergrund und Zielsetzung: Kinder mit chronischen Erkrankungen nehmen oftmals Leistungen unterschiedlicher Sektoren in Anspruch (z.B. stationäre und ambulante Versorgung, Jugend- und Sozialämter). Eine unzureichende Koordination und eingeschränkte Informationsflüsse zwischen Sektoren können zu vermeidbaren Belastungen für Familien und Leistungserbringer führen. Von den Reibungsverlusten an Sektorengrenzen sind insbesondere Familien mit eingeschränkter navigationaler Gesundheitskompetenz und Kindern mit komplexen Versorgungsbedarfen betroffen. Durch die Umsetzung eines kommunalen Versorgungsnetzwerks mit Einsatz von Familienlots:innen (Gesundheits- und Kinderkrankenpfleger:innen, Sozialarbeiter:innen) können Familien auf Basis bestehender Angebotsstrukturen begleitet und unterstützt werden. Zur Stärkung der sektorenübergreifenden Versorgung soll in diesem Projekt ein kommunales Versorgungsnetzwerk mit Familienlots:innen konzeptualisiert und umgesetzt werden. Begleitend werden Bedarfe der Familien sowie mögliche Effekte des Versorgungsnetzwerks identifiziert, die in die weitere Ausgestaltung des Netzwerks einfließen sollen.

INTRODUCTION:Histological confirmation of a lung tumor is the prerequisite for treatment planning. It has been suspected that CT-guided needle biopsy (CTGNB) exposes the patient to a higher risk of pleural recurrence. However, the distance between tumor and pleura has largely been neglected as a possible confounder when comparing CTGNB to bronchoscopy. METHODS:All patients with lung cancer histologically confirmed by bronchoscopy or CTGNB between 2010 and 2020 were enrolled and studied. Patients' medical histories, radiologic and pathologic findings and surgical records were reviewed. Pleural recurrence was diagnosed by pleural biopsy, fluid cytology, or by CT chest imaging showing progressive pleural nodules. RESULTS:In this retrospective unicenter analysis, 844 patients underwent curative resection for early-stage lung cancer between 2010 and 2020. Median follow-up was 47.5 months (3-137). 27 patients (3.2 %) with ipsilateral pleural recurrence (IPR) were identified. The distance of the tumor to the pleura was significantly smaller in patients who underwent CTGNB. A tendency of increased risk of IPR was observed in tumors located in the lower lobe (HR: 2.18 [±0.43], p = 0.068), but only microscopic pleural invasion was a significant independent predictive factor for increased risk of IPR (HR: 5.33 [± 0.51], p = 0.001) by multivariate cox analysis. Biopsy by CTGNB did not affect IPR (HR: 1.298 [± 0.39], p = 0.504). CONCLUSION:CTGNB is safe and not associated with an increased incidence of IPR in our cohort of patients. This observation remains to be validated in a larger multicenter patient cohort.

Rationale: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are characterized by inherited impaired mucociliary clearance leading to chronic progressive lung disease as well as chronic rhinosinusitis (CRS). The diseases share morphological and functional commonalities on magnetic resonance imaging (MRI) of the lungs and paranasal sinuses, but comparative MRI studies are lacking. Objectives: To determine whether PCD shows different associations of pulmonary and paranasal sinus abnormalities on MRI and lung function test results in children (infants to adolescents) compared with children with CF. Methods: Eighteen children with PCD (median age, 9.5 [IQR, 3.4-12.7] yr; range, 0-18 yr) and 36 age-matched CF transmembrane conductance regulator modulator-naive children with CF (median age, 9.4 [3.4-13.2] yr; range, 0-18 yr) underwent same-session chest and paranasal sinus MRI as well as spirometry (to determine forced expiratory volume in 1 s percent predicted) and multiple-breath washout (to determine lung clearance index z-score). Pulmonary and paranasal sinus abnormalities were assessed using previously validated chest MRI and CRS-MRI scoring systems. Results: Mean chest MRI global score was similar in children with PCD and CF (15.0 [13.5-20.8] vs. 15.0 [9.0-15.0]; P = 0.601). Consolidations were more prevalent and severe in children with PCD (56% vs. 25% and 1.0 [0.0-2.8] vs. 0.0 [0.0-0.3], respectively; P < 0.05). The chest MRI global score correlated moderately with forced expiratory volume in 1 second percent predicted in children with PCD and children with CF (r = -0.523 and -0.687; P < 0.01) and with lung clearance index in children with CF (r = 0.650; P < 0.001) but not in PCD (r = 0.353; P = 0.196). CRS-MRI sum score and mucopyocele subscore were lower in children with PCD than in children with CF (27.5 [26.3-32.0] vs. 37.0 [37.8-40.0] and 2.0 [0.0-2.0] vs. 7.5 [4.8-9.0], respectively; P < 0.01). CRS-MRI sum score did not correlate with chest MRI score in PCD (r = 0.075-0.157; P = 0.557-0.788) but correlated moderately with MRI morphology score in CF (r = 0.437; P < 0.01). Conclusions: MRI detects differences in lung and paranasal sinus abnormalities between children with PCD and those with CF. Lung disease does not correlate with CRS in PCD but correlates in CF.

INTRODUCTION:Previous studies using magnetic resonance imaging (MRI) demonstrated early onset and progression of chronic rhinosinusitis (CRS) from infancy to school age, and response to lumacaftor/ivacaftor (LUM/IVA) therapy in children with cystic fibrosis (CF). However, the effect of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on CRS detected by MRI in children with CF and at least one F508del mutation, and potential incremental effects of ELX/TEZ/IVA compared to LUM/IVA in F508del homozygous children have not been studied. METHODS:30 children with CF with at least one F508del mutation underwent three longitudinal paranasal sinus MRI before (MRI1), without (n = 16) or with LUM/IVA therapy (n = 14, MRI2), and with ELX/TEZ/IVA therapy (MRI3, mean age at therapy initiation 11.1 ± 3.4y, range 6-16y). MRI were evaluated using the CRS-MRI score. RESULTS:After therapy initiation with ELX/TEZ/IVA, the prevalence and in maxillary and sphenoid sinuses the dominance of mucopyoceles decreased (35% vs. 0 %, p<0.001 and 26% vs. 8 %, p < 0.05, respectively). This leads to a reduction in mucopyocele subscore (-3.4 ± 1.9, p < 0.001), and sinus subscores in MRI3 (maxillary sinus: -5.3 ± 3.1, p < 0.001, frontal sinus: -1.0 ± 1.9, p < 0.01, sphenoid subscore: -2.8 ± 3.5, p < 0.001, ethmoid sinus: -1.7 ± 1.9, p < 0.001). The CRS-MRI sum score decreased after therapy initiation with ELX/TEZ/IVA by -9.6 ± 5.5 score points (p < 0.001). The strength in reduction of mucopyoceles subscore and CRS-MRI sum score was independent of a pretreatment with LUM/IVA from MRI1-MRI2 (p = 0.275-0.999). CONCLUSIONS:ELX/TEZ/IVA therapy leads to improvement of CRS in eligible children with CF. Our data support the role of MRI for comprehensive monitoring of CRS disease severity and response to therapy in children with CF.

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.

Einleitung Bei Patienten mit interstitieller Lungenerkrankung und fehlendem eindeutigen UIP-Muster in der CT sollte eine invasive Diagnostik zur Abklärung erwogen werden. In der vorliegenden Analyse wurden klinische Prädiktoren für das Vorliegen einer idiopathischen Lungenfibrose untersucht.