[Kids Lung Registry and Child-EU Project - Progress in Rare and Interstitial Lung Diseases in Childhood Through Collaboration]. / Kinderlungenregister (chILD-EU) ­ Zusammenarbeit führt zu Fortschritten bei den seltenen und interstitiellen Lungenerkrankungen.

BACKGROUND: Progress in rare and interstitial lung disease in childhood can most usefully be achieved through systematic, registry-based collection. QUESTION AND METHODS: What are the practicalities and benefits of participating in the pediatric lung registry/chILD-EU project? We report our clinical experiences. RESULTS: Pediatricians and pediatric pulmonologists identify children with rare lung diseases. These are reported to the Kid's Lung Register after parental consent. Clinical data, imaging, and blood are sent to the registry. Genetic analysis can be arranged if desired. With completeness of the data, a peer-review process by pediatric radiology, possibly lung pathology, clinical and possibly genetic experts takes place in an interdisciplinary conference. A working diagnosis is established and communicated to the responsible physician via the registry and, if necessary, further discussed in case-related discussions. Assistance in entering the data is provided by the registry. Follow-ups are performed annually, and all registered physicians are invited to regular, web-based case discussions. Significant questions are answered in scientific projects and jointly published (>110 publications to date). CONCLUSIONS: Due to voluntary additional work of all participants beyond clinical routine, more than 1000 children with rare lung diseases have been included in the registry with biobank to date. A deeper understanding of the clinical courses of large cohorts of rare diseases and the initial description of new entities contributes to better care for these children.

[CF Lung Disease - a German S3 Guideline: Pseudomonas aeruginosa]. / S3-Leitlinie: Lungenerkrankung bei Mukoviszidose ­ Pseudomonas aeruginosa.

Cystic Fibrosis (CF) is the most common autosomal recessive genetic multisystemic disease. In Germany, it affects at least 8000 people. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the airway epithelial lining fluid which leads to reduction of the mucociliary clearance.Even if highly effective, CFTR modulator therapy has been available for some years and people with CF are getting much older than before, recurrent and chronic infections of the airways as well as pulmonary exacerbations still occur. In adult CF life, Pseudomonas aeruginosa (PA) is the most relevant pathogen in colonisation and chronic infection of the lung, leading to further loss of lung function. There are many possibilities to treat PA-infection.This is a S3-clinical guideline which implements a definition for chronic PA-infection and demonstrates evidence-based diagnostic methods and medical treatment in order to give guidance for individual treatment options.

ABCA3-related interstitial lung disease beyond infancy.

BACKGROUND: The majority of patients with childhood interstitial lung disease (chILD) caused by pathogenic variants in ATP binding cassette subfamily A member 3 (ABCA3) develop severe respiratory insufficiency within their first year of life and succumb to disease if not lung transplanted. This register-based cohort study reviews patients with ABCA3 lung disease who survived beyond the age of 1 year. METHOD: Over a 21-year period, patients diagnosed as chILD due to ABCA3 deficiency were identified from the Kids Lung Register database. 44 patients survived beyond the first year of life and their long-term clinical course, oxygen supplementation and pulmonary function were reviewed. Chest CT and histopathology were scored blindly. RESULTS: At the end of the observation period, median age was 6.3 years (IQR: 2.8-11.7) and 36/44 (82%) were still alive without transplantation. Patients who had never received supplemental oxygen therapy survived longer than those persistently required oxygen supplementation (9.7 (95% CI 6.7 to 27.7) vs 3.0 years (95% CI 1.5 to 5.0), p=0.0126). Interstitial lung disease was clearly progressive over time based on lung function (forced vital capacity % predicted absolute loss -1.1% /year) and on chest CT (increasing cystic lesions in those with repetitive imaging). Lung histology pattern were variable (chronic pneumonitis of infancy, non-specific interstitial pneumonia, and desquamative interstitial pneumonia). In 37/44 subjects, the ABCA3 sequence variants were missense variants, small insertions or deletions with in-silico tools predicting some residual ABCA3 transporter function. CONCLUSION: The natural history of ABCA3-related interstitial lung disease progresses during childhood and adolescence. Disease-modifying treatments are desirable to delay such disease course.

[Chest X-ray: implementation and indication : Recommendations of the thoracic imaging working group of the German Radiological Society]. / Röntgen-Thorax: Durchführung und Indikationen : Empfehlungen der AG Thoraxdiagnostik der Deutschen Röntgengesellschaft.

BACKGROUND: Even after more than 100 years, the chest X­ray is still an important technique to detect important pathological changes of lungs, heart and vessels in a fast and low-dose manner. For the German-speaking regions, there are only recommendations available published by the "Ständigen Strahlenschutzkommission (SSK)" regarding the indication. These recommendations are not updated on a regular basis and more recent developments are only integrated with delayed. METHODS: The chest division of the German Radiological Society has summarized their expertise for the usage and indication of the chest X­ray. Especially within the field of oncology the usage of chest X­ray is evaluated differently to the aforementioned recommendations; here chest computed tomography (CT) is much more sensitive for evaluation of metastasis and local invasion of tumors. Also, within the area of infectious diseases in non-immunocompetent patients, CT is the method of choice. Based on the structure of the current recommendations, many current guidelines and indications are summarized and presented within the context of the usage of chest X­ray.

Abatacept for treatment-refractory pediatric CTLA4-haploinsufficiency.

CTLA4-haploinsufficiency is a complex disease of immune dysregulation presenting with a broad spectrum of clinical manifestations. CTLA4-Fc fusion proteins such as abatacept have been described to alleviate immune dysregulation in several adult cases of CTLA4-haploinsufficiency. However, until now only few cases of pediatric CTLA4-haploinsufficiency treated with abatacept have been described. Here we present two pediatric cases of severe CTLA4-haploinsufficiency refractory to conventional immunosuppressive therapies that responded rapidly to treatment with abatacept. No side effects were observed during a follow-up period of 7-15 months. While one patient has successfully undergone HSCT the second patient continues to receive abatacept. Our cases demonstrate safe medium-term use of abatacept in the pediatric population.

Sarcopenia is a prognostic outcome marker in children with high-risk hepatoblastoma.

BACKGROUND: Children with hepatoblastoma (HB) are at risk of sarcopenia due to immobility, chemotherapy, and malnutrition. We hypothesized that children with HB have a low preoperative total psoas muscle area (tPMA), reflecting sarcopenia, which negatively impacts outcome. PROCEDURE: Retrospective study of children (1-10 years) with hepatoblastoma treated at a large university children's hospital from 2009 to 2018. tPMA was measured as the sum of the right and left psoas muscle area (PMA) at intervertebral disc levels L3-4 and L4-5. z-Scores were calculated using age- and gender-specific reference values and were compared to anthropometric measurements, clinical variables, and outcomes. Sarcopenia was defined as a tPMA z-score below -2. RESULTS: Thirty-three children were included. Mean tPMA z-score was -2.18 ± 1.08, and 52% were sarcopenic. A poor correlation between tPMA and weight was seen (r = 0.35; confidence interval [CI] 0.01, 0.62; P = .045), and most children had weights within the normal range (mean z-score -0.55 ± 1.39). All children categorized as high risk with relapse (n = 5/12) were sarcopenic before surgery. Relapse was significantly higher in the high-risk sarcopenic group compared to the nonsarcopenic group (P = .008). The change in tPMA z-score 1-4 months after surgery did not improve in patients with relapse, but did improve in 75% of children without relapse. CONCLUSIONS: The majority of children with HB were sarcopenic prior to surgery. Especially in children with high-risk hepatoblastoma, sarcopenia is an additional risk factor for relapse. Large multicenter studies are needed to confirm these preliminary results.

Joint Statement of the German Respiratory Society and German Society of Thoracic Surgery in Cooperation with the German Radiological Society: Structural Prerequisites of Centres for Interventional Treatment of Emphysema.

Interventional treatment of emphysema offers a wide range of surgical and endoscopic options for patients with advanced disease. Multidisciplinary collaboration of pulmonology, thoracic surgery, and imaging disciplines in patient selection, therapy, and follow-up ensures treatment quality. The present joint statement describes the required structural and quality prerequisites of treatment centres. This is a translation of the German article "Positionspapier der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin und der Deutschen Gesellschaft für Thoraxchirurgie in Kooperation mit der Deutschen Röntgengesellschaft: Strukturvoraussetzungen von Zentren für die interventionelle Emphysemtherapie" Pneumologie. 2020;74:17-23.

Fully Automated Segmentation of Pulmonary Fibrosis Using Different Software Tools.

OBJECTIVE: Evaluation of software tools for segmentation, quantification, and characterization of fibrotic pulmonary parenchyma changes will strengthen the role of CT as biomarkers of disease extent, evolution, and response to therapy in idiopathic pulmonary fibrosis (IPF) patients. METHODS: 418 nonenhanced thin-section MDCTs of 127 IPF patients and 78 MDCTs of 78 healthy individuals were analyzed through 3 fully automated, completely different software tools: YACTA, LUFIT, and IMBIO. The agreement between YACTA and LUFIT on segmented lung volume and 80th (reflecting fibrosis) and 40th (reflecting ground-glass opacity) percentile of the lung density histogram was analyzed using Bland-Altman plots. The fibrosis and ground-glass opacity segmented by IMBIO (lung texture analysis software tool) were included in specific regression analyses. RESULTS: In the IPF-group, LUFIT outperformed YACTA by segmenting more lung volume (mean difference 242 mL, 95% limits of agreement -54 to 539 mL), as well as quantifying higher 80th (76 HU, -6 to 158 HU) and 40th percentiles (9 HU, -73 to 90 HU). No relevant differences were revealed in the control group. The 80th/40th percentile as quantified by LUFIT correlated positively with the percentage of fibrosis/ground-glass opacity calculated by IMBIO (r = 0.78/r = 0.92). CONCLUSIONS: In terms of segmentation of pulmonary fibrosis, LUFIT as a shape model-based segmentation software tool is superior to the threshold-based YACTA, tool, since the density of (severe) fibrosis is similar to that of the surrounding soft tissues. Therefore, shape modeling as used in LUFIT may serve as a valid tool in the quantification of IPF, since this mainly affects the subpleural space.

[Imaging of Mediastinal Tumors]. / Bildgebung bei Mediastinaltumoren.

In computed tomography, the mediastinum is split into ventral prevascular, middle visceral and dorsal paravertebral compartments. Each compartment contains specific tumours which will be presented and discussed briefly. The focus is on image-based analysis of morphological signs to differentiate between entities using X-rays, ultrasound, CT and MRI. It is however difficult to differentiate between various types of lesions based on morphology and to estimate the grade of malignancy. For this reason, functional imaging techniques like PET and MRI are essential tools for detailed and non-invasive work-up. If a histological tissue examination is required, these tissue samples can be acquired using CT guided biopsies with high diagnostic yield and low complication rates.

One-year outcomes in a multicentre cohort study of incident rare diffuse parenchymal lung disease in children (ChILD).

We performed a prospective, observational, cohort study of children newly diagnosed with children's interstitial lung disease (ChILD), with structured follow-up at 4, 8, 12 weeks and 6 and 12 months. 127 children, median age 0.9 (IQR 0.3-7.9) years had dyspnoea (68%, 69/102), tachypnoea (75%, 77/103) and low oxygen saturation (SpO2) median 92% (IQR 88-96). Death (n=20, 16%) was the most common in those <6 months of age with SpO2<94% and developmental/surfactant disorders. We report for the first time that ChILD survivors improved multiple clinical parameters within 8-12 weeks of diagnosis. These data can inform family discussions and support clinical trial measurements.

Lung disease in STAT3 hyper-IgE syndrome requires intense therapy.

BACKGROUND: Pulmonary complications are responsible for high morbidity and mortality rates in patients with the rare immunodeficiency disorder STAT3 hyper-IgE syndrome (STAT3-HIES). The aim of this study was to expand knowledge about lung disease in STAT3-HIES. METHODS: The course of pulmonary disease, radiological and histopathological interrelations, therapeutic management, and the outcome of 14 STAT3-HIES patients were assessed. RESULTS: The patients' quality of life was compromised most by pulmonary disease. All 14 patients showed first signs of lung disease at a median onset of 1.5 years of age. Lung function revealed a mixed obstructive-restrictive impairment with reduced FEV1 and FVC in 75% of the patients. The severity of lung function impairment was associated with Aspergillus fumigatus infection and prior lung surgery. Severe lung tissue damage, with reduced numbers of ATP-binding cassette sub-family A member 3 (ABCA3) positive type II pneumocytes, was observed in the histological assessment of two deceased patients. Imaging studies of all patients above 6 years of age showed severe airway and parenchyma destruction. Lung surgeries frequently led to complications, including fistula formation. Long-term antifungal and antibacterial treatment proved to be beneficial, as were inhalation therapy, chest physiotherapy, and exercise. Regular immunoglobulin replacement therapy tended to stabilize lung function. CONCLUSIONS: Due to its severity, pulmonary disease in STAT3-HIES patients requires strict monitoring and intensive therapy.

Management of Spontaneous Pneumothorax and Post-Interventional Pneumothorax: German S3 Guideline.

In Germany, 10,000 cases of spontaneous pneumothorax are treated inpatient every year. The German Society for Thoracic Surgery, in co-operation with the German Society for Pulmonology, the German Radiological Society, and the German Society of Internal Medicine has developed an S3 guideline on spontaneous pneumothorax and post-interventional pneumothorax moderated by the German Association of Scientific Medical Societies. METHOD: Based on the source guideline of the British Thoracic Society (2010) for spontaneous pneumothorax, a literature search on spontaneous pneumothorax was carried out from 2008 onwards, for post-interventional pneumothorax from 1960 onwards. Evidence levels according to the Oxford Center for Evidence-Based Medicine (2011) were assigned to the relevant studies found. Recommendations according to grade (A: "we recommend"/"we do not recommend," B: "we suggest"/"we do not suggest") were determined in 3 consensus conferences by the nominal group process. RESULTS: The algorithms for primary and secondary pneumothorax differ in the indication for CT scan as well as in the indication for chest drainage application and video-assisted thoracic surgery. Indication for surgery is recommended individually taking into account the risk of recurrence, life circumstances, patient preferences, and procedure risks. For some forms of secondary pneumothorax, a reserved indication for surgery is recommended. Therapy of post-interventional spontaneous pneumothorax is similar to that of primary spontaneous pneumothorax. DISCUSSION: The recommendations of the S3 Guideline provide assistance in managing spontaneous pneumothorax and post-interventional pneumothorax. Whether this will affect existing deviant diagnostic and therapeutic measures will be demonstrated by future epidemiological studies.

Aortic Coarctation a Systemic Vessel Disease-Insights from Magnetic Resonance Imaging.

BACKGROUND: Even after successful aortic coarctation (CoA) repair, hypertension causes premature morbidity and mortality. The mechanisms are not clear. The aim was to evaluate elastic wall properties and aortic morphology and to correlate these results with severity of restenosis, hypertension, aortic arch geometry, noninvasive pressure gradients, and time and kind of surgical procedure. METHODS: Eighty-nine patients (17 ± 6.3 years) and 20 controls (18 ± 4.9 years) were examined using magnetic resonance imaging (MRI). In addition to contrast-enhanced MR angiography and flow measurements, CINE MRI was performed to assess the relative change of aortic cross-sectional areas at diaphragm level to calculate aortic compliance (C). RESULTS: Fifty-four percent of all patients showed hypertension (> 95th percentile), but more than half of them had no significant stenosis (defined as ≥30%). C was lower in CoA than in controls (3.30 ± 2.43 vs. 4.67 ± 2.21 [10-5 Pa-1 m-2]; p = 0.024). Significant differences in compliance were found between hyper- and normotensive patients (2.61 ± 1.60 vs. 4.11 ± 2.95; p = 0.01), and gothic and Romanesque arch geometry (2.64 ± 1.58 vs. 3.78 ± 2.81; p = 0.027). There was a good correlation between C and hypertension (r = 0.671; p < 0.01), but no correlation between C (and hypertension) and time or kind of repair, restenosis, or pressure gradients. CONCLUSION: The decreased compliance, a high rate of hypertension without restenosis, and independency of time and kind of repair confirm the hypothesis that CoA may not be limited to isthmus region but rather be a widespread (systemic) vascular anomaly at least in some of the CoA patients. Therefore, aortic compliance should be assessed in these patients to individually tailor treatment of CoA patients with restenosis and/or hypertension.

International management platform for children's interstitial lung disease (chILD-EU).

BACKGROUND: Children's interstitial lung diseases (chILD) cover many rare entities, frequently not diagnosed or studied in detail. There is a great need for specialised advice and for internationally agreed subclassification of entities collected in a register.Our objective was to implement an international management platform with independent multidisciplinary review of cases at presentation for long-term follow-up and to test if this would allow for more accurate diagnosis. Also, quality and reproducibility of a diagnostic subclassification system were assessed using a collection of 25 complex chILD cases. METHODS: A web-based chILD management platform with a registry and biobank was successfully designed and implemented. RESULTS: Over a 3-year period, 575 patients were included for observation spanning a wide spectrum of chILD. In 346 patients, multidisciplinary reviews were completed by teams at five international sites (Munich 51%, London 12%, Hannover 31%, Ankara 1% and Paris 5%). In 13%, the diagnosis reached by the referring team was not confirmed by peer review. Among these, the diagnosis initially given was wrong (27%), imprecise (50%) or significant information was added (23%).The ability of nine expert clinicians to subcategorise the final diagnosis into the chILD-EU register classification had an overall exact inter-rater agreement of 59% on first assessment and after training, 64%. Only 10% of the 'wrong' answers resulted in allocation to an incorrect category. Subcategorisation proved useful but training is needed for optimal implementation. CONCLUSIONS: We have shown that chILD-EU has generated a platform to help the clinical assessment of chILD. TRIAL REGISTRATION NUMBER: Results, NCT02852928.

Management of Spontaneous Pneumothorax and Postinterventional Pneumothorax: German S3-Guideline. / S3-Leitlinie: Diagnostik und Therapie von Spontanpneumothorax und postinterventionellem Pneumothorax.

In Germany, 10,000 cases of spontaneous pneumothorax are treated inpatient every year. The German Society for Thoracic Surgery (DGT), in co-operation with the German Society for Pulmonology (DGP), the German Radiological Society (DRG) and the German Society of Internal Medicine (DGIM) has developed an S3 guideline on spontaneous pneumothorax and postinterventional pneumothorax moderated by the German Association of Scientific Medical Societies (AWMF). METHOD: Based on the source guideline of the British Thoracic Society (BTS2010) for spontaneous pneumothorax, a literature search on spontaneous pneumothorax was carried out from 2008 onwards, for post-interventional pneumothorax from 1960 onwards. Evidence levels according to the Oxford Center for Evidence-Based Medicine (2011) were assigned to the relevant studies found. Recommendations according to GRADE (A: "we recommend"/"we do not recommend", B: "we suggest"/"we do not suggest") were determined in three consensus conferences by the nominal group process. RESULTS: The algorithms for primary and secondary pneumothorax differ in the indication for CT scan as well as in the indication for chest drainage application and video-assisted thoracic surgery (VATS). Indication for surgery is recommended individually taking into account the risk of recurrence, life circumstances, patient preferences and procedure risks. For some forms of secondary pneumothorax, a reserved indication for surgery is recommended. Therapy of postinterventional spontaneous pneumothorax is similar to that of primary spontaneous pneumothorax. DISCUSSION: The recommendations of the S3 Guideline provide assistance in managing spontaneous pneumothorax and post-interventional pneumothorax. Whether this will affect existing deviant diagnostic and therapeutic measures will be demonstrated by future epidemiological studies.

Persistent Tachypnea of Infancy. Usual and Aberrant.

RATIONALE: Persistent tachypnea of infancy (PTI) is a specific clinical entity of undefined etiology comprising the two diseases neuroendocrine cell hyperplasia of infancy (NEHI) and pulmonary interstitial glycogenosis. The outcome of typical NEHI is favorable. The outcome may be different for patients without a typical NEHI presentation, and thus a lung biopsy to differentiate the diseases is indicated. OBJECTIVES: To determine whether infants with the characteristic clinical presentation and computed tomographic (CT) imaging of NEHI (referred to as "usual PTI") have long-term outcome and biopsy findings similar to those of infants with an aberrant presentation and/or with additional localized minor CT findings (referred to as "aberrant PTI"). METHODS: In a retrospective cohort study, 89 infants with PTI were diagnosed on the basis of clinical symptoms and, if available, CT scans and lung biopsies. Long-term outcome in childhood was measured on the basis of current status. MEASUREMENTS AND MAIN RESULTS: Infants with usual PTI had the same respiratory and overall outcomes during follow-up of up to 12 years (mean, 3.8 yr) as infants who had some additional localized minor findings (aberrant PTI) visualized on CT images. Both usual and aberrant PTI had a relatively favorable prognosis, with 50% of the subjects fully recovered by age 2.6 years. None of the infants died during the study period. This was independent of the presence or absence of histological examination. CONCLUSIONS: PTI can be diagnosed on the basis of typical history taking, clinical findings, and a high-quality CT scan. Further diagnostic measures, including lung biopsies, may be limited to rare, complicated cases, reducing the need for an invasive and potentially harmful procedure.

Changes of Emphysema Parameters over the Respiratory Cycle During Free Breathing: Preliminary Results Using Respiratory Gated 4D-CT.

The purpose of this research was to evaluate respiratory gated CT of the lung in patients with COPD for analysis of parenchymal characteristics who were potential candidates for volume reduction surgery. Eleven patients with clinically known emphysematous disease underwent a respiratory gated, free-breathing 64-multislice-CT (Aquilion 64, Toshiba). Retrospective image reconstruction was performed similar to cardiac CT at every 10% of the respiratory loop, resulting in 10 complete volumetric datasets at 10 equidistant time points. All images were transferred onto a PC for calculation of the total lung volume, emphysema volume, emphysema index, and mean lung density. Complete datasets could be successfully reconstructed in all patients. The mean lung volume increased from 6.9 L to 7.5 L over the respiratory cycle. Emphysema volume increased from 1.6 L to 2.0 L and emphysema index from 22.6% to 26.5% from expiration to inspiration. In conclusion, respiratory gated chest 4D-CT allows for combined morphologic and functional image analysis, which can provide new insight into functional impairment and individual treatment planning.

Imaging in vascular diseases of the lung.

PURPOSE OF REVIEW: The present review focuses on the recent developments in the field of pulmonary angiography and perfusion by means of computed tomography and magnetic resonance imaging. RECENT FINDINGS: Computed tomography allows for a detailed analysis of large and small pulmonary vascular pathologies and simultaneously allows for evaluation of the lung parenchyma. Magnetic resonance imaging allows for large and small vessel evaluation as well as noninvasive pressure assessment. Furthermore, recently non-breath-hold contrast-enhanced and noncontrast-enhanced techniques have been developed making magnetic resonance imaging an ideal tool for comprehensive thoracic imaging, even in challenging patients. SUMMARY: Noninvasive imaging using computed tomography and magnetic resonance imaging further increases their value in daily clinical practice when it comes to assessment of large and small pulmonary artery disease. As computed tomography is more easy to use and widely available, it remains the diagnostic modality of choice. However, magnetic resonance imaging is the modality of choice when a comprehensive angiographic and functional assessment is deemed necessary.

Assessment of the relationship between morphological emphysema phenotype and corresponding pulmonary perfusion pattern on a segmental level.

PURPOSE: Distinct morphological emphysema phenotypes were assessed by CT to show characteristic perfusion defect patterns. MATERIAL/METHODS: Forty-one patients with severe emphysema (GOLD III/IV) underwent three-dimensional high resolution computed tomography (3D-HRCT) and contrast-enhanced magnetic resonance (MR) perfusion. 3D-HRCT data was visually analyzed for emphysema phenotyping and quantification by consensus of three experts in chest-radiology. The predominant phenotype per segment was categorized as normal, centrilobular, panlobular or paraseptal. Segmental lung perfusion was visually analyzed using six patterns of pulmonary perfusion (1-normal; 2-mild homogeneous reduction in perfusion; 3-heterogeneous perfusion without focal defects; 4-heterogeneous perfusion with focal defects; 5-heterogeneous absence of perfusion; 6-homogeneous absence of perfusion), with the extent of the defect given as a percentage. RESULTS: 730 segments were evaluated. CT categorized 566 (78%) as centrilobular, 159 (22%) as panlobular and 5 (<1%) as paraseptal with no normals. Scores with regards to MR perfusion patterns were: 1-0; 2-0; 3-28 (4%); 4-425 (58%); 5-169 (23%); 6-108 (15%). The predominant perfusion pattern matched as follows: 70 % centrilobular emphysema - heterogeneous perfusion with focal defects (score 4); 42% panlobular--homogeneous absence of perfusion (score 5); and 43% panlobular--heterogeneous absence of perfusion (score 6). CONCLUSION: MR pulmonary perfusion patterns correlate with the CT phenotype at a segmental level in patients with severe emphysema. KEY POINTS: • MR perfusion patterns correlate with the CT phenotype in emphysema. • Reduction of MR perfusion is associated with loss of lung parenchyma on CT • Centrilobular emphysema shows heterogeneous perfusion reduction while panlobular emphysema shows loss of perfusion.