Rationale Information is missing on the tissue cell expression patterns of interleukin 33 (IL-33) and splice variants of the IL-33 receptor ST2 in normal and chronic obstructive pulmonary disease (COPD) lungs.Objectives To characterize the expression patterns of IL-33, the soluble ST2 (sST2) and membrane-bound ST2 (ST2L) splice variants in the poorly studied small airway and distal lung compartments in COPD and controls.Methods Surgically excised lung tissue was collected from 38 COPD patients and 21 non-COPD controls. Lung compartment expression of IL-33 and ST2 and key expressing cell types were assessed histologically by combined in situ hybridization and multiplex immunohistochemistry. Expression dynamics of IL-33, ST2L, and sST2 were explored by spatially resolved single-cell analysis.Measurements and Main Results COPD lungs displayed increased IL-33 mRNA and IL-33 mRNA/protein ratios, suggesting increased IL-33 turnover. Total ST2/IL1RL1 mRNA levels were upregulated in COPD lungs. Mast cells constituted the major ST2-expressing immune cell population in controls and displayed a microenvironmental-specific upregulation of both ST2L and sST2 in COPD. In control alveolar regions, ST2Lhigh sST2high mast cells were present alongside IL-33-expressing general capillary (gCap) and sST2moderate ST2Llow aerocyte endothelial subsets. In COPD, patchy alveolar regions displayed markedly elevated capillary sST2 and numbers of ST2L+ and IL-33+ gCaps.Conclusions By unraveling the expression patterns of IL-33 and the biologically opposing ST2L and sST2 splice variants in control and COPD lungs, the present study provides novel insights into IL-33-mediated immunity in the distal lung, information that has bearing on treatment strategies targeting this pathway in lung diseases.

Background:Monitoring lung function course in patients with pulmonary fibrosis is crucial to guide treatment decisions. Online home spirometry holds great potential for close monitoring and improving care access in times of pressured healthcare systems. However, little data is available on its large-scale use in daily practice. We evaluated the clinical applicability of online home spirometry in pulmonary fibrosis care. Methods:We analysed data of a nationwide Dutch cohort of patients with idiopathic pulmonary fibrosis (IPF) and other forms of pulmonary fibrosis (PF) that used a home monitoring programme with home spirometry (forced vital capacity (FVC)) as part of daily practice. Changes in FVC were evaluated with a joint model. Within-patient variability was measured using coefficient of variation. Home spirometry use over time and patient experiences were assessed. Results:Online home spirometry data of 334 patients (IPF 73.1%) were analysed. Patients with IPF had a mean baseline FVC of 3.02 L (95% CI: 2.27-3.79), with a mean annualised decline of 170 mL (4.0%). Patients with PF had a mean baseline FVC of 2.81 L (95% CI: 2.07-3.55) with a mean annualised decline of 88 mL (1.2%). Mean±sd within-patient variability was 5.6±4.6%. Overall patient satisfaction was high, and after 1 year 63.4% still performed online home spirometry. Conclusion:Online home spirometry is feasible on a large scale in daily clinical practice to monitor disease trajectories in patients with IPF and PF. Online home spirometry could guide management decisions and improve care access for a majority of patients with pulmonary fibrosis.

BackgroundAn adequate diagnosis for interstitial lung disease (ILD) is important for clinical decision making and prognosis. In most patients with ILD, an accurate diagnosis can be made by clinical and radiological data assessment, but in a considerable proportion of patients, a lung biopsy is required. Surgical lung biopsy (SLB) is the most common method to obtain tissue, but it is associated with high morbidity and even mortality. More recently, transbronchial cryobiopsy has been introduced, with fewer adverse events but a lower diagnostic yield than SLB. The aim of this study is to compare two diagnostic strategies: a step-up strategy (transbronchial cryobiopsy, followed by SLB if the cryobiopsy is insufficiently informative) versus immediate SLB.MethodsThe COLD study was a multicentre, randomised controlled trial in six hospitals across the Netherlands. We included patients with ILD with an indication for lung biopsy as assessed by a multidisciplinary team discussion. Patients were randomly assigned in a 1:1 ratio to the step-up or immediate SLB strategy, with follow-up for 12 weeks from the initial procedure. Patients, clinicians, and pathologists were not masked to the study treatment. The primary endpoint was unexpected chest tube drainage, defined as requiring any chest tube after transbronchial cryobiopsy, or prolonged (>24 h) chest tube drainage after SLB. Secondary endpoints were diagnostic yield, in-hospital stay, pain, and serious adverse events. A modified intention-to-treat analysis was performed. This trial is registered with the Dutch Trial Register, NL7634, and is now closed.FindingsBetween April 8, 2019, and Oct 24, 2021, 122 patients with ILD were assessed for study participation; and 55 patients were randomly assigned to the step-up strategy (n=28) or immediate SLB (n=27); three patients from the immediate SLB group were excluded. Unexpected chest tube drainage occurred in three of 28 patients (11%; 95% CI 4–27%) in the step-up group, and the number of patients for whom the chest tube could not be removed within 24 h was 11 of 24 patients (46%; 95% CI 2–65%) in the SLB group, with an absolute risk reduction of 35% (11–56%; p=0·0058). In the step-up strategy, the multidisciplinary team diagnostic yield after transbronchial cryobiopsy alone was 82% (64–92%), which increased to 89% (73–96%) when subsequent SLB was performed after inconclusive transbronchial cryobiopsy. In the immediate surgery strategy, the multidisciplinary team diagnostic yield was 88% (69–97%). Total in-hospital stay was 1 day (IQR 1–1) in the step-up group versus 5 days (IQR 4–6) in the SLB group. One (4%) serious adverse event occurred in step-up strategy versus 12 (50%) in the immediate SLB strategy.InterpretationIn ILD diagnosis, if lung tissue assessment is required, a diagnostic strategy starting with transbronchial cryobiopsy, followed by SLB when transbronchial cryobiopsy is inconclusive, appears to result in a significant reduction of patient burden and in-hospital stay with a similar diagnostic yield versus immediate SLB.FundingNetherlands Organisation for Health Research and Development (ZonMW) and Amsterdam University Medical Centers.

IntroductionSarcoidosis is a highly variable disease in terms of organ involvement, type of onset and course. Associations of genetic polymorphisms with sarcoidosis phenotypes have been observed and suggest genetic signatures.MethodsAfter obtaining a positive vote of the competent ethics committee we genotyped 1909 patients of the deeply phenotyped Genetic-Phenotype Relationship in Sarcoidosis (GenPhenReSa) cohort of 31 European centers in 12 countries with 116 potentially disease-relevant single-nucleotide polymorphisms (SNPs). Using a meta-analysis, we investigated the association of relevant phenotypes (acute vs. sub-acute onset, phenotypes of organ involvement, specific organ involvements, and specific symptoms) with genetic markers. Subgroups were built on the basis of geographical, clinical and hospital provision considerations.ResultsIn the meta-analysis of the full cohort, there was no significant genetic association with any considered phenotype after correcting for multiple testing. In the largest sub-cohort (Serbia), we confirmed the known association of acute onset with TNF and reported a new association of acute onset an HLA polymorphism. Multi-locus models with sets of three SNPs in different genes showed strong associations with the acute onset phenotype in Serbia and Lublin (Poland) demonstrating potential region-specific genetic links with clinical features, including recently described phenotypes of organ involvement.DiscussionThe observed associations between genetic variants and sarcoidosis phenotypes in subgroups suggest that gene–environment-interactions may influence the clinical phenotype. In addition, we show that two different sets of genetic variants are permissive for the same phenotype of acute disease only in two geographic subcohorts pointing to interactions of genetic signatures with different local environmental factors. Our results represent an important step towards understanding the genetic architecture of sarcoidosis.

Background: Adequate diagnosis for interstitial lung disease (ILD) is important for clinical decision making and prognosis. In most ILD patients, an accurate diagnosis can be made by clinical and radiological data assessment, but in a considerable proportion lung biopsy is required. Surgical lung biopsy (SLB) is the most common modality to obtain tissue but is associated with morbidity and even mortality. More recently, transbronchial cryobiopsy (TBCB) has been introduced, with less adverse events, but lower diagnostic yield. We compared a step-up diagnostic strategy (TBCB, if insufficiently informative followed by SLB) with immediate surgical lung biopsy.Methods: In a multicentre, randomized controlled trial, we included consecutive ILD patients with an indication for lung biopsy as assessed by multi-disciplinary team (MDT) discussion(Dutch Trial Register (NL7634)). Patients were randomly (1:1) assigned to the step-up or immediate SLB strategy. The primary endpoint was unexpected chest tube drainage, defined as requiring any chest tube following TBCB, or prolonged (>24h) chest tube following SLB. Secondary endpoints were diagnostic yield and in-hospital stay, pain, health-related quality of life and serious adverse events (SAE). A modified intention to treat analysis was performed. Patients, clinicians and pathologists were not blinded.Findings: Between April 2019 and September 2021, 52 analysed patients were randomised to the step-up strategy (n=28) or immediate SLB (n=24). Unexpected chest tube drainage occurred in 10.7% (n=3) and 45.8% (n=11), respectively, an absolute risk reduction of 35.1% (95%CI 10.8 to 55.5; p<0.005). In the step-up strategy, MDT diagnostic yield following TBCB alone was 82.1% (95%CI 64.4-92.1), which increased to 89.3% (95%CI 72.8-96.3) when subsequent SLB was performed after inconclusive TBCB. In the surgery strategy, MDT diagnostic yield was 87.5% (95%CI 69-96.6). Total in-hospital stay was 1 (IQR 1-1) versus 4.5 (IQR 4-5.75) days, respectively.Interpretation: In ILD diagnosis, a diagnostic strategy starting with TBCB, followed by SLB when TBCB is inconclusive, results in significant reduction of patient burden and in hospital stay with a similar diagnostic yield versus immediate SLB. .Trial Registration: (Dutch Trial Register (NL7634)). Funding: Amsterdam UMC, Netherlands Organisation for Health Research and Development (ZonMW).Declaration of Interest: All authors declare no personal competing interests. Ethical Approval: The trial was approved by the Medical Ethics Committee of the Academic Medical Centre Amsterdam (identifier: 2018_224), and was performed in accordance with good clinical practice guidelines and the declaration of Helsinki. All patients provided written informed consent before randomization.

Aims: Home spirometry has increasingly been studied in pulmonary fibrosis, but is not yet widely implemented in regular care. We evaluated the use of home spirometry in regular care, and assessed lung function course in patients with idiopathic pulmonary fibrosis (IPF) and other forms of pulmonary fibrosis (PF). Methods: In this real-world national multicenter cohort, patients with IPF and PF measured their forced vital capacity (FVC) with online home spirometry. Disease course was classified as stable/slowly progressive when ∆FVC decline was < 5%, moderate progressive if ∆FVC decline was >5% and <10%, and rapidly progressive if ∆FVC decline was >10% over 1 year. Linear mixed models were used to analyze 1-years data. Results: We analyzed home spirometry data from 373 patients (IPF n=273,PF n=100, median age 72 years (IQR11),79% male) from 9 centers in the Netherlands. Patients had a mean baseline FVC of 3.06L. Mean FVC decline after 1 year was 0.23L. The coefficient of variation was 6%. There was no significant difference between lung function course in patients with IPF and PF (p=0.18). In the IPF group, 41% had a stable/slowly progressive disease course, 31% had a moderate progressive disease course and 28% rapidly progressive. In the PF group, 46% were stable/slowly progressive, 17% moderate progressive and 37% had a rapidly progressive disease course. Conclusion: Home spirometry is feasible and reliable as part of regular care for patients with IPF and PF. FVC decline measured with home spirometry was comparable to previous studies and real-life cohorts measured with in-hospital spirometry. Interestingly, patients with PF had an IPF-like disease course, which was rapidly progressive in one third of the patients.

Transfusion‐related acute lung injury (TRALI) is a severe complication of blood transfusion that is thought of as a two‐hit event: first the underlying patient condition (e.g., sepsis), and then the transfusion. Transfusion factors include human leukocyte antigen antibodies or biologic response modifiers (BRMs) accumulating during storage. Preclinical studies show an increased TRALI risk with longer stored platelets, clinical studies are conflicting. We aim to discover whether longer platelet concentrate (PC) storage time increases TRALI risk in a controlled human experiment.

Background In ILD patients with an indication for lung biopsy, surgical lung biopsy (SLB) has high diagnostic yield but is associated with significant morbidity and requires in-hospital stay. Transbronchial cryobiopsy (TBCB) is a less invasive outpatient alternative to SLB. The optimal diagnostic strategy is unknown. Aim To compare 2 diagnostic strategies: TBCB, if inconclusive followed by SLB (cryo-strategy) versus immediate SLB (surgery-strategy). Methods Randomized study in 5 Dutch hospitals(NL7634). Consecutive ILD patients with an indication for lung biopsy were randomized. Primary endpoint: (prolonged) chest tube drainage, as surrogate outcome for patient burden, defined as: proportion of patients requiring post-procedural chest tube (cryo-strategy) or prolonged (>24h) chest tube drainage (surgery-strategy). Secondary endpoints included diagnostic yield, in-hospital stay and adverse events. Results 55 patients (80% male; Age 66+7 years; FVC 85%+9%; DLCO 55%+11%) were randomised: 28 to cryo-strategy (step-up SLB was performed in 3 after inconclusive TBCB) and 27 to surgery-strategy. 14% patients in de cryo-strategy required (prolonged) chest tube drainage (95%CI 1.3-27; n=4) versus 41% in the surgery-strategy (95%CI 22-59; n=11; p=0.028); diagnostic yield for MDT diagnosis was 89% (72-98) and 88% (74-100); median in-hospital stay 0 days (range: 0-14) and 3.5 days (range: 1-16); serious adverse events (other than chest tube drainage) 0.04%(n=1) and 25%(n=6), respectively. Conclusion A cryobiopsy centered diagnostic strategy, if inconclusive followed by SLB, results in significant reduction of adverse events and hospital stay, while diagnostic yield of MDT diagnosis appears similar.

Antecedentes: Con frecuencia, los pacientes con enfermedades pulmonares intersticiales (EPI) presentan resultados no-diagnósticos en la tomografía computarizada de alta resolución (TCAR) y el lavado broncoalveolar (LBA), lo que hace necesario realizar una biopsia quirúrgica o criopulmonar invasiva, que se asocia con morbilidad significativa. La endomicroscopía láser confocal (CLE) y la tomografía de coherencia óptica (OCT) son técnicas de alta resolu­ción basadas en el láser y la luz, que proporcionan imágenes en tiempo real del compartimento alveolar durante la broncoscopía, con distinta profundidad y campo de visión. Objetivos: El objetivo del estudio fue correlacionar las imágenes por OCT y CLE con imágenes por TCAR en la EPI. Métodos: Presentamos una serie retrospectiva con casos de 20 pacientes con EPI, en quienes se tomaron imágenes por CLE y OCT alveolares durante una broncoscopía estándar con LBA, seguida de una biopsia pulmonar cuando ésta estaba indicada. Las imágenes por CLE y OCT se compararon con cuatro patrones principales de TCAR y con la histología. El diagnóstico final se basó en una discusión multidisciplinaria. Resultados: La obtención de imágenes por CLE y OCT broncoscópicas fue factible y segura, y proporcionó información anatómica adicional de gran detalle en comparación con la TCAR. La CLE broncoscópica en tiempo real fue capaz de identificar «células alveolares» (opacidades con aspecto de vidrio esmerilado) y fibrosis pulmonar (aumento de las fibras de elastina alveolares). La OCT broncoscópica en tiempo real permitió visualizar la «enfermedad fibrótica en parches», el «panal de abejas» (microquistes) y granulomas en la mucosa respiratoria. Conclusiones: La CLE y la OCT broncoscópicas del compartimento alveolar son factibles y seguras, y permiten detectar de forma mínimamente invasiva y con alta resolución las características específicas de la EPI, con potencial para mejorar el diagnóstico y el seguimiento de la enfermedad, y reducir la necesidad de realizar biopsias quirúrgicas o criopulmonares.

BACKGROUND:Patients with interstitial lung diseases (ILDs) frequently present with nondiagnostic high-resolution CT (HRCT) scan and bronchoalveolar lavage (BAL) results, resulting in the need for invasive surgical or cryo-lung biopsy that is associated with significant morbidity. Confocal laser endomicroscopy (CLE) and optical coherence tomography (OCT) are high-resolution laser and light-based techniques that provide real-time imaging of the alveolar compartment during bronchoscopy with a different depth and field of view.OBJECTIVES:The aim of the study was to correlate OCT and CLE imaging to HRCT imaging in ILD.METHODS:This is a retrospective case series of 20 ILD patients who underwent alveolar CLE and OCT imaging during a standard bronchoscopy with BAL, followed by a lung biopsy when indicated. CLE and OCT imaging were compared to four main HRCT patterns and histology. The final diagnosis was based on the multidisciplinary discussion diagnosis.RESULTS:Bronchoscopic CLE and OCT imaging were feasible and safe and provided additional high-detailed anatomical information compared to the HRCT. Bronchoscopic real-time CLE was capable of identification of "alveolar cells" (ground glass opacities) and lung fibrosis (increased alveolar elastin fibers). Bronchoscopic real-time OCT allowed for visualization of "patchy fibrotic disease", "honeycombing" (microcysts), and mucosal granulomas in the airways.CONCLUSIONS:Bronchoscopic CLE and OCT of the alveolar compartment is feasible and safe and enables minimally invasive, high-resolution detection of specific ILD features with the potential to improve ILD diagnostics and monitoring and decrease the need for surgical or cryo-lung biopsies.

Background Treatment for interstitial lung disease (ILD) patients with acute respiratory failure (ARF) is challenging, and literature to guide such treatment is scarce. The reported in-hospital mortality rates of ILD patients with ARF are high (62–66%). Cyclophosphamide is considered a second-line treatment in steroid-refractory ILD-associated ARF. The first aim of this study was to evaluate the in-hospital mortality in patients with ILD-associated ARF treated with cyclophosphamide. The second aim was to compare computed tomographic (CT) patterns and physiological and ventilator parameters between survivors and non-survivors. Methods Retrospective analysis of patients with ILD-associated ARF treated with cyclophosphamide between February 2016 and October 2017. Patients were categorized into three subgroups: connective tissue disease (CTD)-associated ILD, other ILD or vasculitis. In-hospital mortality was evaluated in the whole cohort and in these subgroups. Clinical response was determined using physiological and ventilator parameters: Sequential Organ Failure Assessment Score (SOFA), PaO2/FiO2 (P/F) ratio and dynamic compliance (Cdyn) before and after cyclophosphamide treatment. The following CT features were quantified: ground-glass opacification (GGO) proportion, reticulation proportion, overall extent of parenchymal disease and fibrosis coarseness score. Results Fifteen patients were included. The overall in-hospital mortality rate was 40%. In-hospital mortality rates for CTD-associated ILD, other ILD and vasculitis were 20, 57, and 33%, respectively. The GGO proportion (71% vs 45%) was higher in non-survivors. There were no significant differences in the SOFA score, P/F ratio or Cdyn between survivors and non-survivors. However, in survivors the P/F ratio increased from 129 to 220 mmHg and Cdyn from 75 to 92 mL/cmH2O 3 days after cyclophosphamide treatment. In non-survivors the P/F ratio hardly changed (113–114 mmHg) and Cdyn even decreased (27–20 mL/cmH2O). Conclusion In this study, we found a mortality rate of 40% in patients treated with cyclophosphamide for ILD-associated ARF. Connective tissue disease-associated ILD and vasculitis were associated with a lower risk of death. In non-survivors, the CT GGO proportion was significantly higher. The P/F ratio and Cdyn in survivors increased after 3 days of cyclophosphamide treatment.

High titers and low fucosylation of human anti–spike protein IgG promote alveolar macrophage activation and inflammation.

ScopeThe Dutch Working Party on Antibiotic Policy constituted a multidisciplinary expert committee to provide evidence-based recommendation for the use of antibacterial therapy in hospitalized adults with a respiratory infection and suspected or proven 2019 Coronavirus disease (COVID-19).MethodsWe performed a literature search to answer four key questions. The committee graded the evidence and developed recommendations by using Grading of Recommendations Assessment, Development, and Evaluation methodology.Questions addressed by the guideline and RecommendationsWe assessed evidence on the risk of bacterial infections in hospitalized COVID-19 patients, the associated bacterial pathogens, how to diagnose bacterial infections and how to treat bacterial infections. Bacterial co-infection upon admission was reported in 3.5% of COVID-19 patients, while bacterial secondary infections during hospitalization occurred up to 15%. No or very low quality evidence was found to answer the other key clinical questions. Although the evidence base on bacterial infections in COVID-19 is currently limited, available evidence supports restrictive antibiotic use from an antibiotic stewardship perspective, especially upon admission. To support restrictive antibiotic use, maximum efforts should be undertaken to obtain sputum and blood culture samples as well as pneumococcal urinary antigen testing. We suggest to stop antibiotics in patients who started antibiotic treatment upon admission when representative cultures as well as urinary antigen tests show no signs of involvement of bacterial pathogens after 48 hours. For patients with secondary bacterial respiratory infection we recommend to follow other guideline recommendations on antibacterial treatment for patients with hospital-acquired and ventilator-associated pneumonia. An antibiotic treatment duration of five days in patients with COVID-19 and suspected bacterial respiratory infection is recommended upon improvement of signs, symptoms and inflammatory markers. Larger, prospective studies about the epidemiology of bacterial infections in COVID-19 are urgently needed to confirm our conclusions and ultimately prevent unnecessary antibiotic use during the COVID-19 pandemic.

For yet unknown reasons, severely ill COVID-19 patients often become critically ill around the time of activation of adaptive immunity. Here, we show that anti-Spike IgG from serum of severely ill COVID-19 patients induces a hyper-inflammatory response by human macrophages, which subsequently breaks pulmonary endothelial barrier integrity and induces microvascular thrombosis. The excessive inflammatory capacity of this anti-Spike IgG is related to glycosylation changes in the IgG Fc tail. Moreover, the hyper-inflammatory response induced by anti-Spike IgG can be specifically counteracted in vitro by use of the active component of fostamatinib, an FDA- and EMA-approved therapeutic small molecule inhibitor of Syk. One sentence summary Anti-Spike IgG promotes hyper-inflammation. ### Competing Interest Statement The authors have declared no competing interest.

Human hematopoiesis is critically dependent on the transcription factor GATA2. Patients with GATA2 deficiency typically present with myelodysplastic syndrome, reduced numbers of monocytes, NK cells and B cells, and/or opportunistic infections. Here, we present two families that harbor distinct GATA2 mutations with highly variable onset and course of disease. We discuss the use of allogeneic hematopoietic cell transplantation in these patients, especially as treatment for pulmonary alveolar proteinosis.

Many patients with chronic obstructive lung disease (COPD) experience exacerbations. The diagnosis of an exacerbation is solely based on symptoms. We hypothesized that exhaled breath profiles, measured by Gas Chromatography-Mass Spectrometry (GC-MS) or electronic nose (eNose), are different between stable disease and exacerbations and may have the potential to serve as biomarkers for COPD exacerbations. In this prospective follow-up study, breath samples were taken during stable COPD, during a subsequent exacerbation and after recovery. Samples were analyzed by GC-MS and eNose. CCQ symptom scores were associated with univariate outcomes of GC-MS and eNose using analysis of covariance (ANCOVA). After multivariate modeling by Principal Component Analysis (PCA), paired student t-tests were performed. Sixty-eight patients were included, 31 had an exacerbation and 16 patients had breath sampled at all three time points. Significant differences were found in breathprints taken during exacerbation as compared to baseline and recovery for both GC-MS and eNose. Breath profiles obtained by GC-MS as well as by eNose showed a correct classification of 71% (10/14) for baseline vs exacerbation and of 78% (11/14) for exacerbation vs recovery. These results provide proof of principle that exhaled breath can serve as a noninvasive biomarker for the diagnosis of COPD exacerbations.

Journal of the European Academy of Dermatology and VenereologyVolume 34, Issue 4 p. e167-e169 Letter to the Editor Tattoos and self-reported adverse events in sarcoidosis patients S.A.S. van der Bent, Corresponding Author S.A.S. van der Bent s.bent@amsterdamumc.nl orcid.org/0000-0003-3380-4075 Department of Dermatology, Academic Tattoo Clinic Amsterdam, Amsterdam University Medical Center, Amsterdam, The NetherlandsBoth authors contributed equally to this manuscript.Correspondence: S.A.S. van der Bent. E-mail: s.bent@amsterdamumc.nlSearch for more papers by this authorM.J.C. Engel, M.J.C. Engel orcid.org/0000-0001-9303-9171 Department of Dermatology, Academic Tattoo Clinic Amsterdam, Amsterdam University Medical Center, Amsterdam, The NetherlandsBoth authors contributed equally to this manuscript.Search for more papers by this authorE.J. Nossent, E.J. Nossent Department of Pulmonology, Amsterdam University Medical Center, Amsterdam, The NetherlandsSearch for more papers by this authorR.E. Jonkers, R.E. Jonkers Department of Pulmonology, Amsterdam University Medical Center, Amsterdam, The NetherlandsSearch for more papers by this authorA. Wolkerstorfer, A. Wolkerstorfer Department of Dermatology, Academic Tattoo Clinic Amsterdam, Amsterdam University Medical Center, Amsterdam, The NetherlandsSearch for more papers by this authorT. Rustemeyer, T. Rustemeyer orcid.org/0000-0001-7580-0684 Department of Dermatology, Academic Tattoo Clinic Amsterdam, Amsterdam University Medical Center, Amsterdam, The NetherlandsSearch for more papers by this author S.A.S. van der Bent, Corresponding Author S.A.S. van der Bent s.bent@amsterdamumc.nl orcid.org/0000-0003-3380-4075 Department of Dermatology, Academic Tattoo Clinic Amsterdam, Amsterdam University Medical Center, Amsterdam, The NetherlandsBoth authors contributed equally to this manuscript.Correspondence: S.A.S. van der Bent. E-mail: s.bent@amsterdamumc.nlSearch for more papers by this authorM.J.C. Engel, M.J.C. Engel orcid.org/0000-0001-9303-9171 Department of Dermatology, Academic Tattoo Clinic Amsterdam, Amsterdam University Medical Center, Amsterdam, The NetherlandsBoth authors contributed equally to this manuscript.Search for more papers by this authorE.J. Nossent, E.J. Nossent Department of Pulmonology, Amsterdam University Medical Center, Amsterdam, The NetherlandsSearch for more papers by this authorR.E. Jonkers, R.E. Jonkers Department of Pulmonology, Amsterdam University Medical Center, Amsterdam, The NetherlandsSearch for more papers by this authorA. Wolkerstorfer, A. Wolkerstorfer Department of Dermatology, Academic Tattoo Clinic Amsterdam, Amsterdam University Medical Center, Amsterdam, The NetherlandsSearch for more papers by this authorT. Rustemeyer, T. Rustemeyer orcid.org/0000-0001-7580-0684 Department of Dermatology, Academic Tattoo Clinic Amsterdam, Amsterdam University Medical Center, Amsterdam, The NetherlandsSearch for more papers by this author First published: 27 November 2019 https://doi.org/10.1111/jdv.16115Citations: 1 Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume34, Issue4April 2020Pages e167-e169 RelatedInformation

Background: Transbronchial lung cryobiopsy (TBLCB) is a minimal invasive alternative for surgical lung biopsy in ILD patients (Hetzel et al, Respiration,2018). However, there is a substantial non-diagnostic rate of ~20-30% and a risk of pneumothorax ~10%. Confocal laser endomicroscopy (CLE) is a real-time imaging technique that visualizes elastin fibers in the intralobular compartment. We assessed whether the CLE technique has potential in guiding cryobiopsies by identification of fibrotic lung areas and the pleura. Methods: In ILD patients scheduled for lung biopsies (cryo- or VATS), CLE in vivo video imaging of lung tissue and visceral pleura was obtained during bronchoscopy, followed by biopsies at the same location. Ex-vivo measurements were performed subsequently. Results: In 14 patients, CLE imaging and corresponding histology were obtained. Two important biopsy-location characteristics were distinguished on CLE imaging: 1. A densely packed fiber-pattern that correlated with areas with fibrosis 2. A dense bright cross-pattern structure correlated with pleura Conclusion: Real-time CLE was capable of identifying key-characteristics for identification of the optimal biopsy area. Therefore CLE might have potential as a guidance tool for transbronchial lung cryobiopsies (TBLCB) to optimize diagnostic yield (identification of fibrotic areas and subpleural space) and reduce the complication rate (by avoidance the pleura).

Idiopathic inflammatory myopathy (IIM), commonly referred to as "myositis", is a rare but treatable auto-immune disease that is often misdiagnosed or diagnosed after significant delay. Using three clinical case reports as introductory examples, an overview is given - and pitfalls are discussed - of the diagnosis and treatment of myositis. Disease features are often extra-muscular in nature, may vary considerably between patients, and are frequently non-specific. Myositis-related morbidity is high and myositis can be fatal, mainly due to cancer and interstitial lung disease. As such, we stress the importance of early recognition of this severe disease and timely referral of a patient with a (suspected) IIM to a multidisciplinary team for optimal diagnosis and disease management.