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Background Idiopathic Pulmonary Fibrosis (IPF) represents a chronic lung disease with unpredictable course. Methods We aimed to investigate prognostic performance of complete blood count parameters in IPF. Treatment-naïve patients with IPF were retrospectively enrolled from two independent cohorts (derivation and validation) and split into subgroups (high and low) based on median baseline monocyte count and red cell distribution width (RDW). Results Overall, 489 patients (derivation cohort: 300, validation cohort: 189) were analyzed. In the derivation cohort, patients with monocyte count ≥ 0.60 K/μL had significantly lower median FVC%pred [75.0, (95% CI 71.3–76.7) vs. 80.9, (95% CI 77.5–83.1), (P = 0.01)] and DLCO%pred [47.5, (95% CI 44.3–52.3) vs. 53.0, (95% CI 48.0–56.7), (P = 0.02)] than patients with monocyte count P = 0.04)] and DLCO%pred [45.4, (95% CI 43.3–50.5) vs. 53.0, (95% CI 50.8–56.8), (P = 0.008)] than patients with RDW P P P = 0.09) and significantly lower median FVC%pred (P = 0.001), respectively. Kaplan–Meier analysis in the derivation cohort demonstrated higher all-cause mortality in patients with high (≥ 0.60 K/μL) vs. low monocyte count (P = 0.01)]. Conclusions Increased monocyte count and RDW may represent negative prognostic biomarkers in patients with IPF.

Interstitial lung diseases (ILDs) cover a wide heterogeneous group of disorders, both of unknown and known causes. Accurate diagnosis is essential but, at the same time, presents many challenges. Typically, the distinction between idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis (HP) can prove extremely difficult. However, another major, but underestimated, challenge is the diagnosis of connective tissue disease-associated ILD (CTD-ILD), specifically when ILD is the initial manifestation or when extrapulmonary manifestations are subclinical. Antisynthetase syndrome (ASyS) is a characteristic example where lung involvement can be the predominant feature in the absence of other evidence suggestive of CTD. In ASyS, lung involvement can be the initial manifestation or muscle involvement can be subclinical with normal muscle enzymes. Furthermore, a negative antinuclear antibody test does not indicate autoantibody negativity in the context of ASyS. Imaging and pathology findings in ASyS are not specific and overlap with other ILDs. Finally, bronchoalveolar lavage can exhibit pronounced lymphocytosis (>30–40%). The latter, in combination with a history of exposure to an inciting antigen, can lead to an erroneous diagnosis of HP with obvious negative impact on patients’ outcome. Herein, we report 3 female patients aged 61, 65, and 70 years and 1 male patient aged 43 years, with ASyS masquerading as HP and analyze the underlying reasons of misdiagnosis, aiming to raise awareness of the need for close collaboration between pulmonologists and rheumatologists.

Das Spektrum der interstitiellen Lungenerkrankungen (ILD) umfasst eine große und heterogene Gruppe von Krankheiten mit teils unbekannten und teils bekannten Ursachen. Die richtige Diagnosestellung ist von essenzieller Wichtigkeit, aber zugleich mit vielen Schwierigkeiten verbunden. Ein klassischer Problemfall ist die Abgrenzung zwischen idiopathischer pulmonaler Fibrose und fibrosierender Hypersensitivitätspneumonitis (HP). Eine ebenso große, jedoch unterschätzte Herausforderung ist die Diagnose von ILD im Kontext von Bindegewebserkrankungen (CTD-ILD), insbesondere wenn die ILD die erste Manifestation darstellt oder weitere, extrapulmonale Manifestationen subklinisch sind. Das Antisynthetase-Syndrom (ASyS) ist ein typisches Beispiel für eine solche Erkrankung, bei der es sein kann, dass die pulmonale Beteiligung das dominierende Merkmal ist und keine weiteren Anzeichen für eine CTD vorliegen. Beim ASyS kann die pulmonale Beteiligung die Erstmanifestation darstellen, oder die Muskelbeteiligung kann subklinisch sein, mit Muskelenzymwerten im Normbereich. Auch ein negativer Test auf antinukleäre Antikörper ist im Fall des ASyS kein Beweis für Autoantikörper-Negativität. Die radiologischen und pathologischen Befunde sind beim ASyS wenig spezifisch und überschneiden sich mit denen anderer ILD. In der bronchoalveolären Lavage schließlich kann eine ausgeprägte Lymphozytose vorliegen (>30–40%). Dies kann in Kombination mit einer anamnestisch bekannten Exposition gegenüber einem auslösenden Antigen zur Fehldiagnose einer HP führen, mit entsprechend negativen Auswirkungen auf das Outcome des Patienten. Wir berichten hier über die Fälle von drei Patientinnen im Alter von 61, 65 und 70 Jahren und einem Patienten im Alter von 43 Jahren mit einem als HP maskierten ASyS und analysieren die zugrunde liegenden Ursachen der Fehldiagnose mit dem Ziel, das Bewusstsein für die enge Zusammenarbeit zwischen Pneumologen und Rheumatologen zu schärfen.

Case Presentation A 77-year-old woman was referred to our interstitial lung disease unit. She presented with a history of progressive dyspnea on exertion and nonproductive, persistent cough over the previous year. She was diagnosed with Sjogren syndrome two years ago by a rheumatologist. In the context of Sjogren syndrome, she reported chronic xerostomia and xerophthalmia for the last 5 years. Her history was also notable for the presence of arterial hypertension and hypothyroidism. She denied the presence of shortness of breath, chest pain, arthralgia, muscle weakness, weight loss, night sweats, and fatigue. She reported exposure to house mold. There was no family history of respiratory diseases. The patient never smoked and denied alcohol consumption, illicit drug use, or any occupational exposures.

High-Resolution Computed Tomography (HRCT) plays a pivotal role in the diagnosis of Idiopathic Pulmonary Fibrosis (IPF). First, it establishes the presence of lung fibrosis. Second, it allows the recognition of specific patterns, namely typical and probable Usual Interstitial Pneumonia (UIP) pattern obviating the need for tissue confirmation in the appropriate clinical context.Acknowledging the extreme versatility of modern radiology and the heavy burden of knowledge the modern radiologist has to cope with, this review addresses the diagnostic pitfalls of honeycombing in IPF diagnosis. This review focuses on two areas: i) when honeycombing is actually present but there are other findings that should raise suspicion of an alternative diagnosis and ii) when honeycombing is misdiagnosed, focusing on the commonest radiographic patterns that are responsible for this confusion.It is pivotal to establish the actual presence of honeycombing. Even then, the distribution of honeycombing or the presence of other findings could be suggestive of alternative diagnoses. Reviewing older images can be extremely helpful in reaching the correct diagnosis.

Objectives: Data on COVID-19 in patients with interstitial lung disease are scarce and whether SARS-CoV-2 may trigger interstitial lung disease progression remains unknown. We aimed to analyze outcomes of COVID-19 in patients with systemic sclerosis–associated interstitial lung disease, including possible thoracic radiographic progression. Patients and Methods: All 43 patients with systemic sclerosis–associated interstitial lung disease followed in our center (mean ± SD, 55.2 ± 11.6 years, 36 female) with confirmed SARS-CoV2 infection up to 1 September 2022 were analyzed. Individual interstitial lung disease extent on high resolution CT (HRCT) performed before (up to 3 months) and after COVID-19 (2–5 months) was compared. Results: At SARS-CoV-2 infection, 9/43 patients were unvaccinated, whereas 5, 26, and 3 had received 2, 3, or 4 doses of an mRNA vaccine, respectively. Thirty-one patients were either on monotherapy with immunosuppressives (mycophenolate, n = 7; cyclophosphamide, n = 2; methotrexate, n = 10; tocilizumab, n = 7; rituximab, n = 1; etanercept, n = 1), or their combinations ( n = 3). Eight patients (20%), of whom four unvaccinated, required hospitalization for pneumonia and three (7%) died of acute respiratory failure ( n = 2, both unvaccinated) or cardiac arrest. Lack of vaccination was the only independent predictor for hospitalization (OR = 7.98, 95% CI: 1.25–51.09) and marginally for death (OR = 32.7, 95% CI: 0.97–1110.98), regardless of the presence of diffuse systemic sclerosis, interstitial lung disease extent greater than 20% or immunosuppressive treatment. In 22 patients with available HRCT pairs (vaccinated = 20), the interstitial lung disease extent before COVID-19 (20.4%± 17.8%) remained unchanged (22.4% ± 18.5%) in all but one patient. Conclusion: SARS-CoV-2 vaccination is of outmost importance for every systemic sclerosis patient with interstitial lung disease. COVID-19 does not seem to promote progression of systemic sclerosis–associated interstitial lung disease in vaccinated patients, but further studies are warranted.

The 2019 coronavirus disease (COVID-19) pandemic caused by the SARS-CoV-2 virus has affected virtually all aspects of patient care. Healthcare systems around the world are trying to simultaneously treat patients with COVID-19 infection, prepare for its long-term impacts, and manage patients with other acute and chronic diseases. There are multiple ways that the COVID-19 pandemic will directly affect patients with fibrotic interstitial lung disease (ILD), particularly given their common risk factors for poor outcomes. Major issues for patients with ILD will include restricted access to key components of the diagnostic process, new uncertainties in the use of common ILD pharmacotherapies, limited ability to monitor both disease severity and the presence of medication adverse effects, and significantly curtailed research activities. The purpose of this review is to summarize how COVID-19 has impacted key components of the diagnosis and management of fibrotic ILD as well as to provide strategies to mitigate these challenges. We further review major obstacles for researchers and identify priority areas for future ILD research related to COVID-19. Our goals are to provide practical considerations to support the care of patients with ILD during the COVID-19 pandemic and to provide a road map for clinicians caring for these patients during future infectious disease outbreaks.

Immune checkpoint inhibitors are novel agents that have been proved efficacious in a variety of cancer types, but they are associated with a unique set of organ-specific, immune-related adverse events. Among them, immune-related pneumonitis requires special attention because it is difficult to diagnose and potentially lethal. Accumulating real-world epidemiological data suggest that immune-related pneumonitis is more frequent than previously reported. Its diagnosis requires exclusion of other causes and assessment of radiographic features on high-resolution CT of the chest. Management of immune-related pneumonitis is based on the use of immunosuppressants. Future research should be focused on finding predictive biomarkers for immune-related pneumonitis as well as optimizing its management.

Abundant epidemiologic evidence supports an association between idiopathic pulmonary fibrosis (IPF) and lung cancer. Lung tumors in patients with IPF develop preferentially in the periphery immediately adjacent to fibrotic areas, with different histologic distribution and immunohistochemical features compared with non-IPF-associated lung tumors. In this context, evidence indicates that IPF and lung cancer share many pathogenic similarities including genetic and epigenetic markers. It has been suggested that specific germline mutations predispose toward both IPF and lung cancer, leading to imbalance between oncogenes and tumor suppressor genes and ultimately carcinogenesis within fibrotic lungs. Aberrant epigenetic regulation due to methylation, histone modifications, and mainly deregulation of common noncoding RNAs represents a possible pathogenic link between the two disease paradigms. Genetic and epigenetic alterations lead to abnormal activation of common transduction pathways, including Wnt/β-catenin and phosphoinositide 3-kinase/protein kinase B, mediating metaplasia and hyperproliferation in alveolar type II epithelial cells. Cellular transformations in the mesenchymal phenotype represent a common link between lung fibrosis and carcinogenesis. In this review we summarize current data on common cellular and molecular pathogenic mechanisms between IPF and lung cancer and highlight promising therapeutic targets for this disease combination.

Case Presentation A 63-year-old woman presented with a 2-year history of nonproductive cough. She denied the presence of shortness of breath, chest pain, arthralgia, muscle weakness, weight loss, night sweats, and fatigue. She was a never smoker and had a history of arterial hypertension and diabetes. There was no history of asthma, allergic rhinitis, nasal polyps, or gastroesophageal reflux disease, known malignancy, or collagen tissue disease. She reported exposure to house mold. There was no family history of respiratory diseases. The patient denied alcohol consumption, illicit drug use, occupational exposures, any recent travel, or exposure to TB.

Airway distensibility measurement indices might be useful for providing additional information in detecting airway wall pathology in asthmahttps://bit.ly/3ntNLya

Introduction: Treatment of interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) often includes systemic corticosteroids. Use of steroid-sparing agents is amenable to avoid potential side effects. Methods: Functional indices and high-resolution computed tomography (HRCT) patterns of patients with non-IPF ILDs receiving mycophenolate mofetil (MMF) with a minimum follow-up of 1 year were analyzed. Two independent radiologists and a machine learning software system (Imbio 1.4.2.) evaluated HRCT patterns. Results: Fifty-five (n = 55) patients were included in the analysis (male: 30 [55%], median age: 65.0 [95% CI: 59.7–70.0], mean forced vital capacity %predicted [FVC %pred.] ± standard deviation [SD]: 69.4 ± 18.3, mean diffusing capacity of lung for carbon monoxide %pred. ± SD: 40.8 ± 14.3, hypersensitivity pneumonitis: 26, connective tissue disease-ILDs [CTD-ILDs]: 22, other ILDs: 7). There was no significant difference in mean FVC %pred. post-6 months (1.59 ± 2.04) and 1 year (−0.39 ± 2.49) of treatment compared to baseline. Radiographic evaluation showed no significant difference between baseline and post-1 year %ground glass opacities (20.0 [95% CI: 14.4–30.0] vs. 20.0 [95% CI: 14.4–25.6]) and %reticulation (5.0 [95% CI: 2.0–15.6] vs. 7.5 [95% CI: 2.0–17.5]). A similar performance between expert radiologists and Imbio software analysis was observed in assessing ground glass opacities (intraclass correlation coefficient [ICC] = 0.73) and reticulation (ICC = 0.88). Fourteen patients (25.5%) reported at least one side effect and 8 patients (14.5%) switched to antifibrotics due to disease progression. Conclusion: Our data suggest that MMF is a safe and effective steroid-sparing agent leading to disease stabilization in a proportion of patients with non-IPF ILDs. Machine learning software systems may exhibit similar performance to specialist radiologists and represent fruitful diagnostic and prognostic tools.

Case Presentation A 58-year-old woman was referred to our department with a cough of 1 year duration; her condition was unresponsive to the administration of inhaled steroid and beta-2 agonists. She denied the presence of dyspnea, chest pain, or other extrapulmonary symptoms. She was a never-smoker with a negative medical history and no occupational or domestic exposures. There was no history of cancer, gastroesophageal reflux disease, asthma, allergic rhinitis, or other allergies.

Background: Telomere-related genes (TRG) mutations are detected in 30% of familial pulmonary fibrosis (FPF) and are also associated with personal/familial extra-pulmonary disease suggestive of short telomere syndrome (STS). Aim: to evaluate the prevalence of TRG mutations in a national cohort of interstitial lung disease (ILD) with suspected genetic predisposition in Greece and to define patients’ characteristics and impact on outcome.Methods: Genetic diagnoses made between December 2014-September 2019 in patients with FPF, pulmonary fibrosis at young age or personal and/or family extra-pulmonary disease suggestive of STS were analyzed. Results: 150 ILD patients, 73% male, age-at-diagnosis 67 years old, 33% non-smokers, 75% with definite/probable UIP or CPFE, 93% with dyspnea/cough, FVC% pred 75%, DLCO% pred 49% were tested; FPF was 65%, 19% young age, 21% personal and 17% family extra-pulmonary disease suggestive of STS; 21% were tested for more than 1 indication. TRG pathogenic variations were detected in 19 (13%): 13 in a familial, 3 in young age and 3 in extra-pulmonary disease suggestive of STS context as follows: TERT, TERC, RTEL1, PARN, NOP10, NHP2 in 8,5,2,2,1 and 1 respectively. No difference was detected in epidemiological-clinical-radiologic-functional characteristics between patients without and with mutations apart from age-at-diagnosis [69 vs 62 years, p

Introduction: Alpha1-antitrypsin (A1AT) deficiency is an autosomal co-dominant disease related to SERPINA-1 gene mutations predisposing to pulmonary emphysema and/or liver disease. Aim: to examine the genotype and phenotype of a Greek cohort of A1AT-deficiency patients. Methods: This multicenter study enrolled A1AT-deficiency patients under augmentation treatment from 8 centers in Greece. Results: 23 adult patients, 61% male, 17.4% non-smokers, diagnosed at a median age (IQR) of 47(42-58) years with A1AT levels of 0.24 (0.04-0.4) g/L were identified. Indications for A1AT measurement were severe obstructive pulmonary disease at the age Conclusion: In contrast with the literature, where Z and S are the most common A1AT-deficiency alleles, the present study reveals extremely rare deleterious variants in Greek A1AT-deficiency patients under augmentation treatment. Further examination of phenotype-genotype associations and outcome is expected to unveil their clinical significance in the future.

During the lockdown, IPF patients experienced lower than expected COVID-19, suggesting that simple measures for the winter months, such as face masking, clean hands, physical distancing could be adopted as promising advances in non-pharmacological therapyhttps://bit.ly/2ISi2EV

Aim To investigate the perspective of physicians treating chronic airway diseases on the importance of device and substance characteristics influencing the compliance of patients with chronic obstructive airways diseases. Objective We surveyed physicians’ perspective on the impact of device and substance characteristics on patients’ compliance. Methods This study was carried out by running a structured questionnaire, to a total of 144 physicians, conducting personal interviews and evaluating answers on a scale from 1 for most to 6 for least important influencing parameter. Results Overall, the most important parameters influencing patients’ compliance according to physicians’ perspective were rapid onset of action, type of inhalation device and duration of action. Adverse events were considered as the least important parameter. When COPD and asthma were examined separately, the most important parameters influencing compliance were rapid onset of action, ease of use and duration of action. Rapid onset of action was significantly more important in asthma than COPD. Conclusion Onset and duration of action and ease of use were classified higher as important parameters to increase patients’ compliance, according to physicians’ perspective.

Treatment with immunomodulators, such as intravenous immunoglobulin (IVIG), may attenuate inflammatory responses observed in the severe stages of acute respiratory distress syndrome (ARDS) caused by coronavirus disease 19 (COVID-19). We retrospectively evaluated the clinical courses of 12 COVID-19 patients who received IVIG at various stages of their illness, including within the first 72 h of clinical presentation, after initiation of mechanical ventilation, and after prolonged ventilation and ICU stay. The patients included 9 men and 3 women with a median age of 50 years (range 23–74), median Charlson Comorbidity Score of 2 (range 0–7), and median Acute Physiology and Chronic Health Evaluation Score of 13 (range 5–33) at the time of IVIG. The IVIG total dose ranged from 0.5 to 2.0 g/kg (median 1.25 g/kg) distributed over 1–4 daily doses. The most common regimen received was 0.5 g/kg daily for 3 days. The median time to IVIG administration was 9 days (range 0–48 days) after admission. The median time from first IVIG dose administration to hospital discharge was 14 days (range 3–48). The 5 patients who received IVIG ≤4 days of admission demonstrated a significantly shorter length of hospital stay after treatment (median 7 days, range 3–14 days) than the 7 patients who received it >7 days after admission (median 33 days, range 8–48 days, p = 0.03, Mann-Whitney U test). These cases demonstrate that IVIG may improve the clinical state of patients with moderate to severe COVID-19 infection. Despite very high illness severity scores, all patients survived hospital discharge. No thrombotic events occurred and IVIG was well tolerated, despite most cases demonstrating very elevated D-dimer suggestive of active intravascular fibrinolysis. We believe that IVIG warrants immediate clinical trial evaluation in COVID-19 to confirm its role as a mainstay treatment of moderate to severe COVID-19 infection as a means to reduce hospital stay and utilization of ICU resources, including mechanical ventilation, and potentially reduce mortality.

We report on a 70-year old woman with dyspnea, systemic lymphadenopathy and abnormal chest computed tomography (CT) findings. A complete laboratory testing as well as mediastinal tissue sampling via Endobronchial Ultrasound (EBUS)-guided Transbronchial Needle Biopsy (TBNB) did not reveal a definite diagnosis. After experiencing acute respiratory failure which led to intensive care unit, the patient underwent a cervical lymph node biopsy which revealed peripheral T-cell lymphoma not otherwise specified (PTCL-NOS). A CT-guided trans-thoracic lung biopsy was performed that showed involvement of the lung parenchyma in the context of PTCL-NOS. Lung involvement is a rare extra-nodal manifestation of PTCL. The imaging patterns of this lymphoma have not been well described. We conclude that the finding of crazy paving pattern is a rare manifestation of this disease. In patients with pre-existing lymphoma, lung involvement should be included in the differential due to high pre-test probability.

Background Nintedanib represents an antifibrotic compound able to slow down disease progression of patients with idiopathic pulmonary fibrosis (IPF). Objective To investigate the safety and efficacy of nintedanib in patients with IPF in a real-life setting. Methods This was a multicentre, retrospective, observational, real-life study for patients with IPF receiving nintedanib between October 2014 and October 2016. Results We identified 94 patients with IPF receiving nintedanib (72 males, mean age±SD: 73.8 ± 7.5, mean%FVC±SD = 68.1 ± 18.3, mean%DLCo±SD = 44.4 ± 14.5). Diarrhea (n = 52, 55.3%) was the most commonly reported adverse event. Twenty patients (21.2%) had to permanently discontinue nintedanib due to severe adverse events. In the 6-months follow-up, median decline in %FVC predicted and %DLCO predicted were 1.36 (95%Cl: 0 to 2.97) and 4.00 (95%Cl: 2.01 to 6.20), respectively, when deaths were censored and excluded from the analysis. At 12 months, mean%FVC±SD and mean%DLCo±SD were 64.5 ± 19.1 and 43.7 ± 15.4, respectively. With regards to mortality, 17 patients (18.1%) died over a study period of 730 days. Conclusion Nintedanib demonstrated an acceptable safety and efficacy profile in our real-world observational study. Prospective observational studies in the context of registries that collect well-defined supporting data over time are sorely needed to answer residual questions on drug's performance.

Background Pirfenidone is a novel anti-fibrotic drug that has shown efficacy in five randomized multicenter clinical trials enrolling patients with Idiopathic Pulmonary Fibrosis of mild-to-moderate disease severity. Scarce data supports the use of pirfenidone in IPF patients with more advanced disease. Objective To investigate the safety and efficacy profile of pirfenidone in IPF patients with severe lung function impairment. Patients and methods This was a retrospective study enrolling patients with advanced IPF (FVC%predicted Results Between September 2011 and March 2013, we identified 43 patients with severe IPF (baseline meanFVC%predicted±SD: 63.8 ± 20.3, meanDL CO %predicted: 27.3 ± 8.2), of mean age±SD: 66.3 + 9.7, 34 males (81%) that received pirfenidone (2.403 mg/daily) for one year. Pirfenidone treatment was associated with a trend towards decrease in functional decline compared to 6-months before treatment initiation but failed to show any benefit after one year of treatment (ΔFVC: −3.3 ± 4.6 vs 0.49 ± 11.4 and vs. −5.8 ± 11.8, p = 0.06 and p = 0.04, respectively and ΔDL CO : −13.3 ± 15.2 vs. −10.1 ± 16.6 and vs. 28.3 ± 19.2, p = 0.39 and p = 0.002, respectively). Gastrointestinal disorders (34.9%), fatigue (23.2%) and photosensitivity (18.6%) were the most common adverse events. Adverse events led to treatment discontinuation in 9 patients (20.9%) and dose reduction in 14 (32.5%). Conclusion Pirfenidone appears to be safe when administered in patients with advanced IPF. Pirfenidone efficacy in IPF patients with severe lung function impairment may diminish after 6 months of treatment.

Nintedanib is a tyrosine kinase inhibitor approved for the treatment of idiopathic pulmonary fibrosis (IPF). In a retrospective, real-world study across seven Greek hospitals, we evaluated the effectiveness and safety of nintedanib in routine clinical practice. Patients diagnosed with IPF, as per guideline criteria or multidisciplinary diagnosis, received nintedanib between January 2013 and January 2018. We evaluated 244 patients: mean±sd age 71.8±7.5 years, 79.1% male, 45.1% current smokers and 33.1% ex-smokers at treatment initiation. At baseline, predicted forced vital capacity (FVC) was 73.3±20.7% and predicted diffusing capacity of the lungs for carbon monoxide (DLCO) was 42.6±16.7%. On average, patients spent 23.6±15.0 months on nintedanib. At 3 years, 78 patients had died, equating to a 3-year survival rate of 59.4% (unaffected by treatment discontinuation or dose reduction). FVC% pred and DLCO% pred were largely stable at 3 years, with no significant difference from baseline (FVC 73.3±20.7% pred versus 78±20.1% pred, p=0.074; DLCO 42.6±16.7% pred versus 40.4±18.1% pred, p=0.334). Of the 244 patients, 55.7% reported an adverse event. Gastrointestinal events were the most common (173 (77.2%) out of 224 total events) and 45.0% of patients experienced diarrhoea. Only 32 (13.1%) patients had to permanently discontinue nintedanib due to an adverse event. This real-world study shows a 3-year survival rate of 59.4% and a low discontinuation rate due to adverse events. Our experience is consistent with previous findings in clinical trials of nintedanib in IPF., Findings from the largest registry of Greek patients with IPF receiving nintedanib in routine clinical practice show, over a 3-year period, a low discontinuation rate and efficacy data that support the results of the INPULSIS clinical trials http://bit.ly/35a2CS5

Introduction: Chronic Hypersensitivity Pneumonitis (c-HP) can exhibit an IPF-like course despite immunosuppressive treatment. In this group of patients there is a need for new therapeutic approaches. Aim: To investigate the effect of antifibrotics in the clinical course of c-HP. Patients and Methods: Retrospective analysis of patients with an initial diagnosis of IPF that were subsequently diagnosed as c-HP in the context of multidisciplinary discussion (Jan2012-Dec2016). HP diagnosis was based in the absence of alternative diagnoses when at least two of the following criteria were present: Recognition of an inciting antigen, compatible HRCT pattern (mainly presence of mosaic pattern), BAL lymphocytosis>20%. Results: We enrolled in the study 96 patients with HP. From this cohort we identified 18 patients, initially diagnosed as IPF (16 males, 88,9%, median age 70 years (95% CI for the median 63 to 73.6) that had received antifibrotics for at least 12 months (10 pirfenidone and 8 nintedanib). Mean (±SE) relative changes in %FVCpred and %DLcopred over one year was -4.19±3.29 and -5.72±5.32, respectively. A decline in FVC was observed in 11 patients (in 4 patients > 10%, and in 5 patients between 5-10%). An increase in relative %FVCpred was observed in 7 patients (mean ΔFVC ± SE = 8.69±3.75). Conclusions: Antifibrotics can stabilize FVC decline in patients with c-HP. Large randomized trials are needed.

Background: Abundant evidence supports an association between Idiopathic Pulmonary Fibrosis (IPF) and lung cancer development. Data on diagnosis and management of patients with IPF and lung cancer are still scarce. Aim: To estimate prevalence of lung cancer in patients with IPF. Patients and Methods: This was a retrospective multicenter study, enrolling 835 patients with IPF from eight different centers between 2009 and 2018 in Greece. Results: We identified 88 cases of patients with IPF and lung cancer (prevalence=10.5% n=88/835, mean age±SD=71.8±7.1, 82 males, meanFVC±SD= 73.5±18.9, meanDLCO±SD= 45.3±15.6). Six patients (7%) had both lung and other type of cancer. We identified 68 cases (77.3%) of non-small cell lung cancer (32 squamous, 25 adenocarcinoma, 11 non-specified), and 13 cases (14.8%) of small cell lung cancer. An equal distribution of anatomic location of lung cancer between upper and lower lobes was observed. Lung cancer was diagnosed post IPF diagnosis (median latency time=15.9 months) in 68% of patients and synchronously in 29.3% of patients. Chemotherapy was applied in 31.8% of cases, while 33.3% of patients underwent surgery. Median survival of patients with IPF and lung cancer was 27.4 months (95% CI: 20.6 to 36.8). Conclusions: IPF is a risk factor for lung cancer development. Squamous cell carcinoma is the most common histologic subtype in patients with IPF. Large randomized controlled studies on the management of patients with IPF and lung cancer are sorely needed.