Idiopathic Pulmonary Fibrosis Guidelines Research Articles

Variability of radiological and clinical features in cases with usual interstitial pneumonia without honeycombing

BackgroundUsual interstitial pneumonia (UIP) cases without honeycombing (possible UIP) included various CT features and was often difficult to diagnose. PurposeThis study aimed to classify the cases with possible UIP on CT features using cluster analysis and evaluate the features of subsets of participants and the correlation of prognosis. Materials and MethodsThe study included 85 patients with possible UIP in the 2011 idiopathic pulmonary fibrosis (IPF) guideline with radiological diagnosis. All cases underwent surgical biopsies and were diagnosed by multidisciplinary discussion (MDD) from the nationwide registry in Japan. The readers evaluated pulmonary opacity, nodules, cysts, and predominant distribution which were reclassified by IPF guidelines in 2018. Additionally, cases were classified into four groups by cluster analysis based on CT findings. The differences in survival among IPF classification and the clusters were evaluated. ResultsCases were diagnosed as IPF (n = 55), NSIP (n = 4), unclassifiable (n = 23), and others (n = 3) by MDD. Cluster analysis revealed 4 clusters by CT features (n = 47, 16, 19 and 3, respectively). Cluster 1 had fewer lesions overall. Cluster 2 have many pure ground-glass opacities and ground-glass opacities with reticulation. Cluster 3 had many reticular opacities and nodules with few lower predominant distributions. Cluster 4 was characterized by peribronchovascular consolidation.The mean survival time of cluster 1 (4518 days) was significantly better than cluster 2, 3, and 4 (1843, 2196, and 1814 days, respectively) (p = 0.03). ConclusionIn conclusion, UIP without honeycombing included various CT patterns and MDD diagnoses. Significangly differences in prognosis were observed among clusters classified by CT findings.

Clinical, radiological and histopathological features of patients with familial pulmonary fibrosis

BackgroundIn familial pulmonary fibrosis (FPF) at least two biological relatives are affected. Patients with FPF have diverse clinical features.Research questionWe aimed to characterize demographic and clinical features, re-evaluate high-resolution computed tomography (HRCT) scans and histopathology of surgical lung biopsies, assess survival and investigate the suitability of risk prediction models for FPF patients.Study designA retrospective cohort study.MethodsFPF data (n = 68) were collected from the medical records of Oulu University Hospital (OUH) and Oulaskangas District Hospital between 1 Jan 2000 and 11 Jan 2023. The inclusion criterion was pulmonary fibrosis (PF) (ICD 10-code J84.X) and at least one self-reported relative with PF. Clinical information was gathered from hospital medical records. HRCT scans and histology were re-evaluated.ResultsThirty-seven (54.4%) of the patients were men, and 31 (45.6%) were women. The mean ages of the women and men were 68.6 and 61.7 years, respectively (p = 0.003). Thirty-seven (54.4%) patients were nonsmokers. The most common radiological patterns were usual interstitial pneumonia (UIP) (51/75.0%), unclassifiable (8/11.8%) and nonspecific interstitial pneumonia (NSIP) (3/4.4%). Pleuroparenchymal fibroelastosis (PPFE) was observed as a single or combined pattern in 13.2% of the patients. According to the 2022 guidelines for idiopathic pulmonary fibrosis (IPF), the patients were categorized as UIP (31/45.6%), probable UIP (20/29.4%), indeterminate for UIP (7/10.3%) or alternative diagnosis (10/14.7%). The histopathological patterns were UIP (7/41.2%), probable UIP (1/5.9%), indeterminate for UIP (8/47.2%) and alternative diagnosis (1/5.9%). Rare genetic variants were found in 9 patients; these included telomerase reverse transcriptase (TERT, n = 6), telomerase RNA component (TERC, n = 2) and regulator of telomere elongation helicase 1 (RTEL1, n = 1). Half of the patients died (n = 29) or underwent lung transplantation (n = 5), with a median survival of 39.9 months. The risk prediction models composite physiology index (CPI), hazard ratio (HR) 1.07 (95.0% CI 1.04–1.10), and gender-age-physiology index (GAP) stage I predicted survival statistically significantly (p<0.001) compared to combined stages II and III.ConclusionsThis study confirmed the results of earlier studies showing that FPF patients’ radiological and histopathological patterns are diverse. Moreover, radiological and histological features revealed unusual patterns and their combinations.

Japanese guidelines for the treatment of idiopathic pulmonary fibrosis 2023:Revised edition

Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease with a poor prognosis and an unknown cause that generally progresses to pulmonary fibrosis and leads to irreversible tissue alteration. The "Guidelines for the treatment of idiopathic pulmonary fibrosis 2017," specializing in the treatment of IPF for the first time in Japan and presenting evidence-based standard treatment methods suited to the state of affairs in Japan, was published in 2017, in line with the 2014 version of "Formulation procedure for Minds Clinical Practice Guidelines."Because new evidence had accumulated, we formulated the “Guidelines for the treatment of Idiopathic Pulmonary Fibrosis 2023 (revised 2nd edition).” While keeping the revision consistent with the ATS/ERS/JRS/ALAT IPF treatment guidelines, new clinical questions (CQs) on pulmonary hypertension were added to the chronic stage, in addition to acute exacerbation and comorbid lung cancer, which greatly affect the prognosis but are not described in the ATS/ERS/JRS/ALAT IPF guidelines. Regarding the advanced stages, we additionally created expert consensus-based advice for palliative care and lung transplantation. The number of CQs increased from 17 in the first edition to 24. It is important that these guidelines be used not only by respiratory specialists but also by general practitioners, patients, and their families; therefore, we plan to revise them appropriately in line with ever-advancing medical progress.

Radiological usual interstitial pneumonia pattern is associated with two-year mortality in patients with idiopathic pulmonary fibrosis

IntroductionThe new diagnostic guidelines for idiopathic pulmonary fibrosis (IPF) did not rule out the possibility of combining the radiological patterns of usual interstitial pneumonia (UIP) and probable UIP, given the similar management and diagnostic capacity. However, the prognostic implications of these patterns have not been fully elucidated, with different studies showing heterogeneous results. We applied the new criteria to a retrospective series of patients with IPF, assessing survival based on radiological patterns, findings, and their extension. MethodsTwo thoracic radiologists reviewed high-resolution computed tomography images taken at diagnosis in 146 patients with IPF, describing the radiological findings and patterns. The association of each radiological finding and radiological patterns with two-year mortality was analysed. ResultsThe two-year mortality rate was 40.2% in IPF patients with an UIP radiological pattern versus 7.1% in those with probable UIP. Compared to the UIP pattern, probable UIP was protective against mortality, even after adjusting for age, sex, pulmonary function, and extent of fibrosis (hazard ratio (HR) 0.23, 95% confidence interval (CI) 0.06–0.99). Receiving antifibrotic treatment was also a protective factor (HR 0.51, 95%CI 0.27–0.98). Honeycombing (HR 3.62, 95%CI 1.27–10.32), an acute exacerbation pattern (HR 4.07, 95%CI 1.84–8.96), and the overall extent of fibrosis (HR 1.04, 95%CI 1.02–1.06) were predictors of mortality. ConclusionsIn our series, two-year mortality was higher in patients with IPF who presented a radiological pattern of UIP versus probable UIP on the initial scan. Honeycombing, an acute exacerbation pattern, and a greater overall extent of fibrosis were also predictors of increased mortality. The prognostic differences between the radiological pattern of UIP and probable UIP in our series would support maintaining them as two differentiated patterns.

The Evolving Concept of the Multidisciplinary Approach in the Diagnosis and Management of Interstitial Lung Diseases.

Interstitial lung diseases (ILDs) are a group of heterogeneous diseases characterized by inflammation and/or fibrosis of the lung interstitium, leading to a wide range of clinical manifestations and outcomes. Over the years, the literature has demonstrated the increased diagnostic accuracy and confidence associated with a multidisciplinary approach (MDA) in assessing diseases involving lung parenchyma. This approach was recently emphasized by the latest guidelines from the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Association for the diagnosis of ILDs. In this review, we will discuss the role, composition, and timing of multidisciplinary diagnosis (MDD) concerning idiopathic pulmonary fibrosis, connective tissue disease associated with ILDs, hypersensitive pneumonia, and idiopathic pneumonia with autoimmune features, based on the latest recommendations for their diagnosis. The integration of clinical, radiological, histopathological, and, often, serological data is crucial in the early identification and management of ILDs, improving patient outcomes. Based on the recent endorsement of transbronchial cryo-biopsy in idiopathic pulmonary fibrosis guidelines, an MDA helps guide the choice of the sampling technique, obtaining the maximum diagnostic performance, and avoiding the execution of more invasive procedures such as a surgical lung biopsy. A multidisciplinary team should include pulmonologists, radiologists, pathologists, and, often, rheumatologists, being assembled regularly to achieve a consensus diagnosis and to review cases in light of new features. The literature highlighted that an MDA is essential to improve the accuracy and reliability of ILD diagnosis, allowing for the early optimization of therapy and reducing the need for invasive procedures. The multidisciplinary diagnosis of ILDs is an ongoing and dynamic process, often referred to as a "working diagnosis", involving the progressive integration and re-evaluation of clinical, radiological, and histological features.

Pulmonary Pathology Society Survey on Practice Approaches in the Histologic Diagnosis of Fibrotic Interstitial Lung Disease: Consensus and Opportunities.

The pathologic diagnosis of usual interstitial pneumonia (UIP) remains a challenging area, and application of histologic UIP guidelines has proved difficult. To understand current practice approaches by pulmonary pathologists for the histologic diagnosis of UIP and other fibrotic interstitial lung diseases (ILDs). The Pulmonary Pathology Society (PPS) ILD Working Group developed and sent a 5-part survey on fibrotic ILD electronically to the PPS membership. One hundred sixty-one completed surveys were analyzed. Of the respondents, 89% reported using published histologic features in clinical guidelines for idiopathic pulmonary fibrosis (IPF) in their pathologic diagnosis; however, there was variability in reporting terminology, quantity and quality of histologic features, and the use of guideline categorization. Respondents were very likely to have access to pulmonary pathology colleagues (79%), pulmonologists (98%), and radiologists (94%) to discuss cases. Half of respondents reported they may alter their pathologic diagnosis based on additional clinical and radiologic history if it is pertinent. Airway-centered fibrosis, granulomas, and types of inflammatory infiltrates were considered important, but there was poor agreement on how these features are defined. There is significant consensus among the PPS membership on the importance of histologic guidelines/features of UIP. There are unmet needs for (1) consensus and standardization of diagnostic terminology and incorporation of recommended histopathologic categories from the clinical IPF guidelines into pathology reports, (2) agreement on how to incorporate into the report relevant clinical and radiographic information, and (3) defining the quantity and quality of features needed to suggest alternative diagnoses.

New Guidelines for Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is the most common and fatal idiopathic interstitial pneumonia and is characterized by chronic progressive pulmonary fibrosis of indeterminate etiology. In 2018, the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society published joint clinical practice guidelines for IPF. The guidelines require exclusion of known causes of interstitial lung disease and identification of a radiological and/or pathologic pattern of usual interstitial pneumonia (UIP). The diagnosis of IPF is a multidisciplinary process, involving pulmonologists, radiologists, pathologists and, if necessary, experts in other medical fields. The 2022 guidelines for IPF revisit and clarify the radiological and pathological features of UIP. In addition, recommendations regarding transbronchial lung cryobiopsy, antacid medication, and antireflux surgery are revised or established based on up-to-date evidence in the 2022 guidelines. The new guidelines also encompass the definition and treatment of progressive pulmonary fibrosis (PPF). PPF comprises diverse fibrotic interstitial lung diseases other than IPF, which progress despite standard treatment. The diagnosis of PPF is based on symptoms, physiologic evidence, and radiologic evidence of progression. The use of nintedanib was suggested for patients with PPF other than IPF who are unresponsive to standard treatment. This review introduces and discusses the recommendations for the diagnosis and treatment of IPF and PPF in the new international guidelines.

Evaluating the consistency with guideline recommendations for diagnosis and management of idiopathic pulmonary fibrosis in non-academic settings.

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with elevated mortality. Delay in diagnosis lead to worse outcomes. Guidelines developed at academic medical centers are difficult to replicate in the community. Our primary objective was to ascertain consistency with the 2011 IPF guidelines. Our secondary objective was to conduct an interdisciplinary review to ascertain whether the evidence supported the original diagnosis of IPF or not. We asked permission from pulmonologists to review records of patients diagnosed with IPF after 2011. We collected physician demographics and training data; patient demographics, clinical and diagnostic/management data. The clinical data and available images were reviewed by the interdisciplinary review panel. 26 practicing pulmonologists located in the Southeast of the United States consented to participate. Mean age was 48, 70% were male and all had current certification. We reviewed data from 96 patients. The mean age was 71.4 and most were male. Only 23% had the recommended screening for a connective tissue disease and 42.6% were screened for exercise-induced hypoxemia. Among patients with available images for review (n=66), only 50% had a high-resolution CT scan. 22% of patients underwent a surgical biopsy and in only 33% of the cases three lobes were sampled. No patient had documentation that a multidisciplinary discussion occurred. In 20% of the cases with available images, the evidence supported an alternative diagnosis. 56% of eligible candidates were ever started on anti-fibrotics. Our findings suggest that consistency with the IPF guidelines is low in non-academic settings.

2022 update on clinical practice guidelines for idiopathic pulmonary fibrosis and progressive pulmonary fibrosis

The 2022 update on guidelines for idiopathic pulmonary fibrosis (IPF) by ATS/ERS/JRS/ALAT1 consists of two parts. The first part is an update on the diagnosis and treatment of IPF and the second deals with the important issue of progressive pulmonary fibrosis (panel).

Predicting Usual Interstitial Pneumonia Histopathology From Chest CT Imaging With Deep Learning

Idiopathic pulmonary fibrosis (IPF) is a progressive, often fatal form of interstitial lung disease (ILD) characterized by the absence of a known cause and usual interstitial pneumonitis (UIP) pattern on chest CT imaging and/or histopathology. Distinguishing UIP/IPF from other ILD subtypes is essential given different treatments and prognosis. Lung biopsy is necessary when noninvasive data are insufficient to render a confident diagnosis. Can we improve noninvasive diagnosis of UIP be improved by predicting ILD histopathology from CT scans by using deep learning? This study retrospectively identified a cohort of 1,239 patients in a multicenter database with pathologically proven ILD who had chest CT imaging. Each case was assigned a label based on histopathologic diagnosis (UIP or non-UIP). A custom deep learning model was trained to predict class labels from CT images (training set, n=894) and was evaluated on a 198-patient test set. Separately, two subspecialty-trained radiologists manually labeled each CT scan in the test set according to the 2018 American Thoracic Society IPF guidelines. The performance of the model in predicting histopathologic class was compared against radiologists' performance by using area under the receiver-operating characteristic curve as the primary metric. Deep learning model reproducibility was compared against intra-rater and inter-rater radiologist reproducibility. For the entire cohort, mean patient age was 62 ± 12 years, and 605 patients were female (49%). Deep learning performance was superior to visual analysis in predicting histopathologic diagnosis (area under the receiver-operating characteristic curve, 0.87 vs0.80, respectively; P< .05). Deep learning model reproducibility was significantly greater than radiologist inter-rater and intra-rater reproducibility (95%CI for difference in Krippendorff's alpha did not include zero). Deep learning may be superior to visual assessment in predicting UIP/IPF histopathology from CT imaging and may serve as an alternative to invasive lung biopsy.

Anti-Myxovirus Resistance Protein-1 Immunoglobulin A Autoantibody in Idiopathic Pulmonary Fibrosis.

Background We have previously analysed serum autoantibody levels in patients with idiopathic pulmonary fibrosis (IPF), idiopathic nonspecific interstitial pneumonia (iNSIP), and healthy controls and identified the autoantibody against anti-myxovirus resistance protein-1 (MX1) to be a specific autoantibody in iNSIP. We found that a higher anti-MX1 autoantibody level was a significant predictor of a good prognosis in patients with non-IPF idiopathic interstitial pneumonias. In this retrospective study, we sought to clarify the prognostic significance of the anti-MX1 autoantibody in IPF. Methods We measured anti-MX1 immunoglobulin (Ig) G, IgA, and IgM autoantibody levels by enzyme-linked immunosorbent assay in serum collected at the time of diagnosis from 71 patients with IPF diagnosed according to the 2018 IPF guideline. The gender-age-physiology (GAP) index was calculated in each case. Results The study population (59 men and 12 women) had a median age of 67 years. Serum anti-MX1 IgG and IgA autoantibody levels correlated positively with GAP stage (p < 0.05). Univariate Cox proportional hazards regression analysis did not identify an elevated anti-MX1 IgG, IgA, or IgM autoantibody level as a significant prognostic factor; however, a higher anti-MX1 IgA autoantibody level heralded significantly poorer survival after adjustment for GAP stage (p=0.030) and for percent forced vital capacity and modified Medical Research Council score (p=0.018). Neither the anti-MX1 IgG autoantibody nor the IgM autoantibody could predict survival after these adjustments. Conclusions The serum anti-MX1 IgA autoantibody level is a significant prognostic factor in IPF. Further studies are needed to clarify the pathophysiological role of this autoantibody in IPF.

The Interaction Between Pulmonary Fibrosis and COVID-19 and the Application of Related Anti-Fibrotic Drugs.

COVID-19 is a highly contagious respiratory disease, which mainly affects the lungs. Critically ill patients are easily complicated by cytokine storms, acute respiratory distress syndrome (ARDS), and respiratory failure, which seriously threaten their lives. Pulmonary fibrosis (PF) is a common interstitial lung disease, and its pathogenesis may involve the participation of a variety of immune cells and inflammatory factors. Current studies have shown that patients with COVID-19 may be complicated by pulmonary fibrosis, and patients with pulmonary fibrosis may also be at higher risk of contracting COVID-19 than healthy people. Pulmonary fibrosis is an important risk factor leading to the aggravation of COVID-19 disease. COVID-19 complicated by cytokine storm and ARDS mechanism pathways are similar to the pathogenesis of pulmonary fibrosis. The potential interaction between pulmonary fibrosis and COVID-19 can cause acute exacerbation of the patient’s condition, but the potential mechanism between the two has not been fully elucidated. Most of the drug treatment programs for COVID-19-related pulmonary fibrosis are currently formulated about the relevant guidelines for idiopathic pulmonary fibrosis (IPF), and there is no clear drug treatment program recommendation. This article aims to summarize the relevant mechanism pathways of COVID-19 and pulmonary fibrosis, explore the interrelationships and possible mechanisms, and discuss the value and risks of existing and potential COVID-19-related pulmonary fibrosis treatment drugs, to provide reference for anti-fibrosis treatment for patients.

Prognostic value of radiological findings indeterminate for UIP pattern and anterior upper lobe honeycomb-like lesion in chronic fibrosing interstitial lung disease associated with MPO-ANCA

BackgroundMyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) is often positive in patients with interstitial lung disease (ILD), which is also often present in patients with microscopic polyangiitis (MPA). A possible association between MPO-ANCA, MPA, and idiopathic ILD remains unclear. The objective of this study was to determine whether high-resolution computed tomography (HRCT) classification based on recent idiopathic pulmonary fibrosis guideline and specific CT findings can obtain new knowledge of prognostic factors in all MPO-ANCA-positive patients with ILD including both idiopathic ILD and MPA-ILD.MethodsWe analyzed 101 consecutive MPO-ANCA-positive patients with respiratory disease. We assessed the diagnostic accuracy of CT findings, HRCT pattern, and specific radiological signs. Prognostic predictors were determined using Cox regression models.ResultsSubjects with chronic ILD included 22 patients with MPA-ILD and 39 patients with ILD but without MPA. A quarter of the patients were radiological indeterminate for usual interstitial pneumonia (UIP) pattern, which resulted in a better prognosis than that for UIP pattern. “Increased attenuation around honeycomb and traction bronchiectasis” and “anterior upper lobe honeycomb-like lesion” were found to be highly frequent radiological findings (39% and 30%, respectively). In addition, the latter finding was a significant negative prognostic factor.ConclusionsRadiological indeterminate for UIP was a useful HRCT classification in MPO-ANCA-positive patients with ILD. In addition, anterior upper lobe honeycomb-like lesion was found to be specific radiological finding that was a significant prognostic factor. The present results might aid in the assessment of appropriate strategies of diagnosis in these patients.

Implementation of guideline recommendations and outcomes in patients with idiopathic pulmonary fibrosis: Data from the IPF-PRO registry

BackgroundFew data are available on the extent to which clinical practice is aligned with international guidelines for the management of idiopathic pulmonary fibrosis (IPF). We investigated the extent to which management guidelines for IPF have been implemented in the US IPF-PRO Registry and associations between implementation of guidelines and clinical outcomes. MethodsWe assessed the implementation of eight recommendations in clinical practice guidelines within the 6 months after enrollment: visit to a specialized clinic; pulmonary function testing; use of oxygen in patients with resting hypoxemia and exercise-induced hypoxemia; referral for pulmonary rehabilitation; treatment of gastro-esophageal reflux disease; initiation of anti-fibrotic therapy; referral for lung transplant evaluation. An implementation score was calculated as the number of recommendations achieved divided by the number for which the patient was eligible. Associations between implementation score and outcomes were analyzed using logistic regression and Cox proportional hazards models. ResultsAmong 727 patients, median (Q1, Q3) implementation score was 0.6 (0.5, 0.8). Patients with an implementation score >0.6 had greater disease severity than those with a lower score. Implementation was lowest for referral for pulmonary rehabilitation (19.5%) and lung transplant evaluation (22.3%). In unadjusted models, patients with higher implementation scores had a greater risk of death, death or lung transplant, and hospitalization, but no significant associations were observed in adjusted models. ConclusionsManagement guidelines were more likely to be implemented in patients with IPF with greater disease severity. When adjusted for disease severity, no association was found between implementation of management guidelines and clinical outcomes.

Social Media Content of Idiopathic Pulmonary Fibrosis Groups and Pages on Facebook: Cross-sectional Analysis.

BackgroundPatients use Facebook as a resource for medical information. We analyzed posts on idiopathic pulmonary fibrosis (IPF)-related Facebook groups and pages for the presence of guideline content, user engagement, and usefulness.ObjectiveThe objective of this study was to describe and analyze posts from Facebook groups and pages that primarily focus on IPF-related content.MethodsCross-sectional analysis was performed on a single date, identifying Facebook groups and pages resulting from separately searching “IPF” and “idiopathic pulmonary fibrosis.” For inclusion, groups and pages needed to meet either search term and be in English, publicly available, and relevant to IPF. Every 10th post was assessed for general characteristics, source, focus, and user engagement metrics. Posts were analyzed for presence of IPF guideline content, useful scientific information (eg, scientific publications), useful support information (eg, information about support groups), and potentially harmful information.ResultsEligibility criteria were met by 12 groups and 27 pages, leading to analysis of 523 posts. Of these, 42% contained guideline content, 24% provided useful support, 20% provided useful scientific information, and 5% contained potentially harmful information. The most common post source was nonmedical users (85%). Posts most frequently focused on IPF-related news (29%). Posts containing any guideline content had fewer likes or comments and a higher likelihood of containing potentially harmful content. Posts containing useful supportive information had more likes, shares, and comments.ConclusionsFacebook contains useful information about IPF, but posts with misinformation and less guideline content have higher user engagement, making them more visible. Identifying ways to help patients with IPF discriminate between useful and harmful information on Facebook and other social media platforms is an important task for health care professionals.

Critical appraisal of the quality of clinical practice guidelines for idiopathic pulmonary fibrosis.

BackgroundClinical practice guidelines (CPGs) have long served as an essential tool for clinicians to rationalize their treatment in practice. However, the quality of guidelines varies greatly. The present study aimed to analyze high-quality CPGs of idiopathic pulmonary fibrosis (IPF) and highlight the potential for further improvement.MethodsThree guideline developers’ websites, PubMed, Embase, and Web of Science, as well as a public search engine, Google Scholar, were searched to retrieve CPGs regarding the management of IPF. The methodology and reporting quality of retrieved CPGs were assessed using the validated Appraisal of Guidelines for Research and Evaluation Instrument II (AGREE II) and Reporting Items for Practice Guidelines in Healthcare (RIGHT) checklist.ResultsTwelve IPF CPGs were reviewed, among which 7 (58.3%) were considered as “recommended” and 1 (8.3%) as “recommended with modifications”. Among the 6 domains of AGREE II, scope and purpose (70.99%) and clarity of presentation (68.06%) were considered to be the fields in which CPGs performed best, evidenced by the highest mean AGREE II scores. The domains in which the reviewed CPGs received the lowest mean scores were rigor of development (50.87%) and applicability (34.14%). The intraclass correlation coefficient scores were excellent in each domain. The basic information domain received the highest overall reporting rate in the 7 domains of the RIGHT checklist; the other 6 domains had a full reporting rate of <50%. Eight items had a satisfactory level of reporting, whereas 14 items had poor reporting according to the RIGHT checklist. Correlation analysis revealed a highly positive correlation between the methodology and reporting quality of CPGs for IPF (r=0.872).ConclusionsThe methodological quality of selected IPF CPGs fluctuated greatly, and the full reporting rate was found to be quite low in some domains. In the future, we should focus not only on improving the methodological quality in the development of guidelines, but also on the reporting quality of guidelines.

Usual Interstitial Pneumonia in Contemporary Surgical Pathology Practice: Impact of International Consensus Guidelines for Idiopathic Pulmonary Fibrosis on Pathologists.

Idiopathic pulmonary fibrosis is a clinical syndrome characterized by the presence of usual interstitial pneumonia (UIP) radiologically and pathologically. Per consensus criteria adopted in 2011, diagnosis of idiopathic pulmonary fibrosis no longer requires a biopsy in an appropriate context if UIP is seen on imaging. As a result, lung biopsies are now typically reserved for patients having indeterminate clinical or imaging findings or suspicion for alternative diagnoses, but the impact of updated guidelines on pathology practice remains unclear. To determine the frequency of histologic UIP before and after 2011. Surgical lung biopsies from adults were studied within two 4-year periods: July 1, 2006 through June 30, 2010 and January 1, 2012 through December 31, 2015. Pathology slides were reviewed in a fashion blinded to clinical information and were classified using current guidelines. Biopsies from 177 and 86 patients (mean [SD] age, 62 [12] and 59 [14] years; 50.3% [89 of 177] and 48.8% [42 of 86] men) before and after 2011, respectively, were reviewed. Probable UIP or UIP was less-frequently encountered after 2011 in all patients with fibrosis (9 of 54 [16.7%] versus 41 of 119 [34.5%] before 2011, P = .02) and also in patients 50 years old and older (8 of 46 [17.4%] versus 39 of 109 [35.8%] before 2011, P = .02), with a concomitant rise in cases indeterminate for UIP or showing alternative diagnoses. Histology for UIP is less frequently encountered in our contemporary practice compared with the historic era. The pretest probability of a non-UIP diagnosis is now high, even in elderly patients, underscoring the need for pathologists to be familiar with the histologic features of alternative diagnoses.

Translating Idiopathic pulmonary fibrosis guidelines into clinical practice

Introduction and objectivesIdiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic interstitial lung disease of unknown cause, which predominantly manifests in older males. IPF diagnosis is a complex, multi-step process and delay in diagnosis cause a negative impact on patient survival. Additionally, a multidisciplinary team of pulmonologists, radiologists and pathologists is necessary for an accurate IPF diagnosis. The present study aims to assess how diagnosis and treatment of IPF are followed in Portugal, as well as the knowledge and implementation of therapeutic guidelines adopted by the Portuguese Society of Pulmonology. Materials and methodsSeventy-eight practicing pulmonologists were enrolled (May–August 2019) in a survey developed by IPF expert pulmonologists comprised of one round of 31 questions structured in three parts. The first part was related to participant professional profile, the second part assessed participant level of knowledge and practice agreement with national consensus and international guidelines for IPF as well as their access to radiology and pathology for IPF diagnosis, and the third part was a self-evaluation of the guidelines adherence for diagnosis and treatment in their daily practice. ResultsParticipants represented a wide spectrum of pulmonologists from 14 districts of Portugal and autonomous regions of Azores and Madeira. The majority were female (65%), with 5–19 years of experience (71%) and working in a public clinical center (83%). Importantly, the majority of pulmonologists follow their IPF patients (n=45) themselves, while 26% referred IPF patients to ILD experts in the same hospital and 22% to another center. Almost all pulmonologists (98%) agreed or absolutely agreed that multidisciplinary discussion is recommended to accurately diagnose IPF. No pulmonologists considered pulmonary biopsy as absolutely required to establish an IPF diagnosis. However, 87% agreed or absolutely agree with considering biopsy in a possible/probable UIP context. If a biopsy is necessary, 96% of pulmonologists absolutely agree or agree with considering criobiopsy as an option for IPF diagnosis. Regarding IPF treatment, 98% absolutely agreed or agreed that antifibrotic therapy should be started once the IPF diagnosis is established. Finally, 76% stated that 6 months is the recommended time for follow-up visit in IPF patients. ConclusionsPortuguese pulmonologists understand and agree with national consensus and international guidelines for IPF treatment but their implementation in Portugal is heterogeneous.

German Guideline for Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since publication of the international IPF guideline in the year 2011 and Update 2018 several studies and technical advances occurred, which made a new assessment of the diagnostic process mandatory. In view of the antifibrotic drugs which have been approved for the treatment of IPF patients, the goal of this guideline is to foster early, confident and effective diagnosis of IPF. The guideline focusses on the typical clinical setting of an IPF patient and provides tools to exclude known causes of interstitial lung disease including standardised questionnaires, serologic testing and cellular analysis of bronchoalveolar lavage. High resolution computed tomography remains crucial in the diagnostic work-up. If it is necessary to obtain specimen for histology transbronchial lung cryobiopsy is the primary approach, while surgical lung biopsy is reserved for patients who are fit for it and in whom bronchoscopic diagnosis did not provide the information needed. Despite considerable progress, IPF remains a diagnosis of exclusion and multidisciplinary discussion remains the golden standard of diagnosis.

Idiopathic pulmonary fibrosis: Significance of the usual interstitial pneumonia (UIP) CT-scan patterns defined in new international guidelines

IntroductionThe new 2018 international guidelines for diagnosing usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF) by CT scan split the old pattern possible UIP (2011 IPF guidelines) into two new patterns: probable UIP and indeterminate for UIP. However, the proportions and prognoses of these new CT-scan patterns are not clear. MethodsWe used a monocentric retrospective cohort of 322 patients suspected of having IPF (University Hospital of Rennes; Competence Center for Rare Lung Diseases; 1 January 2012-31 December 2017). All patients initially diagnosed by CT scan as possible UIP were included. The chest CT-scans were then reclassified according to the new 2018 international guidelines by 3 observers. These data were then subjected to survival analysis with multivariate Cox regression using a composite endpoint of death, lung transplantation, a decline of≥10% in forced vital capacity (FVC), or hospitalization. ResultsOf the 89 possible UIP patients included, 74 (83%) were reclassified as probable UIP and 15 (17%) as indeterminate for UIP. Probable UIP patients were more likely to meet the composite endpoint (56/74 [75.7%] vs. 5/15 [33%] patients; HR [IC 95%] =3.12 [1.24; 7.83], P=0.015). Multivariate analysis indicated that the probable UIP pattern was associated with significantly increased risk of reaching the composite endpoint (HR [95% CI]=2.85[1.00; 8.10], P=0.049). ConclusionThe majority of possible UIP diagnoses corresponded to probable UIP, which was associated with a significantly worse prognosis than indeterminate for UIP. This distinction between these two CT patterns emphasizes the relevance of the new international guidelines for the diagnosis of IPF.