Pulmonary Pathology Society Perspective on the 2018 American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society Idiopathic Pulmonary Fibrosis Clinical Practice Guidelines

2020 ◽  
Vol 17 (5) ◽  
pp. 550-554 ◽  
Author(s):  
Lida P. Hariri ◽  
Maxwell L. Smith ◽  
Mari Mino-Kenudson ◽  
Timothy Craig Allen ◽  
Richard Attanoos ◽  
...  
2020 ◽  
Vol 144 (12) ◽  
pp. 1477-1489
Author(s):  
Maxwell L. Smith ◽  
Lida P. Hariri ◽  
Mari Mino-Kenudson ◽  
Sanja Dacic ◽  
Richard Attanoos ◽  
...  

Context.— Accurate diagnosis of idiopathic pulmonary fibrosis (IPF) requires multidisciplinary diagnosis that includes clinical, radiologic, and often pathologic assessment. In 2018, the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and the Latin American Thoracic Society (ATS/ERS/JRS/ALAT) and the Fleischner Society each published guidelines for the diagnosis of IPF, which include criteria for 4 categories of confidence of a histologic usual interstitial pneumonia (UIP) pattern. Objective.— To (1) identify the role of the guidelines in pathologic assessment of UIP; (2) analyze the 4 guideline categories, including potential areas of difficulty; and (3) determine steps the Pulmonary Pathology Society and the greater pulmonary pathology community can take to improve current guideline criteria and histopathologic diagnosis of interstitial lung disease. Data Sources.— Data were derived from the guidelines, published literature, and clinical experience. Conclusions.— Both guidelines provide pathologists with a tool to relay to the clinician the likelihood that a biopsy represents UIP, and serve as an adjunct, not a replacement, for traditional histologic diagnosis. There are multiple challenges with implementing the guidelines, including (1) lack of clarity on the quantity and quality of histologic findings required, (2) lack of recognition that histologic features cannot be assessed independently, and (3) lack of guidance on how pathologists should incorporate clinical and radiographic information. Current criteria for “probable UIP” and “indeterminate for UIP” hinder accurate reflection of the likelihood of IPF. These challenges highlight the need for further morphologic-based investigations in the field of pulmonary pathology.


2021 ◽  
Vol 29 (3) ◽  
pp. 5-23
Author(s):  
Y. I. Feshchenko ◽  
◽  
V. K. Gavrysyuk ◽  
N. G. Gorovenko ◽  
Y. A. Dziublyk ◽  
...  

EVOLUTION OF PRINCIPLES OF DIAGNOSIS AND TREATMENT OF IDIOPATHIC PULMONARY FIBROSIS IN THE INTERNATIONAL GUIDELINE STATEMENTS Y. I. Feshchenko, V. K. Gavrysyuk, N. G. Gorovenko, Y. A. Dziublyk, I. V. Liskina Abstract Idiopathic pulmonary fibrosis (IPF) is specific form of chronic progressive interstitial fibrosing pneumonia of unknown nature, mainly occurring in patients > 50 years of age, limited to the lungs and associated with histological and/or radiological pattern of usual interstitial pneumonia. Epidemiological studies estimate that prevalence of ILF in different countries varies between 1,25 and 63 cases per 100 000 persons. Along with that, ILF is characterized by unfavorable prognosis — median survival time ranges within 2,5−3,5 years from the time of diagnosis. In 2000 American thoracic society (ATS) and European respiratory society (ERS) published first international statement on diagnosis and treatment of ILF — American Thoracic Society, European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Data from studies, accumulated during next 10 years of research, determined the necessity of update of certain diagnostic criteria and principles of therapy. In this regard, a new guideline for diagnosis and treatment of IPF was published in 2011 and approved by ATS, ERS, Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT) — An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. A new update on “Treatment of IPF” was published in 2015, and chapter “Diagnosis” was updated later in 2018. Current literature review focuses on the principles and algorithms of IPF treatment and the changes in guidelines, occurred from the time of first Statement published. Summarizing review results, we can conclude, that evolution of diagnostics principles, which limited the indications for surgical lung biopsy for the purpose of diagnosis verification, is caused by explosive technological advances in the field of chest computed tomography. The capability of computed tomography has grown to such an extent, that in terms of morphological diagnosing this method can compete with histological examination. The changes in management principles were evoked by the revision of IPF pathogenesis mechanisms. Since 2000, most of the experts concluded, that fibrosis, rather than inflammation, was the leading link of pathogenesis. A fibrosing process, initially playing a reparative role, further due to unknown reasons, gains an uncontrolled progressive character. Accordingly, antifibrotic compounds, such as pirfenidone and nintedanib, came to substitute glucocorticosteroids, known for their powerful antiinflammatory potential. The publication also presents the review of most relevant randomized clinical trials on safety and efficacy of antifibrotic drugs — pirfenidone and nintedanib. Key words: idiopathic pulmonary fibrosis, diagnostics, treatment, algorithms, pirfenidone, nintedanib. Ukr. Pulmonol. J. 2021;29(3):5–23.


2020 ◽  
Vol 8 (21) ◽  
pp. 1405-1405
Author(s):  
Xuanlin Li ◽  
Xueqing Yu ◽  
Yang Xie ◽  
Zhenzhen Feng ◽  
Yanfang Ma ◽  
...  

2016 ◽  
Vol 140 (12) ◽  
pp. 1375-1382 ◽  
Author(s):  
Mikiko Hashisako ◽  
Tomonori Tanaka ◽  
Yasuhiro Terasaki ◽  
Toshimasa Uekusa ◽  
Rosane D. Achcar ◽  
...  

Context.— The histopathologic criteria for idiopathic pulmonary fibrosis were revised in the American Thoracic Society/European Respiratory Society/Japan Respiratory Society/Latin American Thoracic Association guidelines in 2011. However, the evidence of diagnosis based on the guidelines needs further investigation. Objective.— To examine whether the revised histopathologic criteria for idiopathic pulmonary fibrosis improved interobserver agreement among pathologists and the predicted prognosis in patients with interstitial pneumonia. Design.— Twenty, consecutive, surgical lung-biopsy specimens from cases of interstitial pneumonia were examined for histologic patterns by 11 pathologists without knowledge of clinical and radiologic data. Diagnosis was based on American Thoracic Society/European Respiratory Society guidelines of 2002 and 2011. Pathologists were grouped by cluster analysis, and interobserver agreement and association to the patient prognosis were compared with the diagnoses for each cluster. Results.— The generalized κ coefficient of diagnosis for all pathologists was 0.23. If the diagnoses were divided into 2 groups: usual interstitial pneumonia (UIP)/probable UIP (the UIP group) or possible/not UIP (the non-UIP group), according to the 2011 guidelines, the κ improved to 0.37. The pathologists were subdivided into 2 clusters in which 1 showed an association between UIP group diagnosis and patient prognosis (P < .05). Conclusions.— Agreement about pathologic diagnosis of interstitial pneumonia is low; however, results after division into UIP and non-UIP groups provided favorable agreement. The cluster analysis revealed 1 of the 2 clusters providing high interobserver agreement and prediction of patient prognosis.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 982.2-982
Author(s):  
C. Aguilera Cros ◽  
M. Gomez Vargas ◽  
R. J. Gil Velez ◽  
J. A. Rodriguez Portal

Background:There is no specific treatment for interstitial lung disease (ILD) secondary to Rheumatoid Arthritis (RA) other than the treatment of RA without extra-articular involvement. Current regimens usually include corticosteroid therapy with or without immunosuppressants (IS), there is no consensus for the treatment.Objectives:To analyze the different treatment regimens in a cohort of patients with ILD and RA in our clinical practice.Methods:Descriptive study of 57 patients treated in our Hospital (1/1/2018 until 12/31/2019) with a diagnosis of RA (ACR 2010 criteria) and secondary ILD.The most recent American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/Latin American Thoracic Society (ALAT) guidelines define three HRCT (High Resolution Computed Tomography) patterns of fibrosing lung disease in the setting of idiopathic pulmonary fibrosis (IPF): definite Usual Interstitial pneumonia (UIP) (traction bronchiectasis and honeycombing), possible UIP and inconsistent with UIP. The distinction between definite UIP and possible UIP in these to the presence or absence of honeycombing. Approved by the Ethics Committee.Quantitative variables are expressed as mean (SD) and dichotomous variables as percentages (%). Statistical analysis with SPSS version 21.Results:21 men and 36 women were included, with a mean age of 69 ± 10 years (mean ± SD), history of smoking (smokers 14%, non-smokers 43%, former smokers 42%). Clinical ILD at diagnosis (dyspnea 61%, dry cough 56%, crackling 70%, acropachy 7%). 84% were positive rheumatoid factor and 70% positive anticitrullinated protein antibody.Diagnosis of ILD by HRCT in 100% of patients with different patterns: defined UIP 26 (45%), probable UIP 2 (3%) and not UIP 29 (50%). The diagnosis of ILD was confirmed by biopsy in 12 patients.79% underwent (T) treatment prior to the diagnosis of ILD with glucocorticoids and disease-modifying drugs (DMARD). Among the traditional DMARDs used were: Methotrexate 68% (there were no cases of MTX pneumonitis), Leflunomide 47%, Hydroxychloroquine 26% and Sulfasalazine 21%. Biological therapy in 15 patients: Etanercept 19%, Adalimumab 5%, Infliximab 3% and Certolizumab 2%. Two patients presented an exacerbation and rapid progression of the ILD during the T with Etanercept with the final result of death.T with IS after the diagnosis of ILD in 80% of patients (Azathioprine 15, Rituximab 14, Abatacept 10, Tocilizumab 4, Sarilumab 1, Mofetil mycophenolate 1 and Cyclophosphamide 1).Two patients with defined UIP perform T with antifibrotic: 1st Nintedanib (INBUILD Trial, This article was published on September 29, 2019, at NEJM.org) 2nd Pirfenidone (initial diagnosis of IPF Idiopathic Pulmonary Fibrosis and subsequent of seropositive RA with UIP). Both improved greater than 10% in forced vital capacity (FVC) and diffusion capacity of the lung for carbon monoxide (DLCO) in the 6 months after onset of T.Conclusion:Our results, in general, agree with what is published in the literature. Prospective, multicentre and larger sample studies are necessary to better define which patients would benefit more from IS T or antifibrotic T (or if the antifibrotic should be added to the previous IS).Disclosure of Interests:None declared


2021 ◽  
pp. 2004173
Author(s):  
Erol A. Gaillard ◽  
Claudia E. Kuehni ◽  
Steve Turner ◽  
Myrofora Goutaki ◽  
Karl A. Holden ◽  
...  

Diagnosing asthma in children represents an important clinical challenge. There is no single gold standard test to confirm the diagnosis. Consequently, both over-, and under-diagnosis of asthma are frequent in children.A Task Force (TF) supported by the European Respiratory Society has developed these evidence-based clinical practice guidelines for the diagnosis of asthma in children aged 5–16 years using nine PICO (Population, Intervention, Comparator and Outcome) questions. The TF conducted systematic literature searches for all PICO questions and screened the outputs from these, including relevant full text articles. All TF members approved the final decision for inclusion of research papers. The TF assessed the quality of the evidence using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach.The TF then developed a diagnostic algorithm based on the critical appraisal of the PICO questions, preferences expressed by lay members and test availability. Proposed cut-offs were determined based on the best available evidence. The TF formulated recommendations using the GRADE Evidence to Decision framework.Based on the critical appraisal of the evidence and the Evidence to Decision Framework the TF recommends spirometry, bronchodilator reversibility testing and FeNO as first line diagnostic tests in children under investigation for asthma. The TF recommends against diagnosing asthma in children based on clinical history alone or following a single abnormal objective test. Finally, this guideline also proposes a set of research priorities to improve asthma diagnosis in children in the future.


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