Clinical Decision-Making in Hypersensitivity Pneumonitis: Diagnosis and Management

This review provides an updated approach to the diagnosis and management of hypersensitivity pneumonitis (HP). The importance of using a multidisciplinary discussion to increase diagnostic and treatment confidence is emphasized. The role of Bayesian reasoning is highlighted throughout, underscoring the importance of hypothesis generation (differential diagnosis) and diagnostic test interpretation based on the probability of HP. Probability estimates of diagnostic certainty (i.e., a confident versus a working diagnosis) and treatment thresholds are carefully examined.Therapeutically, beyond antigen avoidance and newly available antifibrotic therapy for patients with a progressive fibrosing phenotype; the role, timing, and expected response to anti-inflammatory therapy in individual patients are unanswered questions. Since the evidence and validation of testing generally performed during the diagnostic work-up and longitudinal monitoring of HP is feeble at best, the viewpoints discussed are not intended to resolve current controversies but rather to provide a conceptual framework for evaluating discordant information when evaluating and caring for patients with HP.

[1]  R. Sussman,et al.  A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. , 2014, The New England journal of medicine.

[2]  Sherlock,et al.  Prevalence of Farmer's Lung in Scotland: A Pilot Survey , 1972, British medical journal.

[3]  K. Batra,et al.  The MUC5B promoter polymorphism and telomere length in patients with chronic hypersensitivity pneumonitis , 2017, The Lancet. Respiratory medicine.

[4]  K. Bang,et al.  Twenty-three years of hypersensitivity pneumonitis mortality surveillance in the United States. , 2006, American journal of industrial medicine.

[5]  F. Martinez,et al.  Hypersensitivity Pneumonitis: Radiologic Phenotypes Are Associated With Distinct Survival Time and Pulmonary Function Trajectory , 2019, Chest.

[6]  O. Hilberg,et al.  A cohort study of interstitial lung diseases in central Denmark. , 2014, Respiratory medicine.

[7]  Shandra L. Protzko,et al.  An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. , 2013, American journal of respiratory and critical care medicine.

[8]  D. Bouros,et al.  Epidemiology of interstitial lung diseases in Greece. , 2009, Respiratory medicine.

[9]  A. B. Hill,et al.  "The Environment and Disease: Association or Causation?" (1965), by Austin Bradford Hill , 2017 .

[10]  E. Kazerooni,et al.  High-resolution CT of the lungs. , 2001, AJR. American journal of roentgenology.

[11]  J. Ancochea,et al.  Report on the incidence of interstitial lung diseases in Spain. , 2004, Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG.

[12]  V. Navaratnam,et al.  Mortality trends in asbestosis, extrinsic allergic alveolitis and sarcoidosis in England and Wales. , 2011, Respiratory medicine.

[13]  C. Lin,et al.  Mosaic Attenuation: Etiology, Methods of Differentiation, and Pitfalls. , 2015, Radiographics : a review publication of the Radiological Society of North America, Inc.

[14]  W. Black,et al.  The epidemiology of interstitial lung diseases. , 1994, American journal of respiratory and critical care medicine.

[15]  M. Frydenberg,et al.  Risk of hypersensitivity pneumonitis and interstitial lung diseases among pigeon breeders , 2016, European Respiratory Journal.

[16]  O. Turan,et al.  Epidemiology and distribution of interstitial lung diseases in Turkey , 2014, The clinical respiratory journal.

[17]  O. Heinonen,et al.  Incidence of farmer's lung leading to hospitalization and its relation to meteorological observations in Finland. , 2009, Acta medica Scandinavica.

[18]  Y. Lacasse,et al.  High-resolution CT predictors of hypersensitivity pneumonitis. , 2016, Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG.

[19]  J. Robbins,et al.  The prevalence of farmer's lung in an agricultural population. , 1976, The American review of respiratory disease.

[20]  F. Martinez,et al.  Hypersensitivity pneumonitis and antigen identification--An alternate approach. , 2016, Respiratory medicine.

[21]  D. Boyd.  The Incidence of Farmer's Lung in Caithness , 1971, Scottish medical journal.

[22]  H. Collard,et al.  Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. , 2014, The New England journal of medicine.

[23]  J E Earis,et al.  The inspiratory "squawk" in extrinsic allergic alveolitis and other pulmonary fibroses. , 1982, Thorax.

[24]  A. Woolcock,et al.  Bird breeder's hypersensitivity pneumonitis: progress studies of lung function after cessation of exposure to the provoking antigen. , 2015, The American review of respiratory disease.

[25]  J. Remy,et al.  Importance of ground-glass attenuation in chronic diffuse infiltrative lung disease: pathologic-CT correlation. , 1993, Radiology.

[26]  E. Terho,et al.  Effect of corticosteroid treatment on the recovery of pulmonary function in farmer's lung. , 1992, The American review of respiratory disease.

[27]  E. R. F. Pérez.  The Role of Computed Tomography Honeycombing in Profiling Disease Progression in Chronic Interstitial Lung Disease , 2019 .

[28]  H. Schweisfurth.  [Report by the Scientific Working Group for Therapy of Lung Diseases: German Fibrosis Register with initial results]. , 1996, Pneumologie.

[29]  J. Coalson.  The ultrastructure of human fibrosing alveolitis , 2004, Virchows Archiv A.

[30]  H. Sørensen,et al.  The incidence of interstitial lung disease 1995–2005: a Danish nationwide population-based study , 2008, BMC pulmonary medicine.

[31]  Jonathan H. Chung,et al.  CT findings associated with survival in chronic hypersensitivity pneumonitis , 2017, European Radiology.

[32]  K. Raimundo,et al.  Epidemiology of Hypersensitivity Pneumonitis among an Insured Population in the United States: A Claims‐based Cohort Analysis , 2017, Annals of the American Thoracic Society.

[33]  M. Bando,et al.  The Relationship between the Incidence of Summer-type Hypersensitivity Pneumonitis and Environmental Factors in Southern Tochigi Prefecture , 2017, Internal medicine.

[34]  X. Muñoz,et al.  Inhalation challenge in the differential diagnosis of usual interstitial pneumonia , 2015, European Respiratory Review.

[35]  D. Hendrick,et al.  Budgerigar-fancier's lung: the commonest variety of allergic alveolitis in Britain. , 1978, British medical journal.

[36]  A. Papali,et al.  Evaluation of the patient with an exposure-related disease: the occupational and environmental history , 2015, Current opinion in pulmonary medicine.

[37]  K. Batra,et al.  Telomere-related lung fibrosis is diagnostically heterogeneous but uniformly progressive , 2016, European Respiratory Journal.

[38]  A. Wells,et al.  Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis , 2018, Respiratory medicine.

[39]  K Torizuka,et al.  Centrilobular lesions of the lung: demonstration by high-resolution CT and pathologic correlation. , 1986, Radiology.

[40]  D. Pelclova,et al.  Occupational hypersensitivity pneumonitis reported to the Czech National Registry Of Occupational Diseases in the period 1992-2005. , 2009, Industrial health.

[41]  U. Tateishi,et al.  Chronic Hypersensitivity Pneumonitis With a Usual Interstitial Pneumonia-Like Pattern: Correlation Between Histopathologic and Clinical Findings. , 2016, Chest.

[42]  A. Pardo,et al.  Idiopathic Pulmonary Fibrosis: Prevailing and Evolving Hypotheses about Its Pathogenesis and Implications for Therapy , 2001, Annals of Internal Medicine.

[43]  F. Martinez,et al.  Development and validation of a radiological diagnosis model for hypersensitivity pneumonitis , 2018, European Respiratory Journal.

[44]  F. Morell,et al.  Time of exposure as a prognostic factor in avian hypersensitivity pneumonitis. , 1989, Respiratory medicine.

[45]  Susan Murray,et al.  Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? , 2004, American journal of respiratory and critical care medicine.

[46]  D. Lynch,et al.  Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. , 2008, Radiology.

[47]  T. Akashi,et al.  The pathogenesis of chronic hypersensitivity pneumonitis in common with idiopathic pulmonary fibrosis: expression of apoptotic markers. , 2010, American journal of clinical pathology.

[48]  S. Walsh,et al.  Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. , 2019, The New England journal of medicine.

[49]  C. Tinelli,et al.  First report of the Italian register for diffuse infiltrative lung disorders (RIPID). , 2001, Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace.

[50]  H. Collard,et al.  A diagnostic model for chronic hypersensitivity pneumonitis , 2016, Thorax.

[51]  A. Christe,et al.  Accuracy of low-dose computed tomography (CT) for detecting and characterizing the most common CT-patterns of pulmonary disease. , 2013, European journal of radiology.

[52]  Jonathan H. Chung,et al.  Prognosticating Outcomes in Interstitial Lung Disease by Mediastinal Lymph Node Assessment. An Observational Cohort Study with Independent Validation , 2019, American journal of respiratory and critical care medicine.

[53]  J. Dalphin,et al.  Prevalence and risk factors for chronic bronchitis and farmer's lung in French dairy farmers. , 1993, British journal of industrial medicine.

[54]  H. Collard,et al.  Use of Mycophenolate Mofetil or Azathioprine for the Management of Chronic Hypersensitivity Pneumonitis , 2017, Chest.

[55]  Y. Lacasse,et al.  Classification of Hypersensitivity Pneumonitis , 2009, International Archives of Allergy and Immunology.

[56]  K. Brown,et al.  Fibrotic hypersensitivity pneumonitis , 2014, Current Respiratory Care Reports.

[57]  G. Raghu,et al.  Chronic hypersensitivity pneumonitis in patients diagnosed with idiopathic pulmonary fibrosis: a prospective case-cohort study. , 2013, The Lancet. Respiratory medicine.

[58]  Jonathan H. Chung,et al.  Presence of Air Trapping and Mosaic Attenuation on Chest Computed Tomography Predicts Survival in Chronic Hypersensitivity Pneumonitis , 2017, Annals of the American Thoracic Society.

[59]  H. Collard,et al.  Radiographic fibrosis score predicts survival in hypersensitivity pneumonitis. , 2013, Chest.

[60]  G. Raghu,et al.  Interstitial Lung Disease in India. Results of a Prospective Registry , 2017, American journal of respiratory and critical care medicine.

[61]  V. Kurup,et al.  The long-term outcome in acute, subacute, and chronic forms of pigeon breeder's disease hypersensitivity pneumonitis. , 2002, Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology.

[62]  B. Yaspan,et al.  Rare Protein-altering Telomere-related Gene Variants in Patients with Chronic Hypersensitivity Pneumonitis. , 2019, American journal of respiratory and critical care medicine.

[63]  M. Demedts,et al.  REGISTRATION OF INTERSTITIAL LUNG DISEASES BY 20 CENTRES OF RESPIRATORY MEDICINE IN FLANDERS , 2001, Acta clinica Belgica.

[64]  Jonathan H. Chung,et al.  Outcomes of immunosuppressive therapy in chronic hypersensitivity pneumonitis , 2017, ERJ Open Research.

[65]  R. Hubbard,et al.  Extrinsic allergic alveolitis: incidence and mortality in the general population. , 2007, QJM : monthly journal of the Association of Physicians.

[66]  I. Annesi-Maesano,et al.  Prevalence and incidence of interstitial lung diseases in a multi-ethnic county of Greater Paris , 2017, European Respiratory Journal.

[67]  Lilian T. Kuranishi,et al.  Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis , 2017, Thorax.

[68]  O. Hilberg,et al.  Incidence, comorbidity and survival rate of hypersensitivity pneumonitis: a national population-based study , 2019, ERJ Open Research.

[69]  I. Noth,et al.  Circulating Plasma Biomarkers of Progressive Interstitial Lung Disease. , 2020, American journal of respiratory and critical care medicine.

[70]  A. Wells,et al.  Diagnostic value of BAL lymphocytosis in patients with indeterminate for usual interstitial pneumonia imaging pattern , 2019, European Respiratory Journal.

[71]  M. Carder,et al.  Epidemiology of occupational hypersensitivity pneumonitis; reports from the SWORD scheme in the UK from 1996 to 2015 , 2016, Occupational and Environmental Medicine.

[72]  A. Robertson,et al.  Characteristics of hypersensitivity pneumonitis diagnosed by interstitial and occupational lung disease multi-disciplinary team consensus. , 2019, Respiratory medicine.

[73]  P. Malmberg,et al.  Incidence of organic dust toxic syndrome and allergic alveolitis in Swedish farmers. , 1988, International archives of allergy and applied immunology.

[74]  M J Hodgson,et al.  Chest X-rays in hypersensitivity pneumonitis: a metaanalysis of secular trend. , 1989, American journal of industrial medicine.

[75]  Carl E. Grimes,et al.  Procedures to Assist Health Care Providers to Determine When Home Assessments for Potential Mold Exposure Are Warranted. , 2016, The journal of allergy and clinical immunology. In practice.

[76]  David P. Naidich,et al.  Fibrosing interstitial lung disease. A practical high-resolution computed tomography-based approach to diagnosis and management and a review of the literature. , 2013, American journal of respiratory and critical care medicine.

[77]  O. Gajic,et al.  Ventilator settings and outcome of respiratory failure in chronic interstitial lung disease. , 2008, Chest.

[78]  Shuichi Tanoue,et al.  Clinical/pathologic correlations in 553 patients with primary centrilobular findings on high-resolution CT scan of the thorax. , 2007, Chest.

[79]  K. Brown,et al.  Identifying an inciting antigen is associated with improved survival in patients with chronic hypersensitivity pneumonitis. , 2013, Chest.

[80]  K. Brown,et al.  Increasing Hypersensitivity Pneumonitis-related Mortality in the United States from 1988 to 2016. , 2019, American journal of respiratory and critical care medicine.

[81]  D M Hansell,et al.  Hypersensitivity pneumonitis: correlation of individual CT patterns with functional abnormalities. , 1996, Radiology.

[82]  O. Heinonen,et al.  Incidence of clinically confirmed farmer's lung in Finland and its relation to meteorological factors. , 1987, European journal of respiratory diseases. Supplement.

[83]  A. Wells,et al.  Variable utility of mosaic attenuation to distinguish fibrotic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis , 2019, European Respiratory Journal.

[84]  D. Lynch,et al.  Hypersensitivity pneumonitis: sensitivity of high-resolution CT in a population-based study. , 1992, AJR. American journal of roentgenology.

[85]  E. Alhamad.  Interstitial lung diseases in Saudi Arabia: A single-center study , 2013, Annals of thoracic medicine.