Semin Respir Crit Care Med 2022; 43(06): 764-779
DOI: 10.1055/s-0042-1755563
Review Article

High-Resolution Computed Tomography of Fibrotic Interstitial Lung Disease

Karen Rodriguez
1   Division of Thoracic Imaging and Intervention, Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts
Christian L. Ashby
2   School of Medicine, Universidad Central del Caribe School of Medicine, Bayamón, Puerto Rico
Valeria R. Varela
2   School of Medicine, Universidad Central del Caribe School of Medicine, Bayamón, Puerto Rico
Amita Sharma
1   Division of Thoracic Imaging and Intervention, Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts
› Author Affiliations


While radiography is the first-line imaging technique for evaluation of pulmonary disease, high-resolution computed tomography (HRCT) provides detailed assessment of the lung parenchyma and interstitium, allowing normal anatomy to be differentiated from superimposed abnormal findings. The fibrotic interstitial lung diseases have HRCT features that include reticulation, traction bronchiectasis and bronchiolectasis, honeycombing, architectural distortion, and volume loss. The characterization and distribution of these features result in distinctive CT patterns. The CT pattern and its progression over time can be combined with clinical, serologic, and pathologic data during multidisciplinary discussion to establish a clinical diagnosis. Serial examinations identify progression, treatment response, complications, and can assist in determining prognosis. This article will describe the technique used to perform HRCT, the normal and abnormal appearance of the lung on HRCT, and the CT patterns identified in common fibrotic lung diseases.

Publication History

Article published online:
28 October 2022

© 2022. Thieme. All rights reserved.

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  • References

  • 1 Lynch DA, Sverzellati N, Travis WD. et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med 2018; 6 (02) 138-153
  • 2 Hodnett PA, Naidich DP. Fibrosing interstitial lung disease. A practical high-resolution computed tomography-based approach to diagnosis and management and a review of the literature. Am J Respir Crit Care Med 2013; 188 (02) 141-149
  • 3 American College of Radiology. ACR-STR Practice Parameter for the Performance of High- Resolution Computed Tomography (HRCT) of the Lungs in Adults. 2020. Accessed March 6, 2022, at:
  • 4 American Thoracic Society, European Respiratory Society. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002; 165 (02) 277-304
  • 5 Kishaba T. Acute exacerbation of idiopathic pulmonary fibrosis. Medicina (Kaunas) 2019; 55 (03) E70
  • 6 Kim M, Lee SM, Song JW. et al. Added value of prone CT in the assessment of honeycombing and classification of usual interstitial pneumonia pattern. Eur J Radiol 2017; 91: 66-70
  • 7 Verschakelen JA, Van fraeyenhoven L, Laureys G, Demedts M, Baert AL. Differences in CT density between dependent and nondependent portions of the lung: influence of lung volume. AJR Am J Roentgenol 1993; 161 (04) 713-717
  • 8 Lee KN, Yoon SK, Sohn CH, Choi PJ, Webb WR. Dependent lung opacity at thin-section CT: evaluation by spirometrically-gated CT of the influence of lung volume. Korean J Radiol 2002; 3 (01) 24-29
  • 9 Remy-Jardin M, Campistron P, Amara A. et al. Usefulness of coronal reformations in the diagnostic evaluation of infiltrative lung disease. J Comput Assist Tomogr 2003; 27 (02) 266-273
  • 10 Tokura S, Okuma T, Akira M, Arai T, Inoue Y, Kitaichi M. Utility of expiratory thin-section CT for fibrotic interstitial pneumonia. Acta Radiol 2014; 55 (09) 1050-1055
  • 11 Raghu G, Remy-Jardin M, Ryerson CJ. et al. Diagnosis of hypersensitivity pneumonitis in adults: an official ATS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2020; 202 (03) e36-e69
  • 12 Bhalla M, Naidich DP, McGuinness G, Gruden JF, Leitman BS, McCauley DI. Diffuse lung disease: assessment with helical CT–preliminary observations of the role of maximum and minimum intensity projection images. Radiology 1996; 200 (02) 341-347
  • 13 Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology 2008; 246 (03) 697-722
  • 14 Oikonomou A, Prassopoulos P. Mimics in chest disease: interstitial opacities. Insights Imaging 2013; 4 (01) 9-27
  • 15 Webb WR. Thin-section CT of the secondary pulmonary lobule: anatomy and the image–the 2004 Fleischner lecture. Radiology 2006; 239 (02) 322-338
  • 16 Weibel ER. Fleischner Lecture. Looking into the lung: what can it tell us?. AJR Am J Roentgenol 1979; 133 (06) 1021-1031
  • 17 Hatabu H, Hunninghake GM, Lynch DA. Interstitial lung abnormality: recognition and perspectives. Radiology 2019; 291 (01) 1-3
  • 18 Hochhegger B, Marchiori E, Zanon M. et al. Imaging in idiopathic pulmonary fibrosis: diagnosis and mimics. Clinics (São Paulo) 2019; 74: e225
  • 19 Remy-Jardin M, Giraud F, Remy J, Copin MC, Gosselin B, Duhamel A. Importance of ground-glass attenuation in chronic diffuse infiltrative lung disease: pathologic-CT correlation. Radiology 1993; 189 (03) 693-698
  • 20 Suzuki Y, Saito J, Togawa R, Minemura H, Munakata M. Intralobular septal thickening on chest CT in a patient with pulmonary amyloidosis: a rare case study. Thorax 2017; 72 (07) 673-674
  • 21 Mai C, Verleden SE, McDonough JE. et al. Thin-section CT features of idiopathic pulmonary fibrosis correlated with micro-CT and histologic analysis. Radiology 2017; 283 (01) 252-263
  • 22 Mukhopadhyay S, Parambil JG. Acute interstitial pneumonia (AIP): relationship to Hamman-Rich syndrome, diffuse alveolar damage (DAD), and acute respiratory distress syndrome (ARDS). Semin Respir Crit Care Med 2012; 33 (05) 476-485
  • 23 Taniguchi H, Kondoh Y. Acute and subacute idiopathic interstitial pneumonias. Respirology 2016; 21 (05) 810-820
  • 24 Ichikado K. High-resolution computed tomography findings of acute respiratory distress syndrome, acute interstitial pneumonia, and acute exacerbation of idiopathic pulmonary fibrosis. Semin Ultrasound CT MR 2014; 35 (01) 39-46
  • 25 Walsh SL, Sverzellati N, Devaraj A, Wells AU, Hansell DM. Chronic hypersensitivity pneumonitis: high resolution computed tomography patterns and pulmonary function indices as prognostic determinants. Eur Radiol 2012; 22 (08) 1672-1679
  • 26 Walsh SL, Sverzellati N, Devaraj A, Keir GJ, Wells AU, Hansell DM. Connective tissue disease related fibrotic lung disease: high resolution computed tomographic and pulmonary function indices as prognostic determinants. Thorax 2014; 69 (03) 216-222
  • 27 Tominaga J, Bankier AA, Lee KS. et al; Study Group of Diffuse Interstitial Lung Disease in Japan. Inter-observer agreement in identifying traction bronchiectasis on computed tomography: its improvement with the use of the additional criteria for chronic fibrosing interstitial pneumonia. Jpn J Radiol 2019; 37 (11) 773-780
  • 28 Watadani T, Sakai F, Johkoh T. et al. Interobserver variability in the CT assessment of honeycombing in the lungs. Radiology 2013; 266 (03) 936-944
  • 29 Desai SR, Wells AU, Rubens MB, du Bois RM, Hansell DM. Traction bronchiectasis in cryptogenic fibrosing alveolitis: associated computed tomographic features and physiological significance. Eur Radiol 2003; 13 (08) 1801-1808
  • 30 Raghu G, Collard HR, Egan JJ. et al; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183 (06) 788-824
  • 31 Westcott JL, Cole SR. Traction bronchiectasis in end-stage pulmonary fibrosis. Radiology 1986; 161 (03) 665-669
  • 32 Sundaram B, Gross BH, Martinez FJ. et al. Accuracy of high-resolution CT in the diagnosis of diffuse lung disease: effect of predominance and distribution of findings. AJR Am J Roentgenol 2008; 191 (04) 1032-1039
  • 33 Egashira R, Jacob J, Kokosi MA. et al. Diffuse pulmonary ossification in fibrosing interstitial lung diseases: prevalence and associations. Radiology 2017; 284 (01) 255-263
  • 34 Montag M. Radiographic Signs and Differential Diagnoses. In: Lange S, Walsh G. eds. Radiology of Chest Diseases. 3rd ed. Stuttgart: Thieme Verlagsgruppe; 2007: 328-330
  • 35 Chalmers JD, Goeminne P, Aliberti S. et al. The bronchiectasis severity index. An international derivation and validation study. Am J Respir Crit Care Med 2014; 189 (05) 576-585
  • 36 Matsuoka S, Uchiyama K, Shima H, Ueno N, Oish S, Nojiri Y. Bronchoarterial ratio and bronchial wall thickness on high-resolution CT in asymptomatic subjects: correlation with age and smoking. AJR Am J Roentgenol 2003; 180 (02) 513-518
  • 37 Alhamad EH. Clinical characteristics and survival in idiopathic pulmonary fibrosis and connective tissue disease-associated usual interstitial pneumonia. J Thorac Dis 2015; 7 (03) 386-393
  • 38 Prenzel F, Harfst J, Schwerk N. et al; LIP/FB-Kids-Lung-Registry Study Group. Lymphocytic interstitial pneumonia and follicular bronchiolitis in children: a registry-based case series. Pediatr Pulmonol 2020; 55 (04) 909-917
  • 39 Flaherty KR, King Jr. TE, Raghu G. et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis?. Am J Respir Crit Care Med 2004; 170 (08) 904-910
  • 40 Chaudhuri N, Spencer L, Greaves M, Bishop P, Chaturvedi A, Leonard C. A review of the multidisciplinary diagnosis of interstitial lung diseases: a retrospective analysis in a single UK specialist centre. J Clin Med 2016; 5 (08) E66
  • 41 Hobbs S, Chung JH, Leb J, Kaproth-Joslin K, Lynch DA. Practical imaging interpretation in patients suspected of having idiopathic pulmonary fibrosis: official recommendations from the Radiology Working Group of the Pulmonary Fibrosis Foundation. Radiol Cardiothorac Imaging 2021; 3 (01) e200279
  • 42 Raghu G, Remy-Jardin M, Myers JL. et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Diagnosis of idiopathic pulmonary fibrosis: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2018; 198 (05) e44-e68
  • 43 Madan R, Chansakul T, Goldberg HJ. Imaging in lung transplants: checklist for the radiologist. Indian J Radiol Imaging 2014; 24 (04) 318-326
  • 44 Popper HH. Fibrosing pneumonia – how to diagnose, and how to recognize the etiology?. Surg Exp Pathol 2020;3(15)
  • 45 Travis WD, Costabel U, Hansell DM. et al; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188 (06) 733-748
  • 46 Bonifazi M, Montero MA, Renzoni EA. Idiopathic pleuroparenchymal fibroelastosis. Curr Pulmonol Rep 2017; 6 (01) 9-15
  • 47 Fell CD, Martinez FJ, Liu LX. et al. Clinical predictors of a diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2010; 181 (08) 832-837
  • 48 Brownell R, Moua T, Henry TS. et al. The use of pretest probability increases the value of high-resolution CT in diagnosing usual interstitial pneumonia. Thorax 2017; 72 (05) 424-429
  • 49 Shih AR, Nitiwarangkul C, Little BP. et al. Practical application and validation of the 2018 ATS/ERS/JRS/ALAT and Fleischner Society guidelines for the diagnosis of idiopathic pulmonary fibrosis. Respir Res 2021; 22 (01) 124
  • 50 Yagihashi K, Huckleberry J, Colby TV. et al; Idiopathic Pulmonary Fibrosis Clinical Research Network (IPFnet). Radiologic-pathologic discordance in biopsy-proven usual interstitial pneumonia. Eur Respir J 2016; 47 (04) 1189-1197
  • 51 Sumikawa H, Johkoh T, Colby TV. et al. Computed tomography findings in pathological usual interstitial pneumonia: relationship to survival. Am J Respir Crit Care Med 2008; 177 (04) 433-439
  • 52 Sverzellati N, Wells AU, Tomassetti S. et al. Biopsy-proved idiopathic pulmonary fibrosis: spectrum of nondiagnostic thin-section CT diagnoses. Radiology 2010; 254 (03) 957-964
  • 53 Raghu G, Lynch D, Godwin JD. et al. Diagnosis of idiopathic pulmonary fibrosis with high-resolution CT in patients with little or no radiological evidence of honeycombing: secondary analysis of a randomised, controlled trial. Lancet Respir Med 2014; 2 (04) 277-284
  • 54 Chung JH, Chawla A, Peljto AL. et al. CT scan findings of probable usual interstitial pneumonitis have a high predictive value for histologic usual interstitial pneumonitis. Chest 2015; 147 (02) 450-459
  • 55 Silva CI, Müller NL, Lynch DA. et al. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology 2008; 246 (01) 288-297
  • 56 Flaherty KR, Thwaite EL, Kazerooni EA. et al. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax 2003; 58 (02) 143-148
  • 57 Inomata M, Jo T, Kuse N. et al. Clinical impact of the radiological indeterminate for usual interstitial pneumonia pattern on the diagnosis of idiopathic pulmonary fibrosis. Respir Investig 2021; 59 (01) 81-89
  • 58 Kwon BS, Choe J, Do KH, Hwang HS, Chae EJ, Song JW. Computed tomography patterns predict clinical course of idiopathic pulmonary fibrosis. Respir Res 2020; 21 (01) 295
  • 59 Fukihara J, Kondoh Y, Brown KK. et al. Probable usual interstitial pneumonia pattern on chest CT: is it sufficient for a diagnosis of idiopathic pulmonary fibrosis?. Eur Respir J 2020; 55 (04) 1802465
  • 60 Salisbury ML, Tolle LB, Xia M. et al. Possible UIP pattern on high-resolution computed tomography is associated with better survival than definite UIP in IPF patients. Respir Med 2017; 131: 229-235
  • 61 Mononen ME, Kettunen HP, Suoranta SK. et al. Several specific high-resolution computed tomography patterns correlate with survival in patients with idiopathic pulmonary fibrosis. J Thorac Dis 2021; 13 (04) 2319-2330
  • 62 Lee HY, Lee KS, Jeong YJ. et al. High-resolution CT findings in fibrotic idiopathic interstitial pneumonias with little honeycombing: serial changes and prognostic implications. AJR Am J Roentgenol 2012; 199 (05) 982-989
  • 63 Salvatore M, Singh A, Yip R. et al. Progression of probable UIP and UIP on HRCT. Clin Imaging 2019; 58 (58) 140-144
  • 64 Raghu G, Chen SY, Yeh WS. et al. Idiopathic pulmonary fibrosis in US Medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respir Med 2014; 2 (07) 566-572
  • 65 Richeldi L, du Bois RM, Raghu G. et al; INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370 (22) 2071-2082
  • 66 King Jr. TE, Bradford WZ, Castro-Bernardini S. et al; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370 (22) 2083-2092
  • 67 Balestro E, Cocconcelli E, Giraudo C. et al. High-resolution CT change over time in patients with idiopathic pulmonary fibrosis on antifibrotic treatment. J Clin Med 2019; 8 (09) E1469
  • 68 Iwasawa T, Ogura T, Sakai F. et al. CT analysis of the effect of pirfenidone in patients with idiopathic pulmonary fibrosis. Eur J Radiol 2014; 83 (01) 32-38
  • 69 Jacob J, Aksman L, Mogulkoc N. et al. Serial CT analysis in idiopathic pulmonary fibrosis: comparison of visual features that determine patient outcome. Thorax 2020; 75 (08) 648-654
  • 70 Humphries SM, Yagihashi K, Huckleberry J. et al. Idiopathic pulmonary fibrosis: data-driven textural analysis of extent of fibrosis at baseline and 15-month follow-up. Radiology 2017; 285 (01) 270-278
  • 71 Kligerman SJ, Groshong S, Brown KK, Lynch DA. Nonspecific interstitial pneumonia: radiologic, clinical, and pathologic considerations. Radiographics 2009; 29 (01) 73-87
  • 72 Travis WD, Hunninghake G, King Jr. TE. et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med 2008; 177 (12) 1338-1347
  • 73 Daniil ZD, Gilchrist FC, Nicholson AG. et al. A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 1999; 160 (03) 899-905
  • 74 MacDonald SL, Rubens MB, Hansell DM. et al. Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparative appearances at and diagnostic accuracy of thin-section CT. Radiology 2001; 221 (03) 600-605
  • 75 Lynch DA, Travis WD, Müller NL. et al. Idiopathic interstitial pneumonias: CT features. Radiology 2005; 236 (01) 10-21
  • 76 Ebner L, Christodoulidis S, Stathopoulou T. et al. Meta-analysis of the radiological and clinical features of usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP). PLoS One 2020; 15 (01) e0226084
  • 77 Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol 1994; 18 (02) 136-147
  • 78 Silva CI, Müller NL, Hansell DM, Lee KS, Nicholson AG, Wells AU. Nonspecific interstitial pneumonia and idiopathic pulmonary fibrosis: changes in pattern and distribution of disease over time. Radiology 2008; 247 (01) 251-259
  • 79 Kim DS, Collard HR, King Jr TE. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006; 3 (04) 285-292
  • 80 Akira M, Inoue G, Yamamoto S, Sakatani M. Non-specific interstitial pneumonia: findings on sequential CT scans of nine patients. Thorax 2000; 55 (10) 854-859
  • 81 Schneider F, Hwang DM, Gibson K, Yousem SA. Nonspecific interstitial pneumonia: a study of 6 patients with progressive disease. Am J Surg Pathol 2012; 36 (01) 89-93
  • 82 Enomoto N, Sumikawa H, Sugiura H. et al. Clinical, radiological, and pathological evaluation of “NSIP with OP overlap” pattern compared with NSIP in patients with idiopathic interstitial pneumonias. Respir Med 2020; 174: 106201
  • 83 Jonigk D, Stark H, Braubach P. et al. Morphological and molecular motifs of fibrosing pulmonary injury patterns. J Pathol Clin Res 2019; 5 (04) 256-271
  • 84 Bjoraker JA, Ryu JH, Edwin MK. et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 157 (01) 199-203
  • 85 Riha RL, Duhig EE, Clarke BE, Steele RH, Slaughter RE, Zimmerman PV. Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia. Eur Respir J 2002; 19 (06) 1114-1118
  • 86 Flaherty KR, Toews GB, Travis WD. et al. Clinical significance of histological classification of idiopathic interstitial pneumonia. Eur Respir J 2002; 19 (02) 275-283
  • 87 Cottin V, Donsbeck AV, Revel D, Loire R, Cordier JF. Nonspecific interstitial pneumonia. Individualization of a clinicopathologic entity in a series of 12 patients. Am J Respir Crit Care Med 1998; 158 (04) 1286-1293
  • 88 Jain A, Shannon VR, Sheshadri A. Immune-related adverse events: pneumonitis. Adv Exp Med Biol 2018; 995: 131-149
  • 89 Suda T, Kono M, Nakamura Y. et al. Distinct prognosis of idiopathic nonspecific interstitial pneumonia (NSIP) fulfilling criteria for undifferentiated connective tissue disease (UCTD). Respir Med 2010; 104 (10) 1527-1534
  • 90 Nunes H, Schubel K, Piver D. et al. Nonspecific interstitial pneumonia: survival is influenced by the underlying cause. Eur Respir J 2015; 45 (03) 746-755
  • 91 Latsi PI, du Bois RM, Nicholson AG. et al. Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med 2003; 168 (05) 531-537
  • 92 Jegal Y, Kim DS, Shim TS. et al. Physiology is a stronger predictor of survival than pathology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med 2005; 171 (06) 639-644
  • 93 Tanizawa K, Handa T, Kubo T. et al. Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study. Respir Res 2018; 19 (01) 162
  • 94 Nunes H, Jeny F, Bouvry D. et al. Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations. Eur Respir J 2017; 49 (05) 1602022
  • 95 Pakhale SS, Hadjiliadis D, Howell DN. et al. Upper lobe fibrosis: a novel manifestation of chronic allograft dysfunction in lung transplantation. J Heart Lung Transplant 2005; 24 (09) 1260-1268
  • 96 von der Thüsen JH, Hansell DM, Tominaga M. et al. Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation. Mod Pathol 2011; 24 (12) 1633-1639
  • 97 Parish JM, Muhm JR, Leslie KO. Upper lobe pulmonary fibrosis associated with high-dose chemotherapy containing BCNU for bone marrow transplantation. Mayo Clin Proc 2003; 78 (05) 630-634
  • 98 Konen E, Weisbrod GL, Pakhale S, Chung T, Paul NS, Hutcheon MA. Fibrosis of the upper lobes: a newly identified late-onset complication after lung transplantation?. AJR Am J Roentgenol 2003; 181 (06) 1539-1543
  • 99 Beynat-Mouterde C, Beltramo G, Lezmi G. et al. Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents. Eur Respir J 2014; 44 (02) 523-527
  • 100 Piciucchi S, Tomassetti S, Casoni G. et al. High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res 2011; 12: 111
  • 101 Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest 2004; 126 (06) 2007-2013
  • 102 Morshid A, Moshksar A, Das A, Duarte AG, Palacio D, Villanueva-Meyer J. HRCT diagnosis of pleuroparenchymal fibroelastosis: report of two cases. Radiol Case Rep 2021; 16 (06) 1564-1569
  • 103 Fujisawa T, Horiike Y, Egashira R. et al. Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias. Respir Res 2021; 22 (01) 290
  • 104 Esteves C, Costa FR, Redondo MT. et al. Pleuroparenchymal fibroelastosis: role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core lung biopsy. Insights Imaging 2016; 7 (01) 155-162
  • 105 Reddy TL, Tominaga M, Hansell DM. et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012; 40 (02) 377-385
  • 106 Chua F, Desai SR, Nicholson AG. et al. Pleuroparenchymal fibroelastosis. A review of clinical, radiological, and pathological characteristics. Ann Am Thorac Soc 2019; 16 (11) 1351-1359
  • 107 Rasciti E, Cancellieri A, Romagnoli M, Dell'Amore A, Zompatori M. Suspected pleuroparenchymal fibroelastosis relapse after lung transplantation: a case report and literature review. BJR Case Rep 2019; 5 (04) 20190040
  • 108 Ofek E, Sato M, Saito T. et al. Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis. Mod Pathol 2013; 26 (03) 350-356
  • 109 Bonham CA, Strek ME, Patterson KC. From granuloma to fibrosis: sarcoidosis associated pulmonary fibrosis. Curr Opin Pulm Med 2016; 22 (05) 484-491
  • 110 Handa T, Nagai S, Fushimi Y. et al. Clinical and radiographic indices associated with airflow limitation in patients with sarcoidosis. Chest 2006; 130 (06) 1851-1856
  • 111 Silva M, Nunes H, Valeyre D, Sverzellati N. Imaging of sarcoidosis. Clin Rev Allergy Immunol 2015; 49 (01) 45-53
  • 112 Polverosi R, Russo R, Coran A. et al. Typical and atypical pattern of pulmonary sarcoidosis at high-resolution CT: relation to clinical evolution and therapeutic procedures. Radiol Med (Torino) 2014; 119 (06) 384-392
  • 113 Tana C, Donatiello I, Coppola MG. et al. CT findings in pulmonary and abdominal sarcoidosis. Implications for diagnosis and classification. J Clin Med 2020; 9 (09) E3028
  • 114 Sawahata M, Johkoh T, Kawanobe T. et al. Computed tomography images of fibrotic pulmonary sarcoidosis leading to chronic respiratory failure. J Clin Med 2020; 9 (01) E142
  • 115 Nunes H, Uzunhan Y, Gille T, Lamberto C, Valeyre D, Brillet PY. Imaging of sarcoidosis of the airways and lung parenchyma and correlation with lung function. Eur Respir J 2012; 40 (03) 750-765
  • 116 Salvatore M, Toussie D, Pavlishyn N. et al. The right upper lobe bronchus angle: a tool for differentiating fibrotic and non-fibrotic sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (02) 99-103
  • 117 Verleden SE, Vanstapel A, De Sadeleer L. et al. Distinct airway involvement in subtypes of end-stage fibrotic pulmonary sarcoidosis. Chest 2021; 160 (02) 562-571
  • 118 Shigemitsu H, Oblad JM, Sharma OP, Koss MN. Chronic interstitial pneumonitis in end-stage sarcoidosis. Eur Respir J 2010; 35 (03) 695-697
  • 119 Zhang C, Chan KM, Schmidt LA, Myers JL. Histopathology of explanted lungs from patients with a diagnosis of pulmonary sarcoidosis. Chest 2016; 149 (02) 499-507
  • 120 Abehsera M, Valeyre D, Grenier P, Jaillet H, Battesti JP, Brauner MW. Sarcoidosis with pulmonary fibrosis: CT patterns and correlation with pulmonary function. AJR Am J Roentgenol 2000; 174 (06) 1751-1757
  • 121 Baughman RP, Engel PJ, Taylor L, Lower EE. Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest 2010; 138 (05) 1078-1085
  • 122 Diaz-Guzman E, Farver C, Parambil J, Culver DA. Pulmonary hypertension caused by sarcoidosis. Clin Chest Med 2008; 29 (03) 549-563 , x
  • 123 Spagnolo P, Rossi G, Trisolini R, Sverzellati N, Baughman RP, Wells AU. Pulmonary sarcoidosis. Lancet Respir Med 2018; 6 (05) 389-402
  • 124 Walsh SL, Wells AU, Sverzellati N. et al. An integrated clinicoradiological staging system for pulmonary sarcoidosis: a case-cohort study. Lancet Respir Med 2014; 2 (02) 123-130
  • 125 Hoffstein V, Ranganathan N, Mullen JB. Sarcoidosis simulating pulmonary veno-occlusive disease. Am Rev Respir Dis 1986; 134 (04) 809-811
  • 126 Hanak V, Golbin JM, Ryu JH. Causes and presenting features in 85 consecutive patients with hypersensitivity pneumonitis. Mayo Clin Proc 2007; 82 (07) 812-816
  • 127 Fernández Pérez ER, Swigris JJ, Forssén AV. et al. Identifying an inciting antigen is associated with improved survival in patients with chronic hypersensitivity pneumonitis. Chest 2013; 144 (05) 1644-1651
  • 128 Inase N, Ohtani Y, Sumi Y. et al. A clinical study of hypersensitivity pneumonitis presumably caused by feather duvets. Ann Allergy Asthma Immunol 2006; 96 (01) 98-104
  • 129 Jordan LE, Guy E. Paediatric feather duvet hypersensitivity pneumonitis. BMJ Case Rep 2015; 2015: bcr2014207956
  • 130 Ryerson CJ, Vittinghoff E, Ley B. et al. Predicting survival across chronic interstitial lung disease: the ILD-GAP model. Chest 2014; 145 (04) 723-728
  • 131 Morell F, Roger À, Reyes L, Cruz MJ, Murio C, Muñoz X. Bird fancier's lung: a series of 86 patients. Medicine (Baltimore) 2008; 87 (02) 110-130
  • 132 Thomeer MJ, Costabe U, Rizzato G, Poletti V, Demedts M. Comparison of registries of interstitial lung diseases in three European countries. Eur Respir J Suppl 2001; 32: 114s-118s
  • 133 Schweisfurth H. Report by the Scientific Working Group for Therapy of Lung Diseases: German Fibrosis Register with initial results. Pneumologie 1996; 50 (12) 899-901 [Mitteilung der Wissenschaftlichen Arbeitsgemeinschaft für die Therapie von Lungenkrankheiten (WATL): Deutsches Fibroseregister mit ersten Ergebnissen]
  • 134 Thomeer M, Demedts M, Vandeurzen K. VRGT Working Group on Interstitial Lung Diseases. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders. Acta Clin Belg 2001; 56 (03) 163-172
  • 135 Fisher JH, Kolb M, Algamdi M. et al. Baseline characteristics and comorbidities in the CAnadian REgistry for Pulmonary Fibrosis. BMC Pulm Med 2019; 19 (01) 223
  • 136 Kreuter M, Herth FJ, Wacker M. et al. Exploring clinical and epidemiological characteristics of interstitial lung diseases: rationale, aims, and design of a nationwide prospective registry – the EXCITING-ILD Registry. BioMed Res Int 2015; 2015: 123876
  • 137 Karakatsani A, Papakosta D, Rapti A. et al; Hellenic Interstitial Lung Diseases Group. Epidemiology of interstitial lung diseases in Greece. Respir Med 2009; 103 (08) 1122-1129
  • 138 Ansarie M. A national guideline and ILD PAK Registry Report: recent landmarks in the understanding of interstitial lung diseases in Pakistan. J Pak Med Assoc 2016; 66 (09) 1050-1053
  • 139 Strâmbu I. REGIS–Romanian National Registry for Interstitial Lung Diseases and Sarcoidosis: launch of the website and building-up the database. Pneumologia 2014; 63 (02) 96-99 [ REGIS–Registrul Naţional de Pneumopatii Interstiţiale Difuze şi Sarcoidoză: lansarea site-ulu web şi modul de alcătuire a bazei de date]
  • 140 Singh S, Collins BF, Sharma BB. et al. Interstitial lung disease in India. Results of a prospective registry. Am J Respir Crit Care Med 2017; 195 (06) 801-813
  • 141 Singh S, Collins BF, Sharma BB. et al. Hypersensitivity pneumonitis: clinical manifestations - prospective data from the interstitial lung disease-India registry. Lung India 2019; 36 (06) 476-482
  • 142 Seed MJ, Agius RM. Progress with structure-activity relationship modelling of occupational chemical respiratory sensitizers. Curr Opin Allergy Clin Immunol 2017; 17 (02) 64-71
  • 143 Salisbury ML, Gross BH, Chughtai A. et al. Development and validation of a radiological diagnosis model for hypersensitivity pneumonitis. Eur Respir J 2018; 52 (02) 1800443
  • 144 Lalancette M, Carrier G, Laviolette M. et al. Farmer's lung. Long-term outcome and lack of predictive value of bronchoalveolar lavage fibrosing factors. Am Rev Respir Dis 1993; 148 (01) 216-221
  • 145 Baqir M, White D, Ryu JH. Emphysematous changes in hypersensitivity pneumonitis: a retrospective analysis of 12 patients. Respir Med Case Rep 2018; 24: 25-29
  • 146 Miyazaki Y, Tateishi T, Akashi T, Ohtani Y, Inase N, Yoshizawa Y. Clinical predictors and histologic appearance of acute exacerbations in chronic hypersensitivity pneumonitis. Chest 2008; 134 (06) 1265-1270
  • 147 Fink JN, Ortega HG, Reynolds HY. et al. Needs and opportunities for research in hypersensitivity pneumonitis. Am J Respir Crit Care Med 2005; 171 (07) 792-798
  • 148 Richerson HB, Bernstein IL, Fink JN. et al. Guidelines for the clinical evaluation of hypersensitivity pneumonitis. Report of the Subcommittee on Hypersensitivity Pneumonitis. J Allergy Clin Immunol 1989; 84 (5, Pt 2): 839-844
  • 149 Fernández Pérez ER, Kong AM, Raimundo K, Koelsch TL, Kulkarni R, Cole AL. Epidemiology of hypersensitivity pneumonitis among an insured population in the United States: a claims-based cohort analysis. Ann Am Thorac Soc 2018; 15 (04) 460-469
  • 150 Wells AU, Flaherty KR, Brown KK. et al; INBUILD trial investigators. Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. Lancet Respir Med 2020; 8 (05) 453-460
  • 151 Wang BR, Edwards R, Freiheit EA. et al. The Pulmonary Fibrosis Foundation Patient Registry. Rationale, design, and methods. Ann Am Thorac Soc 2020; 17 (12) 1620-1628
  • 152 Fernández Pérez ER, Travis WD, Lynch DA. et al. Diagnosis and evaluation of hypersensitivity pneumonitis: CHEST Guideline and Expert Panel Report. Chest 2021; 160 (02) e97-e156
  • 153 Hansell DM, Wells AU, Padley SP, Müller NL. Hypersensitivity pneumonitis: correlation of individual CT patterns with functional abnormalities. Radiology 1996; 199 (01) 123-128
  • 154 Okada F, Ando Y, Yoshitake S. et al. Clinical/pathologic correlations in 553 patients with primary centrilobular findings on high-resolution CT scan of the thorax. Chest 2007; 132 (06) 1939-1948
  • 155 Chung MH, Edinburgh KJ, Webb EM, McCowin M, Webb WR. Mixed infiltrative and obstructive disease on high-resolution CT: differential diagnosis and functional correlates in a consecutive series. J Thorac Imaging 2001; 16 (02) 69-75
  • 156 Morisset J, Johannson KA, Jones KD. et al; HP Delphi Collaborators. Identification of diagnostic criteria for chronic hypersensitivity pneumonitis: an international modified Delphi survey. Am J Respir Crit Care Med 2018; 197 (08) 1036-1044
  • 157 Barnett J, Molyneaux PL, Rawal B. et al. Variable utility of mosaic attenuation to distinguish fibrotic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis. Eur Respir J 2019; 54 (01) 1900531
  • 158 Chung JH, Zhan X, Cao M. et al. Presence of air trapping and mosaic attenuation on chest computed tomography predicts survival in chronic hypersensitivity pneumonitis. Ann Am Thorac Soc 2017; 14 (10) 1533-1538
  • 159 Salisbury ML, Gu T, Murray S. et al. Hypersensitivity pneumonitis: radiologic phenotypes are associated with distinct survival time and pulmonary function trajectory. Chest 2019; 155 (04) 699-711
  • 160 Chung JH, Montner SM, Adegunsoye A. et al. CT findings associated with survival in chronic hypersensitivity pneumonitis. Eur Radiol 2017; 27 (12) 5127-5135
  • 161 Lynch DA, Newell JD, Logan PM, King Jr. TE, Müller NL. Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis?. AJR Am J Roentgenol 1995; 165 (04) 807-811
  • 162 Adler BD, Padley SP, Müller NL, Remy-Jardin M, Remy J. Chronic hypersensitivity pneumonitis: high-resolution CT and radiographic features in 16 patients. Radiology 1992; 185 (01) 91-95
  • 163 Walsh SL, Wells AU, Sverzellati N. et al. Relationship between fibroblastic foci profusion and high resolution CT morphology in fibrotic lung disease. BMC Med 2015; 13: 241
  • 164 Sahin H, Brown KK, Curran-Everett D. et al. Chronic hypersensitivity pneumonitis: CT features comparison with pathologic evidence of fibrosis and survival. Radiology 2007; 244 (02) 591-598
  • 165 Chiba S, Tsuchiya K, Akashi T. et al. Chronic hypersensitivity pneumonitis with a usual interstitial pneumonia-like pattern: correlation between histopathologic and clinical findings. Chest 2016; 149 (06) 1473-1481
  • 166 Buendia-Roldan I, Aguilar-Duran H, Johannson KA, Selman M. Comparing the performance of two recommended criteria for establishing a diagnosis for hypersensitivity pneumonitis. Am J Respir Crit Care Med 2021; 204 (07) 865-868
  • 167 Ley B, Newton CA, Arnould I. et al. The MUC5B promoter polymorphism and telomere length in patients with chronic hypersensitivity pneumonitis: an observational cohort-control study. Lancet Respir Med 2017; 5 (08) 639-647
  • 168 Newton CA, Batra K, Torrealba J. et al. Telomere-related lung fibrosis is diagnostically heterogeneous but uniformly progressive. Eur Respir J 2016; 48 (06) 1710-1720
  • 169 Okamoto T, Miyazaki Y, Tomita M, Tamaoka M, Inase N. A familial history of pulmonary fibrosis in patients with chronic hypersensitivity pneumonitis. Respiration 2013; 85 (05) 384-390
  • 170 Jacob J, Odink A, Brun AL. et al. Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis. Respir Med 2018; 138: 95-101
  • 171 Soumagne T, Chardon ML, Dournes G. et al. Emphysema in active farmer's lung disease. PLoS One 2017; 12 (06) e0178263