Download PDF
Semin Respir Crit Care Med
DOI: 10.1055/s-0044-1785674
Review Article

Diagnostic Approach to Interstitial Lung Diseases Associated with Connective Tissue Diseases

Ana C. Zamora
1   Division of Pulmonary and Sleep Medicine, Department of Medicine, Mayo Clinic Arizona, Scottsdale, Arizona
,
Lewis J. Wesselius
1   Division of Pulmonary and Sleep Medicine, Department of Medicine, Mayo Clinic Arizona, Scottsdale, Arizona
,
Michael B. Gotway
2   Division of Cardiothoracic Radiology, Department of Radiology, Mayo Clinic Arizona, Scottsdale, Arizona
,
Henry D. Tazelaar
3   Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic Arizona, Scottsdale, Arizona
,
Alejandro Diaz-Arumir
1   Division of Pulmonary and Sleep Medicine, Department of Medicine, Mayo Clinic Arizona, Scottsdale, Arizona
,
Vivek Nagaraja
4   Division of Rheumatology, Department of Medicine, Mayo Clinic Arizona, Scottsdale, Arizona
› Author Affiliations
› Further Information
    Permissions and Reprints

Abstract

Interstitial lung disorders are a group of respiratory diseases characterized by interstitial compartment infiltration, varying degrees of infiltration, and fibrosis, with or without small airway involvement. Although some are idiopathic (e.g., idiopathic pulmonary fibrosis, idiopathic interstitial pneumonias, and sarcoidosis), the great majority have an underlying etiology, such as systemic autoimmune rheumatic disease (SARD, also called Connective Tissue Diseases or CTD), inhalational exposure to organic matter, medications, and rarely, genetic disorders. This review focuses on diagnostic approaches in interstitial lung diseases associated with SARDs. To make an accurate diagnosis, a multidisciplinary, personalized approach is required, with input from various specialties, including pulmonary, rheumatology, radiology, and pathology, to reach a consensus. In a minority of patients, a definitive diagnosis cannot be established. Their clinical presentations and prognosis can be variable even within subsets of SARDs.

Keywords

systemic autoimmune rheumatic diseases - interstitial lung diseases - screening - diagnosis - pulmonary function tests - high-resolution computed tomography - artificial intelligence


Publication History

Article published online:
17 April 2024

© 2024. Thieme. All rights reserved.

Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA

 

  • References

  • 1 Mira-Avendano I, Abril A, Burger CD. et al. Interstitial lung disease and other pulmonary manifestations in connective tissue diseases. Mayo Clin Proc 2019; 94 (02) 309-325
    CrossrefPubMedGoogle Scholar
  • 2 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
    CrossrefPubMedGoogle Scholar
  • 3 Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest 2010; 138 (02) 251-256
    CrossrefPubMedGoogle Scholar
  • 4 Homma Y, Ohtsuka Y, Tanimura K. et al. Can interstitial pneumonia as the sole presentation of collagen vascular diseases be differentiated from idiopathic interstitial pneumonia?. Respiration 1995; 62 (05) 248-251
    CrossrefPubMedGoogle Scholar
  • 5 Kinder BW, Collard HR, Koth L. et al. Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease?. Am J Respir Crit Care Med 2007; 176 (07) 691-697
    CrossrefPubMedGoogle Scholar
  • 6 Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest 2013; 143 (03) 814-824
    CrossrefPubMedGoogle Scholar
  • 7 American College of Rheumatology. Interstitial Lung Disease Guideline. Available at: https://rheumatology.org/interstitial-lung-disease-guideline . Accessed February 25, 2024
    PubMed
  • 8 Fischer A, Antoniou KM, Brown KK. et al. “ERS/ATS Task Force on Undifferentiated Forms of CTD-ILD”. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J 2015; 46 (04) 976-987
    CrossrefPubMedGoogle Scholar
  • 9 Huapaya JA, Boulougoura A, Fried J. et al. Long-term evaluation of pulmonary function and survival of patients with interstitial pneumonia with autoimmune features. Clin Exp Rheumatol 2023; 41 (01) 15-23
    PubMedGoogle Scholar
  • 10 Joerns EK, Adams TN, Newton CA. et al. Variables associated with response to therapy in patients with interstitial pneumonia with autoimmune features. J Clin Rheumatol 2022; 28 (02) 84-88
    CrossrefPubMedGoogle Scholar
  • 11 Hoffmann-Vold AM, Maher TM, Philpot EE. et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. Lancet Rheumatol 2020; 2 (02) e71-e83
    CrossrefPubMedGoogle Scholar
  • 12 Lee AS, Ryu JH. Aspiration pneumonia and related syndromes. Mayo Clin Proc 2018; 93 (06) 752-762
    CrossrefPubMedGoogle Scholar
  • 13 Salvioli B, Belmonte G, Stanghellini V. et al. Gastro-oesophageal reflux and interstitial lung disease. Dig Liver Dis 2006; 38 (12) 879-884
    CrossrefPubMedGoogle Scholar
  • 14 Cottin V, Cordier JF. Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis?. Eur Respir J 2012; 40 (03) 519-521
    CrossrefPubMedGoogle Scholar
  • 15 Manfredi A, Cassone G, Vacchi C. et al. Usefulness of digital velcro crackles detection in identification of interstitial lung disease in patients with connective tissue diseases. Arch Rheumatol 2020; 36 (01) 19-25
    PubMedGoogle Scholar
  • 16 Steele R, Hudson M, Lo E, Baron M. Canadian Scleroderma Research Group. Clinical decision rule to predict the presence of interstitial lung disease in systemic sclerosis. Arthritis Care Res (Hoboken) 2012; 64 (04) 519-524
    CrossrefPubMedGoogle Scholar
  • 17 Schraufnagel DEMF. History and physical examination. In: Murray and Nadel's Textbook of Respiratory Medicine. 5th ed.. Elsevier Saunders; 2010
    Google Scholar
  • 18 Shiraishi K, Jinta T, Nishimura N. et al. Digital clubbing is associated with higher serum KL-6 levels and lower pulmonary function in patients with interstitial lung disease. Can Respir J 2018; 2018: 3640967
    CrossrefPubMedGoogle Scholar
  • 19 van Manen MJG, Vermeer LC, Moor CC. et al. Clubbing in patients with fibrotic interstitial lung diseases. Respir Med 2017; 132: 226-231
    CrossrefPubMedGoogle Scholar
  • 20 Aparicio IJ, Lee JS. Connective tissue disease-associated interstitial lung diseases: unresolved issues. Semin Respir Crit Care Med 2016; 37 (03) 468-476
    Article in Thieme ConnectPubMedGoogle Scholar
  • 21 Umashankar E, Abdel-Shaheed C, Plit M, Girgis L. Assessing the role for nailfold videocapillaroscopy in interstitial lung disease classification: a systematic review and meta-analysis. Rheumatology (Oxford) 2022; 61 (06) 2221-2234
    CrossrefPubMedGoogle Scholar
  • 22 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
    CrossrefPubMedGoogle Scholar
  • 23 Gibson GJ, Pride NB, Davis J, Schroter RC. Exponential description of the static pressure-volume curve of normal and diseased lungs. Am Rev Respir Dis 1979; 120 (04) 799-811
    PubMedGoogle Scholar
  • 24 Gottlieb D, Snider G. Lung function in pulmonary fibrosis. In: Phan S, Thrall R. eds. Lung Biology in Health and Disease: Pulmonary Fibrosis. New York: Marcel Dekker; 1995: 85-135
    Google Scholar
  • 25 Arcadu A, Ryu JH. Constrictive (obliterative) bronchiolitis as presenting manifestation of connective tissue diseases. J Clin Rheumatol 2020; 26 (05) 176-180
    CrossrefPubMedGoogle Scholar
  • 26 Radovanovic D, Santus P, Blasi F. et al. A comprehensive approach to lung function in bronchiectasis. Respir Med 2018; 145: 120-129
    CrossrefPubMedGoogle Scholar
  • 27 Vergnenègre A, Pugnere N, Antonini MT. et al. Airway obstruction and rheumatoid arthritis. Eur Respir J 1997; 10 (05) 1072-1078
    CrossrefPubMedGoogle Scholar
  • 28 Steen VD, Graham G, Conte C, Owens G, Medsger Jr TA. Isolated diffusing capacity reduction in systemic sclerosis. Arthritis Rheum 1992; 35 (07) 765-770
    CrossrefPubMedGoogle Scholar
  • 29 Nathan SD, Shlobin OA, Ahmad S, Urbanek S, Barnett SD. Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary fibrosis. Chest 2007; 131 (03) 657-663
    CrossrefPubMedGoogle Scholar
  • 30 Jacob J, Song JW, Yoon HY. et al. Prevalence and effects of emphysema in never-smokers with rheumatoid arthritis interstitial lung disease. EBioMedicine 2018; 28: 303-310
    CrossrefPubMedGoogle Scholar
  • 31 Koo BS, Park KY, Lee HJ. et al. Effect of combined pulmonary fibrosis and emphysema on patients with connective tissue diseases and systemic sclerosis: a systematic review and meta-analysis. Arthritis Res Ther 2021; 23 (01) 100
    CrossrefPubMedGoogle Scholar
  • 32 Suliman YA, Dobrota R, Huscher D. et al. Brief report: Pulmonary function tests: high rate of false-negative results in the early detection and screening of scleroderma-related interstitial lung disease. Arthritis Rheumatol 2015; 67 (12) 3256-3261
    CrossrefPubMedGoogle Scholar
  • 33 Raghu G, Remy-Jardin M, Richeldi L. et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2022; 205 (09) e18-e47
    CrossrefPubMedGoogle Scholar
  • 34 Goh NS, Hoyles RK, Denton CP. et al. Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheumatol 2017; 69 (08) 1670-1678
    CrossrefPubMedGoogle Scholar
  • 35 Caron M, Hoa S, Hudson M, Schwartzman K, Steele R. Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease. Eur Respir Rev 2018; 27 (148) 170102
    CrossrefPubMedGoogle Scholar
  • 36 Guler SA, Winstone TA, Murphy D. et al. Does systemic sclerosis–associated interstitial lung disease burn out? Specific phenotypes of disease progression. Ann Am Thorac Soc 2018; 15 (12) 1427-1433
    CrossrefPubMedGoogle Scholar
  • 37 Zamora-Legoff JA, Krause ML, Crowson CS, Ryu JH, Matteson EL. Progressive decline of lung function in rheumatoid arthritis–associated interstitial lung disease. Arthritis Rheumatol 2017; 69 (03) 542-549
    CrossrefPubMedGoogle Scholar
  • 38 Assayag D, Lubin M, Lee JS, King TE, Collard HR, Ryerson CJ. Predictors of mortality in rheumatoid arthritis-related interstitial lung disease. Respirology 2014; 19 (04) 493-500
    CrossrefPubMedGoogle Scholar
  • 39 Chang SH, Lee JS, Ha Y-J. et al. Lung function trajectory of rheumatoid arthritis-associated interstitial lung disease. Rheumatology (Oxford) 2023; 62 (09) 3014-3024
    CrossrefPubMedGoogle Scholar
  • 40 Kalluri M, Oddis CV. Pulmonary manifestations of the idiopathic inflammatory myopathies. Clin Chest Med 2010; 31 (03) 501-512
    CrossrefPubMedGoogle Scholar
  • 41 Villalba WO, Sampaio-Barros PD, Pereira MC. et al. Six-minute walk test for the evaluation of pulmonary disease severity in scleroderma patients. Chest 2007; 131 (01) 217-222
    CrossrefPubMedGoogle Scholar
  • 42 Saleem F, Ryerson CJ, Sarma N. et al. Predicting new-onset exertional and resting hypoxemia in fibrotic interstitial lung disease. Ann Am Thorac Soc 2023; 20 (12) 1726-1734
    CrossrefPubMedGoogle Scholar
  • 43 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
    CrossrefPubMedGoogle Scholar
  • 44 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
    CrossrefPubMedGoogle Scholar
  • 45 Assayag D, Elicker BM, Urbania TH. et al. Rheumatoid arthritis-associated interstitial lung disease: radiologic identification of usual interstitial pneumonia pattern. Radiology 2014; 270 (02) 583-588
    CrossrefPubMedGoogle Scholar
  • 46 Henry TS, Little BP, Veeraraghavan S, Bhalla S, Elicker BM. The spectrum of interstitial lung disease in connective tissue disease. J Thorac Imaging 2016; 31 (02) 65-77
    CrossrefPubMedGoogle Scholar
  • 47 Lee H-K, Kim DS, Yoo B. et al. Histopathologic pattern and clinical features of rheumatoid arthritis-associated interstitial lung disease. Chest 2005; 127 (06) 2019-2027
    CrossrefPubMedGoogle Scholar
  • 48 Moua T, Zamora Martinez AC, Baqir M, Vassallo R, Limper AH, Ryu JH. Predictors of diagnosis and survival in idiopathic pulmonary fibrosis and connective tissue disease-related usual interstitial pneumonia. Respir Res 2014; 15 (01) 154
    CrossrefPubMedGoogle Scholar
  • 49 Kligerman SJ, Groshong S, Brown KK, Lynch DA. Nonspecific interstitial pneumonia: radiologic, clinical, and pathologic considerations. Radiographics 2009; 29 (01) 73-87
    CrossrefPubMedGoogle Scholar
  • 50 Bouros D, Wells AU, Nicholson AG. et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 2002; 165 (12) 1581-1586
    CrossrefPubMedGoogle Scholar
  • 51 Kim DS, Yoo B, Lee JS. et al. The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 2002; 19 (02) 121-127
    PubMedGoogle Scholar
  • 52 Debray M-P, Borie R, Revel M-P. et al. Interstitial lung disease in anti-synthetase syndrome: initial and follow-up CT findings. Eur J Radiol 2015; 84 (03) 516-523
    CrossrefPubMedGoogle Scholar
  • 53 Fraune C, Churg A, Yi ES. et al. Lymphoid interstitial pneumonia (LIP) revisited: a critical reappraisal of the histologic spectrum of “radiologic” and “pathologic” LIP in the context of diffuse benign lymphoid proliferations of the lung. Am J Surg Pathol 2023; 47 (03) 281-295
    CrossrefPubMedGoogle Scholar
  • 54 Chung JH, Cox CW, Montner SM. et al. CT features of the usual interstitial pneumonia pattern: differentiating connective tissue disease–associated interstitial lung disease from idiopathic pulmonary fibrosis. AJR Am J Roentgenol 2018; 210 (02) 307-313
    CrossrefPubMedGoogle Scholar
  • 55 Walkoff L, White DB, Chung JH, Asante D, Cox CW. The Four Corners Sign: a specific imaging feature in differentiating systemic sclerosis-related interstitial lung disease from idiopathic pulmonary fibrosis. J Thorac Imaging 2018; 33 (03) 197-203
    CrossrefPubMedGoogle Scholar
  • 56 Brady D, Berkowitz EA, Sharma A. et al. CT morphologic characteristics and variant patterns of interstitial pulmonary fibrosis in systemic lupus erythematosus. Radiol Cardiothorac Imaging 2021; 3 (04) e200625
    CrossrefPubMedGoogle Scholar
  • 57 Remy-Jardin M, Ryerson CJ, Schiebler ML. et al. Imaging of pulmonary hypertension in adults: a position paper from the Fleischner Society. Eur Respir J 2021; 57 (01) 2004455
    CrossrefPubMedGoogle Scholar
  • 58 Capobianco J, Grimberg A, Thompson BM, Antunes VB, Jasinowodolinski D, Meirelles GS. Thoracic manifestations of collagen vascular diseases. Radiographics 2012; 32 (01) 33-50
    CrossrefPubMedGoogle Scholar
  • 59 Goh NS, Desai SR, Veeraraghavan S. et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 2008; 177 (11) 1248-1254
    CrossrefPubMedGoogle Scholar
  • 60 Wells AU. Interstitial lung disease in systemic sclerosis. Presse Med 2014; 43 (10 Pt 2): e329-e343
    CrossrefPubMedGoogle Scholar
  • 61 Suman G, Koo CW. Recent advancements in computed tomography assessment of fibrotic interstitial lung diseases. J Thorac Imaging 2023; 38 (Suppl. 01) S7-S18
    CrossrefPubMedGoogle Scholar
  • 62 Chung JH, Adegunsoye A, Cannon B. et al. Differentiation of idiopathic pulmonary fibrosis from connective tissue disease-related interstitial lung disease using quantitative imaging. J Clin Med 2021; 10 (12) 2663
    CrossrefPubMedGoogle Scholar
  • 63 Chung JH, Adegunsoye A, Oldham JM. et al. Vessel-related structures predict UIP pathology in those with a non-IPF pattern on CT. Eur Radiol 2021; 31 (10) 7295-7302
    CrossrefPubMedGoogle Scholar
  • 64 Crews MS, Bartholmai BJ, Adegunsoye A. et al. Automated CT analysis of major forms of interstitial lung disease. J Clin Med 2020; 9 (11) 3776
    CrossrefPubMedGoogle Scholar
  • 65 Ohno Y, Aoyagi K, Takenaka D. et al. Machine learning for lung texture analysis on thin-section CT: Capability for assessments of disease severity and therapeutic effect for connective tissue disease patients in comparison with expert panel evaluations. Acta Radiol 2022; 63 (10) 1363-1373
    CrossrefPubMedGoogle Scholar
  • 66 Sun D, Wang Y, Liu Q. et al. Prediction of long-term mortality by using machine learning models in Chinese patients with connective tissue disease-associated interstitial lung disease. Respir Res 2022; 23 (01) 4
    CrossrefPubMedGoogle Scholar
  • 67 Jamal F, Shashi K, Vaz N, Doyle T, Dellaripa P, Hammer M. Quantitative chest computed tomography for progression of interstitial lung disease in antisynthetase patients. J Thorac Imaging 2023; 10: 1097
    PubMedGoogle Scholar
  • 68 Kondoh Y, Makino S, Ogura T. et al. joint committee of Japanese Respiratory Society and Japan College of Rheumatology. 2020 guide for the diagnosis and treatment of interstitial lung disease associated with connective tissue disease. Respir Investig 2021; 59 (06) 709-740
    CrossrefPubMedGoogle Scholar
  • 69 Kowal-Bielecka O, Kowal K, Chyczewska E. Utility of bronchoalveolar lavage in evaluation of patients with connective tissue diseases. Clin Chest Med 2010; 31 (03) 423-431
    CrossrefPubMedGoogle Scholar
  • 70 Jee AS, Sheehy R, Hopkins P. et al. Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: a position statement from the Thoracic Society of Australia and New Zealand. Respirology 2021; 26 (01) 23-51
    CrossrefPubMedGoogle Scholar
  • 71 Hunninghake GW, Zimmerman MB, Schwartz DA. et al. Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2001; 164 (02) 193-196
    CrossrefPubMedGoogle Scholar
  • 72 Hutchinson JP, Fogarty AW, McKeever TM, Hubbard RB. In-hospital mortality after surgical lung biopsy for interstitial lung disease in the United States. 2000 to 2011. Am J Respir Crit Care Med 2016; 193 (10) 1161-1167
    CrossrefPubMedGoogle Scholar
  • 73 Lentz RJ, Argento AC, Colby TV, Rickman OB, Maldonado F. Transbronchial cryobiopsy for diffuse parenchymal lung disease: a state-of-the-art review of procedural techniques, current evidence, and future challenges. J Thorac Dis 2017; 9 (07) 2186-2203
    CrossrefPubMedGoogle Scholar
  • 74 Maldonado F, Danoff SK, Wells AU. et al. Transbronchial cryobiopsy for the diagnosis of interstitial lung diseases: CHEST Guideline and Expert Panel Report. Chest 2020; 157 (04) 1030-1042
    CrossrefPubMedGoogle Scholar
  • 75 Troy LK, Grainge C, Corte TJ. et al. Cryobiopsy versus Open Lung biopsy in the Diagnosis of Interstitial lung disease alliance (COLDICE) Investigators. Diagnostic accuracy of transbronchial lung cryobiopsy for interstitial lung disease diagnosis (COLDICE): a prospective, comparative study. Lancet Respir Med 2020; 8 (02) 171-181
    CrossrefPubMedGoogle Scholar
  • 76 Schneider F, Gruden J, Tazelaar HD, Leslie KO. Pleuropulmonary pathology in patients with rheumatic disease. Arch Pathol Lab Med 2012; 136 (10) 1242-1252
    CrossrefPubMedGoogle Scholar
  • 77 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
    CrossrefPubMedGoogle Scholar
  • 78 Glenn LM, Troy LK, Corte TJ. Diagnosing interstitial lung disease by multidisciplinary discussion: a review. Front Med (Lausanne) 2022; 9: 1017501
    CrossrefPubMedGoogle Scholar
  • 79 Jo HE, Glaspole IN, Levin KC. et al. Clinical impact of the interstitial lung disease multidisciplinary service. Respirology 2016; 21 (08) 1438-1444
    CrossrefPubMedGoogle Scholar
  • 80 Teoh AKY, Holland AE, Morisset J. et al. ILD MDM Delphi Collaborators. Essential features of an interstitial lung disease multidisciplinary meeting: An International Delphi Survey. Ann Am Thorac Soc 2022; 19 (01) 66-73
    CrossrefPubMedGoogle Scholar
  • 81 De Sadeleer LJ, Meert C, Yserbyt J. et al. Diagnostic ability of a dynamic multidisciplinary discussion in interstitial lung diseases: a retrospective observational study of 938 cases. Chest 2018; 153 (06) 1416-1423
    CrossrefPubMedGoogle Scholar
  • 82 Walsh SLF, Wells AU, Desai SR. et al. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study. Lancet Respir Med 2016; 4 (07) 557-565
    CrossrefPubMedGoogle Scholar
  • 83 Richeldi L, Launders N, Martinez F. et al. The characterisation of interstitial lung disease multidisciplinary team meetings: a global study. ERJ Open Res 2019; 5 (02) 00209-2018
    CrossrefPubMedGoogle Scholar
  • 84 Bosello SL, Beretta L, Del Papa N. et al. Interstitial lung disease associated with autoimmune rheumatic diseases: checklists for clinical practice. Front Med (Lausanne) 2021; 8: 732761
    CrossrefPubMedGoogle Scholar
  • 85 De Lorenzis E, Bosello SL, Varone F. et al. Multidisciplinary evaluation of interstitial lung diseases: new opportunities linked to rheumatologist involvement. Diagnostics (Basel) 2020; 10 (09) 664
    CrossrefPubMedGoogle Scholar
  • 86 Khanna D, Mittoo S, Aggarwal R. et al. Connective tissue disease-associated interstitial lung diseases (CTD-ILD)—report from OMERACT CTD-ILD Working Group. J Rheumatol 2015; 42 (11) 2168-2171
    CrossrefPubMedGoogle Scholar
  • 87 Mittoo S, Frankel S, LeSage D. et al. Patient perspectives in OMERACT provide an anchor for future metric development and improved approaches to healthcare delivery in connective tissue disease related interstitial lung disease (CTD-ILD). Curr Respir Med Rev 2015; 11 (02) 175-183
    CrossrefPubMedGoogle Scholar
  • 88 Swigris JJ, Brown KK, Abdulqawi R. et al. Patients' perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases. Eur Respir Rev 2018; 27 (150) 180075
    CrossrefPubMedGoogle Scholar
  • 89 Belkin A, Albright K, Swigris JJ. A qualitative study of informal caregivers' perspectives on the effects of idiopathic pulmonary fibrosis. BMJ Open Respir Res 2014; 1 (01) e000007
    CrossrefPubMedGoogle Scholar
  • 90 Jokerst C, Purdy H, Bhalla S. An overview of collagen vascular disease-associated interstitial lung disease. Semin Roentgenol 2015; 50 (01) 31-39
    CrossrefPubMedGoogle Scholar
  • 91 Zanatta E, Cocconcelli E, Castelli G. et al. Interstitial lung disease with and without progressive fibrosing phenotype in patients with idiopathic inflammatory myopathies: data from a large multicentric cohort. RMD Open 2023; 9 (03) e003121
    CrossrefPubMedGoogle Scholar
  • 92 Egashira R, Kondo T, Hirai T. et al. CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation. Radiographics 2013; 33 (07) 1933-1949
    CrossrefPubMedGoogle Scholar