Multidisciplinary Evaluation in Patients with Lung Disease Associated with Connective Tissue Disease
Open accessAbstract
Multidisciplinary diagnosis is now viewed as the diagnostic reference standard in interstitial lung disease (ILD). This process consists of the integration of the evidence base with clinical reasoning in the formulation of a diagnosis and requires input from clinicians, radiologists, and, in selected cases, histopathologists. In ILD associated with connective tissue disease (CTD-ILD), multidisciplinary evaluation is especially helpful when CTD is suspected but cannot be diagnosed using strict criteria. In this context, the integration of systemic clinical data, serologic information, and computed tomography and biopsy findings may allow CTD-ILD to be diagnosed. However, the value of multidisciplinary evaluation in CTD-ILD is not confined to diagnosis. The frequent coexistence of pulmonary processes other than ILD, including pulmonary vascular disease, extrapulmonic restriction, and airways disease, often has a major impact on symptoms and pulmonary function tests (PFTs). In this review, we highlight the value of multidisciplinary discussion (MDD) in reconciling clinical data, PFT, and imaging data in the accurate staging of disease severity, baseline prognostic evaluation, and the identification of progression of ILD. MDD also provides a means to combine the views of respiratory physicians and rheumatologists in formulating a treatment strategy. It is often possible to reach a robust view as to whether management should be driven by systemic disease, pulmonary disease, or both. When treatment needs to be introduced or modified for both systemic and pulmonary reasons, face-to-face discussion facilities the selection of therapeutic agents that are likely to be efficacious for both systemic and pulmonary diseases.
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When a patient's condition presents with ambiguous or overlapping symptoms, which can be difficult to distinguish from one another, this may lead to consultations with different specialists and referrals to arrive at a final diagnosis.
“When a patient’s condition presents with ambiguous or overlapping symptoms, which can be difficult to distinguish from one another, this may lead to consultations with different specialists and referrals to arrive at a final diagnosis [272,273].”
From Toward Safer Diagnoses: A SEIPS-Based Narrative Review of Diagnostic Errors (2026) · cites this paper as [272]
The section “Diagnosis of CTD-ILD: The Role of MDD” says that uncertain diagnoses benefit from discussion among ILD physicians, CT radiologists, and rheumatologists. This supports the role of multiple specialists when a diagnosis is difficult, but the paper addresses CTD-associated lung disease specifically; it does not describe a general pattern of patients receiving referrals and separate consultations before a final diagnosis. Page numbers were not available in our copy. (LLM, conf=0.82)
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Cites
Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis
Richeldi, L., du Bois, R. M., Raghu, G., Azuma, A., Brown, K. K., Costabel, U., Cottin, V., Flaherty, K. R., Hansell, D. M., Inoue, Y., et al. (2014) · New England Journal of Medicine · doi:10.1056/nejmoa1402584 · Cited by 4,654 · see citing works
A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis.
King, T. E., Bradford, W. Z., Castro-Bernardini, S., Fagan, E. A., Glaspole, I., Glassberg, M. K., Gorina, E., Hopkins, P. M., Kardatzke, D., Lancaster, L., et al. (2014) · New England Journal of Medicine · doi:10.1056/nejmoa1402582 · Cited by 3,975 · see citing works
Flagged first, then by citations
Research works printed but not linked: 42
- 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 Review
- Tansey D, Wells AU, Colby TV, et al. Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology 2004;44(06):585-596
- 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
- Park JH, Kim DS, Park IN, et al. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med 2007;175(07):705-711
- Tsuchiya Y, Takayanagi N, Sugiura H, et al. Lung diseases directly associated with rheumatoid arthritis and their relationship to outcome. Eur Respir J 2011;37(06):1411-1417
- 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;15:154
- Kim EJ, Elicker BM, Maldonado F, et al. Usual interstitial pneu-monia in rheumatoid arthritis- associated interstitial lung dis-ease. Eur Respir J 2010;35(06):1322-1328
- Kelly CA, Saravanan V, Nisar M, et al; British Rheumatoid Inter-stitial Lung (BRILL) Network. Rheumatoid arthritis-related inter-stitial lung disease: associations, prognostic factors and physiological and radiological characteristics-a large multicentre UK study. Rheumatology (Oxford) 2014;53(09):1676-1682
- Yunt ZX, Chung JH, Hobbs S, et al. High resolution computed tomography pattern of usual interstitial pneumonia in rheuma-toid arthritis-associated interstitial lung disease: relationship to survival. Respir Med 2017;126:100-104
- Douglas WW, Tazelaar HD, Hartman TE, et al. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 2001;164(07):1182-1185
- Ito I, Nagai S, Kitaichi M, et al. Pulmonary manifestations of primary Sjogren's syndrome: a clinical, radiologic, and pathologic study. Am J Respir Crit Care Med 2005;171(06):632-638
- Castillo D, Walsh S, Hansell DM, et al; ERICE ILD working group. Validation of multidisciplinary diagnosis in IPF. Lancet Respir Med 2018;6(02):88-89
- Walsh SLF, Wells AU, Desai SR, et al. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in dif-fuse parenchymal lung disease: a case-cohort study. Lancet Respir Med 2016;4(07):557-565
- 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 state-ment: interstitial pneumonia with autoimmune features. Eur Respir J 2015;46(04):976-987
- Luppi F, Wells AU. Interstitial pneumonitis with autoimmune features (IPAF): a work in progress. Eur Respir J 2016;47(06): 1622-1624
- Oldham JM, Adegunsoye A, Valenzi E, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J 2016;47(06):1767-1775
- Gibson GJ. Clinical Tests of Respiratory Function. London: Chap-pell and Hall; 1996:223-224
- Wells AU, Hansell DM, Rubens MB, Cailes JB, Black CM, du Bois RM. Functional impairment in lone cryptogenic fibrosing alveolitis and fibrosing alveolitis associated with systemic sclerosis: a comparison. Am J Respir Crit Care Med 1997;155 (05):1657-1664 21
- Cordier J-F. Organising pneumonia. Thorax 2000;55(04):318-328
- Burke CM, Glanville AR, Morris AJ, et al. Pulmonary function in advanced pulmonary hypertension. Thorax 1987;42(02): 131-135
- 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
- Nathan SD, Shlobin OA, Ahmad S, Urbanek S, Barnett SD. Pul-monary hypertension and pulmonary function testing in idio-pathic pulmonary fibrosis. Chest 2007;131(03):657-663
- Steen VD, Graham G, Conte C, Owens G, Medsger TA Jr. Isolated diffusing capacity reduction in systemic sclerosis. Arthritis Rheum 1992;35(07):765-770
- Broderick A, Fuortes LJ, Merchant JA, Galvin JR, Schwartz DA. Pleural determinants of restrictive lung function and respiratory symptoms in an asbestos-exposed population. Chest 1992;101 (03):684-691
- Wright PH, Hanson A, Kreel L, Capel LH. Respiratory function changes after asbestos pleurisy. Thorax 1980;35(01):31-36
- Vincken WG, Elleker MG, Cosio MG. Flow-volume loop changes reflecting respiratory muscle weakness in chronic neuromuscular disorders. Am J Med 1987;83(04):673-680
- Braun NM, Arora NS, Rochester DF. Respiratory muscle and pulmonary function in polymyositis and other proximal myopa-thies. Thorax 1983;38(08):616-623
- Gibson GJ, Pride NB, Davis JN, Loh LC. Pulmonary mechanics in patients with respiratory muscle weakness. Am Rev Respir Dis 1977;115(03):389-395
- Gould GA, Redpath AT, Ryan M, et al. Lung CT density correlates with measurements of airflow limitation and the diffusing capa-city. Eur Respir J 1991;4(02):141-146
- Hansell DM, Rubens MB, Padley SPG, Wells AU. Obliterative bronchiolitis: individual CT signs of small airways disease and functional correlation. Radiology 1997;203(03):721-726
- Wells AU, King AD, Rubens MB, Cramer D, du Bois RM, Hansell DM. Lone cryptogenic fibrosing alveolitis: a functional-morphologic correlation based on extent of disease on thin-section computed tomography. Am J Respir Crit Care Med 1997;155(04):1367-1375
- Antoniou KM, Margaritopoulos GA, Goh NS, et al. Combined pulmonary fibrosis and emphysema in scleroderma-related lung disease has a major confounding effect on lung physiology and screening for pulmonary hypertension. Arthritis Rheumatol 2016;68(04):1004-1012
- Jacob J, Song JW, Yoon HY, et al. Prevalence and effects of emphysema in never-smokers with rheumatoid arthritis inter-stitial lung disease. EBioMedicine 2018;28:303-310
- Devaraj A, Wells AU, Meister MG, Corte TJ, Wort SJ, Hansell DM. Detection of pulmonary hypertension with multidetector CT and echocardiography alone and in combination. Radiology 2010;254 (02):609-616
- Shin S, King CS, Puri N, et al. Pulmonary artery size as a predictor of outcomes in idiopathic pulmonary fibrosis. Eur Respir J 2016; 47(05):1445-1451
- Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006;129(03):746-752
- Kawut SM, O'Shea MK, Bartels MN, Wilt JS, Sonett JR, Arcasoy SM. Exercise testing determines survival in patients with diffuse parenchymal lung disease evaluated for lung transplantation. Respir Med 2005;99(11):1431-1439
- Leuchte HH, Baumgartner RA, Nounou ME, et al. Brain natriuretic peptide is a prognostic parameter in chronic lung disease. Am J Respir Crit Care Med 2006;173(07):744-750
- Wells AU, Cullinan P, Hansell DM, et al. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 1994;149(06):1583-1590
- Marie I, Hachulla E, Chérin P, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum 2002;47 (06):614-622
- Tashkin DP, Elashoff R, Clements PJ, et al; Scleroderma Lung Study Research Group. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 2006;354(25): 2655-2666
- Ley B, Ryerson CJ, Vittinghoff E, et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Ann Intern Med 2012;156(10):684-691
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Toward Safer Diagnoses: A SEIPS-Based Narrative Review of Diagnostic Errors
Yen, C., Epling, J. W., Rockwell, M., & Vaughn-Cooke, M. (2026) · Diagnostics · doi:10.3390/diagnostics16020347 · Cited by 6 · see citing works
- Authors
- Wells, A., Devaraj, A., Renzoni, E. A., & Denton, C. P.
- Affiliations
- Royal Brompton Hospital (GB)
- Interstitial Lung Disease Unit, Department of Imaging, Royal Brompton Hospital, London, United Kingdom
- The Royal Free Hospital (GB)
- Corresponding author
- Wells, Athol
- Venue
- Seminars in Respiratory and Critical Care Medicine
- Publisher
- Georg Thieme Verlag KG
- Published
- 2019-04
- Volume
- 40
- Issue
- 02
- Pages
- 184-193
- ISSN
- 1069-3424 (ISSN-L)
- 1098-9048
- OpenAlex
- W2946893419
- Cited by
- 36
- References
- 2
- Citations by year
- 2026: 5 · 2025: 3 · 2024: 3 · 2023: 6 · 2022: 10 · 2021: 3 · 2020: 2 · 2019: 4
- FWCI
- 3.49
- Type
- journal article
- Language
- en
- Open access
- green
- discovery.ucl.ac.uk
- Locations
- Seminars in Respiratory and Critical Care Medicine (publishedVersion)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Funders
- Versus Arthritis (20719)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Keywords
- connective tissue disease-associated interstitial lung disease, multidisciplinary assessment, multidisciplinary discussion, disease staging, prognosis, disease progression, pulmonary vascular disease, airway disease, pulmonary function tests, computed tomography, lung biopsy, treatment options
- MeSH terms
- Connective Tissue Diseases, Humans, Prognosis, Lung Diseases, Interstitial, Disease Progression, Interdisciplinary Communication
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