Systemic sclerosis-associated interstitial lung disease: Diagnostic approaches and challenges

Jessica L. Fairley1,2, Nicole S.L. Goh1,3,4, Mandana Nikpour1,2
1Department of Medicine, The University of Melbourne, Melbourne, Australia
2Department of Rheumatology, St. Vincent's Hospital Melbourne, Melbourne, Australia
3Department of Respiratory and Sleep Medicine, Austin Hospital, Melbourne, Australia
4The Institute for Breathing and Sleep, Melbourne, Australia

Tài liệu tham khảo

Bhattacharyya, 2011, Understanding fibrosis in systemic sclerosis: shifting paradigms, emerging opportunities, Nat Rev Rheumatol, 8, 42, 10.1038/nrrheum.2011.149 Rubio-Rivas, 2014, Mortality and survival in systemic sclerosis: systematic review and meta-analysis, Semin Arthritis Rheum, 44, 208, 10.1016/j.semarthrit.2014.05.010 Hao, 2017, Early mortality in a multinational systemic sclerosis inception cohort, Arthritis Rheumatol, 69, 1067, 10.1002/art.40027 Tyndall, 2010, Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database, Ann Rheum Dis, 69, 1809, 10.1136/ard.2009.114264 Denton, 2017, Systemic sclerosis, Lancet, 390, 1685, 10.1016/S0140-6736(17)30933-9 Nihtyanova, 2014, Prediction of pulmonary complications and long-term survival in systemic sclerosis, Arthritis Rheumatol, 66, 1625, 10.1002/art.38390 Schurawitzki, 1990, Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography, Radiology, 176, 755, 10.1148/radiology.176.3.2389033 Bussone, 2011, Interstitial lung disease in systemic sclerosis, Autoimmun Rev, 10, 248, 10.1016/j.autrev.2010.09.012 Steen, 1994, Severe restrictive lung disease in systemic sclerosis, Arthritis Rheum, 37, 1283, 10.1002/art.1780370903 Sanchez-Cano, 2018, Interstitial lung disease in systemic sclerosis: data from the Spanish scleroderma study group, Rheumatol Int, 38, 363, 10.1007/s00296-017-3916-x Liaskos, 2017, Disease-related autoantibody profile in patients with systemic sclerosis, Autoimmunity, 50, 414, 10.1080/08916934.2017.1357699 Khanna, 2020, Etiology, risk factors, and biomarkers in systemic sclerosis with interstitial lung disease, Am J Respir Crit Care Med, 201, 650, 10.1164/rccm.201903-0563CI Steen, 2012, A clinical and serologic comparison of African American and Caucasian patients with systemic sclerosis, Arthritis Rheum, 64, 2986, 10.1002/art.34482 Chung, 2020, Increased mortality in Asians with systemic sclerosis in Northern California, ACR Open Rheumatol, 2, 197, 10.1002/acr2.11126 Fairley, 2023, Clinical characteristics and survival of combined pulmonary arterial hypertension and interstitial lung disease in systemic sclerosis, Arthritis Res Ther, 10.1186/s13075-023-03059-x Christmann, 2010, Gastroesophageal reflux incites interstitial lung disease in systemic sclerosis: clinical, radiologic, histopathologic, and treatment evidence, Semin Arthritis Rheum, 40, 241, 10.1016/j.semarthrit.2010.03.002 Elhai, 2019, Performance of candidate serum biomarkers for systemic sclerosis-associated interstitial lung disease, Arthritis Rheumatol, 71, 972, 10.1002/art.40815 Airo, 2011, Malignancies in Italian patients with systemic sclerosis positive for anti-RNA polymerase III antibodies, J Rheumatol, 38, 1329, 10.3899/jrheum.101144 Hoffmann-Vold, 2017, Cardiopulmonary disease development in anti-RNA polymerase III-positive systemic sclerosis: comparative analyses from an unselected, prospective patient cohort, J Rheumatol, 44, 459, 10.3899/jrheum.160867 Hamaguchi, 2008, The clinical relevance of serum antinuclear antibodies in Japanese patients with systemic sclerosis, Br J Dermatol, 158, 487, 10.1111/j.1365-2133.2007.08392.x Steen, 2005, Autoantibodies in systemic sclerosis, Semin Arthritis Rheum, 35, 35, 10.1016/j.semarthrit.2005.03.005 Rahaghi, 2023, Expert consensus on the management of systemic sclerosis-associated interstitial lung disease, Respir Res, 24, 10.1186/s12931-022-02292-3 Suliman, 2015, 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, 67, 3256, 10.1002/art.39405 Mathieson, 1989, Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography, Radiology, 171, 111, 10.1148/radiology.171.1.2928513 Khanna, 2011, Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung Study Placebo Group, Arthritis Rheum, 63, 3078, 10.1002/art.30467 Moore, 2013, Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease, Rheumatology (Oxford), 52, 155, 10.1093/rheumatology/kes289 Goh, 2008, Interstitial lung disease in systemic sclerosis: a simple staging system, Am J Respir Crit Care Med, 177, 1248, 10.1164/rccm.200706-877OC Launay, 2006, High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis, J Rheumatol, 33, 1789 Hoffmann-Vold, 2015, Predictive value of serial high-resolution computed tomography analyses and concurrent lung function tests in systemic sclerosis, Arthritis Rheumatol, 67, 2205, 10.1002/art.39166 Bouros, 2002, Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome, Am J Respir Crit Care Med, 165, 1581, 10.1164/rccm.2106012 Zhou, 2021, HRCT imaging features of systemic sclerosis-associated interstitial lung disease, J Radiol Oncol, 5, 35, 10.29328/journal.jro.1001036 Tashkin, 2006, Cyclophosphamide versus placebo in scleroderma lung disease, N Engl J Med, 354, 2655, 10.1056/NEJMoa055120 Wells, 2008, High-resolution computed tomography and scleroderma lung disease, Rheumatology (Oxford), 47, v59, 10.1093/rheumatology/ken271 Desai, 2004, CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia, Radiology, 232, 560, 10.1148/radiol.2322031223 Morrisroe, 2020, Incidence, risk factors, and outcomes of cancer in systemic sclerosis, Arthritis Care Res (Hoboken), 72, 1625, 10.1002/acr.24076 Grosse, 2010, CT findings in diseases associated with pulmonary hypertension: a current review, Radiographics, 30, 1753, 10.1148/rg.307105710 Truong, 2012, Reference values for normal pulmonary artery dimensions by noncontrast cardiac computed tomography: the Framingham Heart Study, Circ Cardiovasc Imaging, 5, 147, 10.1161/CIRCIMAGING.111.968610 Smith, 2023, The emerging association between bronchiectasis and systemic sclerosis: assessing prevalence and potential causality, Intern Med J, 53, 1204, 10.1111/imj.15661 Koo, 2021, 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, 23, 100, 10.1186/s13075-021-02494-y Antoniou, 2016, 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, 68, 1004, 10.1002/art.39528 Yamakawa, 2018, Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy?, BMC Pulm Med, 18, 25, 10.1186/s12890-018-0591-y Hoffmann-Vold, 2020, The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements, Lancet Rheumatol, 2, e71, 10.1016/S2665-9913(19)30144-4 Kim, 2020, Using transitional changes on high-resolution computed tomography to monitor the impact of cyclophosphamide or mycophenolate mofetil on systemic sclerosis-related interstitial lung disease, Arthritis Rheumatol, 72, 316, 10.1002/art.41085 Goldin, 2018, Longitudinal changes in quantitative interstitial lung disease on computed tomography after immunosuppression in the Scleroderma Lung Study II, Ann Am Thorac Soc, 15, 1286, 10.1513/AnnalsATS.201802-079OC Khanna, 2022, Diagnosis and monitoring of systemic sclerosis-associated interstitial lung disease using high-resolution computed tomography, J Scleroderma Relat Disord, 7, 168, 10.1177/23971983211064463 2022 Frauenfelder, 2014, Screening for interstitial lung disease in systemic sclerosis: performance of high-resolution CT with limited number of slices: a prospective study, Ann Rheum Dis, 73, 2069, 10.1136/annrheumdis-2014-205637 Mettler, 2008, Effective doses in radiology and diagnostic nuclear medicine: a catalog, Radiology, 248, 254, 10.1148/radiol.2481071451 Larke, 2011, Estimated radiation dose associated with low-dose chest CT of average-size participants in the National Lung Screening Trial, Am J Roentgenol, 197, 1165, 10.2214/AJR.11.6533 2010 Katsura, 2017, Effects of pure and hybrid iterative reconstruction algorithms on high-resolution computed tomography in the evaluation of interstitial lung disease, Eur J Radiol, 93, 243, 10.1016/j.ejrad.2017.06.003 Gould, 2014, Clinical practice. Lung-cancer screening with low-dose computed tomography, N Engl J Med, 371, 1813, 10.1056/NEJMcp1404071 Paks, 2018, Ultralow dose CT for follow-up of solid pulmonary nodules: a pilot single-center study using Bland–Altman analysis, Medicine (Baltimore), 97, e12019, 10.1097/MD.0000000000012019 Pinal-Fernandez, 2015, Pleural irregularity, a new ultrasound sign for the study of interstitial lung disease in systemic sclerosis and antisynthetase syndrome, Clin Exp Rheumatol, 33, S136 Tardella, 2018, Ultrasound B-lines in the evaluation of interstitial lung disease in patients with systemic sclerosis: cut-off point definition for the presence of significant pulmonary fibrosis, Medicine (Baltimore), 97, e0566, 10.1097/MD.0000000000010566 Lavelle, 2017, Pulmonary fibrosis: tissue characterization using late-enhanced MRI compared with unenhanced anatomic high-resolution CT, Diagn Interv Radiol, 23, 106, 10.5152/dir.2016.15331 Mirsadraee, 2016, T1 characteristics of interstitial pulmonary fibrosis on 3T MRI-a predictor of early interstitial change?, Quant Imaging Med Surg, 6, 42 Bentick, 2022, Defining the clinical utility of PET or PET-CT in idiopathic inflammatory myopathies: a systematic literature review, Semin Arthritis Rheum, 57, 152107, 10.1016/j.semarthrit.2022.152107 Moore, 2015, Quantifying change in pulmonary function as a prognostic marker in systemic sclerosis-related interstitial lung disease, Clin Exp Rheumatol, 33, S111 Hoffmann-Vold, 2019, Tracking impact of interstitial lung disease in systemic sclerosis in a complete nationwide cohort, Am J Respir Crit Care Med, 200, 1258, 10.1164/rccm.201903-0486OC Volkmann, 2019, Short-term progression of interstitial lung disease in systemic sclerosis predicts long-term survival in two independent clinical trial cohorts, Ann Rheum Dis, 78, 122, 10.1136/annrheumdis-2018-213708 Kafaja, 2018, Reliability and minimal clinically important differences of forced vital capacity: results from the Scleroderma Lung Studies (SLS-I and SLS-II), Am J Respir Crit Care Med, 197, 644, 10.1164/rccm.201709-1845OC Hoffmann-Vold, 2021, Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database, Ann Rheum Dis, 80, 219, 10.1136/annrheumdis-2020-217455 Morrisroe, 2017, Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension, Arthritis Res Ther, 19, 122, 10.1186/s13075-017-1341-x Humbert, 2012, Early detection and management of pulmonary arterial hypertension, Eur Respir Rev, 21, 306, 10.1183/09059180.00005112 Humbert, 2023, 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension, Eur Respir J, 61, 2200879, 10.1183/13993003.00879-2022 Humbert, 2011, Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival, Arthritis Rheum, 63, 3522, 10.1002/art.30541 Chausow, 1984, Reversible hypercapnic respiratory insufficiency in scleroderma caused by respiratory muscle weakness, Am Rev Respir Dis, 130, 142 Vandecasteele, 2016, Six-minute walk test in systemic sclerosis: a systematic review and meta-analysis, Int J Cardiol, 212, 265, 10.1016/j.ijcard.2016.03.084 Savarese, 2012, Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension? A meta-analysis of 22 randomized trials, J Am Coll Cardiol, 60, 1192, 10.1016/j.jacc.2012.01.083 Tomassetti, 2021, Bronchoalveolar lavage and lung biopsy in connective tissue diseases, to do or not to do?, Ther Adv Musculoskelet Dis, 13, 10.1177/1759720X211059605 Wells, 1994, Fibrosing alveolitis in systemic sclerosis. Bronchoalveolar lavage findings in relation to computed tomographic appearance, Am J Respir Crit Care Med, 150, 462, 10.1164/ajrccm.150.2.8049830 Behr, 1996, Bronchoalveolar lavage for evaluation and management of scleroderma disease of the lung, Am J Respir Crit Care Med, 154, 400, 10.1164/ajrccm.154.2.8756813 Orlandi, 2022, The role of bronchoalveolar lavage in systemic sclerosis interstitial lung disease: a systematic literature review, Pharmaceuticals (Basel), 15, 10.3390/ph15121584 Flaherty, 2004, Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis?, Am J Respir Crit Care Med, 170, 904, 10.1164/rccm.200402-147OC Walsh, 2016, Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study, Lancet Respir Med, 4, 557, 10.1016/S2213-2600(16)30033-9 Furini, 2019, The role of the multidisciplinary evaluation of interstitial lung diseases: systematic literature review of the current evidence and future perspectives, Front Med (Lausanne), 6, 246, 10.3389/fmed.2019.00246 Jo, 2016, Clinical impact of the interstitial lung disease multidisciplinary service, Respirology, 21, 1438, 10.1111/resp.12850 Castelino, 2011, The impact of rheumatological evaluation in the management of patients with interstitial lung disease, Rheumatology (Oxford), 50, 489, 10.1093/rheumatology/keq233 Distler, 2020, Predictors of progression in systemic sclerosis patients with interstitial lung disease, Eur Respir J, 55, 10.1183/13993003.02026-2019 Flaherty, 2019, Nintedanib in progressive fibrosing interstitial lung diseases, N Engl J Med, 381, 1718, 10.1056/NEJMoa1908681 Raghu, 2022, 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, 205, e18, 10.1164/rccm.202202-0399ST Goh, 2017, Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis, Arthritis Rheumatol, 69, 1670, 10.1002/art.40130 Khanna, 2015, Connective tissue disease-associated interstitial lung diseases (CTD-ILD) – report from OMERACT CTD-ILD working group, J Rheumatol, 42, 2168, 10.3899/jrheum.141182 Volkmann, 2022, Early radiographic progression of scleroderma: lung disease predicts long-term mortality, Chest, 161, 1310, 10.1016/j.chest.2021.11.033 Wu, 2019, Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort, Ann Rheum Dis, 78, 648, 10.1136/annrheumdis-2018-213455 Wangkaew, 2016, Correlation of delta high-resolution computed tomography (HRCT) score with delta clinical variables in early systemic sclerosis (SSc) patients, Quant Imaging Med Surg, 6, 381, 10.21037/qims.2016.08.08 Boonstra, 2020, Association of anti-topoisomerase I antibodies of the IgM isotype with disease progression in anti-topoisomerase I-positive systemic sclerosis, Arthritis Rheumatol, 72, 1897, 10.1002/art.41403 Volkmann, 2022, Association of symptoms of gastroesophageal reflux, esophageal dilation and progression of systemic sclerosis-related interstitial lung disease, Arthritis Care Res (Hoboken) Zhang, 2013, Association of gastroesophageal factors and worsening of forced vital capacity in systemic sclerosis, J Rheumatol, 40, 850, 10.3899/jrheum.120705 Wu, 2018, Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model, Ann Rheum Dis, 77, 1326, 10.1136/annrheumdis-2018-213201 Fields, 2022, Mediators of systemic sclerosis-associated interstitial lung disease (SSc-ILD): systematic review and meta-analyses, Thorax Ishikawa, 2012, Utility of KL-6/MUC1 in the clinical management of interstitial lung diseases, Respir Investig, 50, 3, 10.1016/j.resinv.2012.02.001 Tanaka, 2014, IL-6 in inflammation, immunity, and disease, Cold Spring Harb Perspect Biol, 6, 10.1101/cshperspect.a016295 Baldus, 2004, MUC1 and the MUCs: a family of human mucins with impact in cancer biology, Crit Rev Clin Lab Sci, 41, 189, 10.1080/10408360490452040 Abdel-Magied, 2016, Serum interleukin-6 in systemic sclerosis and its correlation with disease parameters and cardiopulmonary involvement, Sarcoidosis Vasc Diffuse Lung Dis, 33, 321 Tiev, 2011, Serum CC chemokine ligand-18 predicts lung disease worsening in systemic sclerosis, Eur Respir J, 38, 1355, 10.1183/09031936.00004711 Hoffmann-Vold, 2016, High level of chemokine CCL18 is associated with pulmonary function deterioration, lung fibrosis progression, and reduced survival in systemic sclerosis, Chest, 150, 299, 10.1016/j.chest.2016.03.004 Valerio Marzano, 1998, Elevated circulating CA 15.3 levels in a subset of systemic sclerosis with severe lung involvement, Arch Dermatol, 134, 645, 10.1001/archderm.134.5.645 Wu, 2017, CCL2 in the circulation predicts long-term progression of interstitial lung disease in patients with early systemic sclerosis: data from two independent cohorts, Arthritis Rheumatol, 69, 1871, 10.1002/art.40171 Hoffmann-Vold, 2016, High level of chemokine CCL2 is associated with lung fibrosis progression and reduced survival in two independent systemic sclerosis cohorts [abstract], Arthritis Rheumatol, 68 Yokoyama, 2006, Prognostic value of circulating KL-6 in idiopathic pulmonary fibrosis, Respirology, 11, 164, 10.1111/j.1440-1843.2006.00834.x Celeste, 2013, Carbohydrate antigen 15.3 as a serum biomarker of interstitial lung disease in systemic sclerosis patients, Eur J Intern Med, 24, 671, 10.1016/j.ejim.2013.04.004 Kumanovics, 2008, Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis, Clin Exp Rheumatol, 26, 414 Elhaj, 2013, Can serum surfactant protein D or CC-chemokine ligand 18 predict outcome of interstitial lung disease in patients with early systemic sclerosis?, J Rheumatol, 40, 1114, 10.3899/jrheum.120997 De Luca, 2015, Tumour-associated antigens in systemic sclerosis patients with interstitial lung disease: association with lung involvement and cancer risk, Rheumatology (Oxford), 54, 1991, 10.1093/rheumatology/kev204 Salazar, 2018, KL-6 but not CCL-18 is a predictor of early progression in systemic sclerosis-related interstitial lung disease, J Rheumatol, 45, 1153, 10.3899/jrheum.170518 Kuwana, 2016, Elevated serum krebs von den lungen-6 in early disease predicts subsequent deterioration of pulmonary function in patients with systemic sclerosis and interstitial lung disease, J Rheumatol, 43, 1825, 10.3899/jrheum.160339 Yanaba, 2004, Comparative study of serum surfactant protein-D and KL-6 concentrations in patients with systemic sclerosis as markers for monitoring the activity of pulmonary fibrosis, J Rheumatol, 31, 1112 Hant, 2009, Surfactant protein D and KL-6 as serum biomarkers of interstitial lung disease in patients with scleroderma, J Rheumatol, 36, 773, 10.3899/jrheum.080633 Benyamine, 2018, Elevated serum Krebs von den Lungen-6 in systemic sclerosis: a marker of lung fibrosis and severity of the disease, Rheumatol Int, 38, 813, 10.1007/s00296-018-3987-3 De Lauretis, 2013, Serum interleukin 6 is predictive of early functional decline and mortality in interstitial lung disease associated with systemic sclerosis, J Rheumatol, 40, 435, 10.3899/jrheum.120725 Jee, 2023, A composite serum biomarker index for the diagnosis of systemic sclerosis interstitial lung disease: a multicentre, observational, cohort study, Arthritis Rheumatol, 10.1002/art.42491 Hoffmann-Vold, 2020, Circulating biomarkers of systemic sclerosis – interstitial lung disease, J Scleroderma Relat Disord, 5, 41, 10.1177/2397198319894851 González García, 2022, Left ventricular diastolic dysfunction in systemic sclerosis: clinical, immunological and survival differences in the Spanish RESCLE registry, Semin Arthritis Rheum, 55, 152033, 10.1016/j.semarthrit.2022.152033 Mohri, 2022, High prevalence of occult left ventricular diastolic dysfunction detected by exercise stress test in systemic sclerosis, Sci Rep, 12, 2423, 10.1038/s41598-022-06400-7 Tennoe, 2018, Left ventricular diastolic dysfunction predicts mortality in patients with systemic sclerosis, J Am Coll Cardiol, 72, 1804, 10.1016/j.jacc.2018.07.068 Tashkin, 2017, Improved cough and cough-specific quality of life in patients treated for scleroderma-related interstitial lung disease: results of Scleroderma Lung Study II, Chest, 151, 813, 10.1016/j.chest.2016.11.052