Generic placeholder image

Current Rheumatology Reviews


ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Research Article

Interstitial Lung Disease in Systemic Sclerosis: A Single-center Retrospective Analysis

Author(s): Müçteba Enes Yayla*, Gülşah Balcı , Murat Torgutalp, Didem Şahin Eroğlu, Ayşe Bahar Keleşoğlu Dinçer , Emine Gözde Aydemir Gülöksüz, Serdar Sezer , Mehmet Levent Yüksel, Aşkın Ateş, Tahsin Murat Turgay and Gülay Kınıklı

Volume 18, Issue 2, 2022

Published on: 13 September, 2021

Page: [150 - 156] Pages: 7

DOI: 10.2174/1573397117666210913104029

Price: $65


Background: Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by microangiopathy, inflammation, fibrosis. Interstitial lung disease (ILD) is common among SSc patients.

Objective: This study aims to define the clinical, laboratory, and serologic characteristics of SSc patients with ILD and to present the frequency of chest computed tomography features.

Methods: Two hundred twenty-six SSc patients who applied to the Rheumatology Department between January 2007 and August 2019 were retrospectively examined. A total of 100 SSc patients with ILD (44.2%) were determined. Clinical, laboratory, and serological features of SSc patients with and without ILD were compared.

Result: Both groups had similar characteristics in terms of age and sex. The duration of disease (p=0.001) and follow-up time (p=0.001) were longer in SSc patients with ILD. Multivariable logistic regression analysis indicated that the duration of disease (OR: 1.06 (1.01-1.13), p=0.029), presence of gastrointestinal system involvement (OR: 3.29 (1.28-8.46), p=0.013) and anti-SCL70-positivity (OR: 6.04 (2.35-15.49), p <0.001) were associated with ILD. There was an inverse relationship between Anti-CENP-B positivity and the presence of ILD (p=0.001). The assessment regarding the chest computed tomography characteristics of interstitial pneumonia patterns were as follows: 82.5% non-specific interstitial pneumonia, 14.4% usual interstitial pneumonia, and 2.1% desquamative interstitial pneumonia. The most frequent abnormal findings included ground-glass opacification (88.7%), reticulation (64.9%), traction bronchiectasis (57.7%), septal thickening (52.6%) and honeycombing (28.9%).

Conclusion: We have shown a relationship between anti-SCL70, disease duration, gastrointestinal system involvement, and ILD in SSc patients.

Keywords: Systemic sclerosis, interstitial lung disease, pulmonary fibrosis, mortality, anti-SCL70, anti-CENP-B.

Graphical Abstract
Denton CP, Khanna D. Systemic sclerosis. Lancet 2017; 390(10103): 1685-99.
[] [PMID: 28413064]
Giacomelli R, Liakouli V, Berardicurti O, et al. Interstitial lung disease in systemic sclerosis: current and future treatment. Rheumatol Int 2017; 37(6): 853-63.
[] [PMID: 28063071]
Hashisako M, Fukuoka J. Pathology of idiopathic interstitial pneumonias. Clin Med Insights Circ Respir Pulm Med 2016; 9(Suppl. 1): 123-33.
[PMID: 26949346]
Steele R, Hudson M, Lo E, Baron M, Group CSR. Clinical decision rule to predict the presence of interstitial lung disease in systemic sclerosis. Arthritis Care Res (Hoboken) 2012; 64(4): 519-24.
[] [PMID: 22213733]
Wynn TA. Integrating mechanisms of pulmonary fibrosis. J Exp Med 2011; 208(7): 1339-50.
[] [PMID: 21727191]
van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 2013; 65(11): 2737-47.
[] [PMID: 24122180]
LeRoy EC, Medsger TA Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol 2001; 28(7): 1573-6.
[PMID: 11469464]
Gilson M, Zerkak D, Wipff J, et al. Prognostic factors for lung function in systemic sclerosis: prospective study of 105 cases. Eur Respir J 2010; 35(1): 112-7.
[] [PMID: 19541715]
Nihtyanova SI, Schreiber BE, Ong VH, et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol 2014; 66(6): 1625-35.
[] [PMID: 24591477]
Wangkaew S, Euathrongchit J, Wattanawittawas P, Kasitanon N, Louthrenoo W. Incidence and predictors of interstitial lung disease (ILD) in Thai patients with early systemic sclerosis: Inception cohort study. Mod Rheumatol 2016; 26(4): 588-93.
[] [PMID: 26561397]
Morelli S, Barbieri C, Sgreccia A, et al. Relationship between cutaneous and pulmonary involvement in systemic sclerosis. J Rheumatol 1997; 24(1): 81-5.
[PMID: 9002015]
Walker UA, Tyndall A, Czirják L, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis 2007; 66(6): 754-63.
[] [PMID: 17234652]
Kayser C, Fritzler MJ. Autoantibodies in systemic sclerosis: unanswered questions. Front Immunol 2015; 6: 167.
[] [PMID: 25926833]
Hénault J, Robitaille G, Senécal JL, Raymond Y. DNA topoisomerase I binding to fibroblasts induces monocyte adhesion and activation in the presence of anti-topoisomerase I autoantibodies from systemic sclerosis patients. Arthritis Rheum 2006; 54(3): 963-73.
[] [PMID: 16508979]
Jung E, Suh CH, Kim HA, Jung JY. Clinical characteristics of systemic sclerosis with interstitial lung disease. Arch Rheumatol 2018; 33(3): 322-7.
[] [PMID: 30632529]
Tangri V, Hewson C, Baron M, Bonner A, Fritzler M, Pope JE. Associations with organ involvement and autoantibodies in systemic sclerosis: results from the Canadian Scleroderma Research Group (CSRG). Open J Rheumatol Autoimmune Dis 2013; 3: 113-8.
Tobin RW, Pope CE II, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998; 158(6): 1804-8.
[] [PMID: 9847271]
Schachter LM, Dixon J, Pierce RJ, O’Brien P. Severe gastroesophageal reflux is associated with reduced carbon monoxide diffusing capacity. Chest 2003; 123(6): 1932-8.
[] [PMID: 12796170]
Marie I, Dominique S, Levesque H, et al. Esophageal involvement and pulmonary manifestations in systemic sclerosis. Arthritis Rheum 2001; 45(4): 346-54.
[<346::AID-ART347>3.0.CO;2-L] [PMID: 11501722]
Troshinsky MB, Kane GC, Varga J, et al. Pulmonary function and gastroesophageal reflux in systemic sclerosis. Ann Intern Med 1994; 121(1): 6-10.
[] [PMID: 8198350]
Lock G, Holstege A, Lang B, Schölmerich J. Gastrointestinal manifestations of progressive systemic sclerosis. Am J Gastroenterol 1997; 92(5): 763-71.
[PMID: 9149182]
Hax V, Bredemeier M, Didonet Moro AL, et al. Clinical algorithms for the diagnosis and prognosis of interstitial lung disease in systemic sclerosis. Semin Arthritis Rheum 2017; 47(2): 228-34.
[] [PMID: 28454677]
Ashmore P, Tikly M, Wong M, Ickinger C. Interstitial lung disease in South Africans with systemic sclerosis. Rheumatol Int 2018; 38(4): 657-62.
[] [PMID: 29185086]
McNearney TA, Reveille JD, Fischbach M, et al. Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum 2007; 57(2): 318-26.
[] [PMID: 17330281]
Jaeger VK, Wirz EG, Allanore Y, et al. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study. PLoS One 2016; 11(10): e0163894.
[] [PMID: 27706206]
Wu W, Jordan S, Becker MO, et al. Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model. Ann Rheum Dis 2018; 77(9): 1326-32.
[] [PMID: 29875097]
Sánchez-Cano D, Ortego-Centeno N, Callejas JL, et al. Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group. Rheumatol Int 2018; 38(3): 363-74.
[] [PMID: 29322341]
Goldin JG, Lynch DA, Strollo DC, et al. High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest 2008; 134(2): 358-67.
[] [PMID: 18641099]
Wells AU. Interstitial lung disease in systemic sclerosis. Presse Med 2014; 43(10 Pt 2): e329-43.
[] [PMID: 25217474]
Tanaka N, Kunihiro Y, Kubo M, Kawano R, Oishi K, Ueda K. HRCT findings of collagen vascular disease-related interstitial pneumonia (CVD-IP): a comparative study among individual underlying diseases. Clin Radiol 2018; 73(9): 833.e1-833.e10.

Rights & Permissions Print Export Cite as
© 2022 Bentham Science Publishers | Privacy Policy