Subscribe to RSS
DOI: 10.4103/liuj.liuj_51_20
Demographic characteristics and clinical manifestations of interstitial lung disease with systemic sclerosis in eastern part of Libya
Abstract
Introduction: Pulmonary involvement, such as Interstitial Lung Disease (ILD) and Pulmonary Hypertension (PH ), accounts for significant morbidity and is the leading cause of Systemic sclerosis (SSc)-related morbidity and mortality. The aim of the current study was to study the frequency of occurrence of ILD in SSc and to describe the clinical and radiological picture of pulmonary involvement in SSc. Patients and Methods: Forty patients attending different rheumatology clinics in eastern part of Libya between January 2018 and September 2020 were included. Basic details including age, gender, disease duration, detailed history, and clinical examination were noted. Autoimmune profiling included rheumatoid factor, antinuclear antibodies, and anti-Scl-70 antibodies. Pulmonary function test, chest X-ray (CXR), and high-resolution computed tomography of the chest (HRCT) in all patients were recorded. Data presented either as frequencies and percentages or as means ± standard deviation. Results: The male: female ratio was 1:9 with a mean age of 37.5 ± 9.6 years and duration of illness 6 ± 4 years. diffuse cutaneous SSc was seen in 62.5% of the patients. 77.5% of the participants had bilateral crepitation and 57.5% had loud P2. Presenting complaints included gastrointestinal reflux in 72.5%, digitalis ulcerations in 40%, and synovitis/arthritis of all patients. Other comorbidities included congestive heart failure in 12.5%, PH in 15%, and renal impairments in 7.5% of all patients. Anti-Scl-70 antibody was the most common in all patients (45%), followed by anti-centromere Ab (25%), anti-U3 RNP (10%), and anti-U1 RNP (5%). 72.5% of the participants had reticulonodular shadows on CXR. HRCT showed honeycombing as the predominant finding (37.5%). Echocardiograms showed that 15% of all patients have signs of PH. Duration of disease, dyspnea, cough, bilateral crepitations, and CXR were found to be significantly associated with extensive ILD (P < 0.05). Conclusion: ILD is a serious complication of SSc, it is more common among patients with dcSSc. Chest HRCT is very sensitive to detect ILD. A significant association was found in Libyan patients between the severity of ILD and the duration of disease, dyspnea, cough, bilateral crepts, and CXR.
Financial support and sponsorship
Nil.
Publication History
Received: 29 December 2020
Accepted: 28 March 2021
Article published online:
14 June 2022
© 2021. Libyan International Medical University. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India
-
References
-
1 Asano Y. Systemic sclerosis. J Dermatol 2018;45:128-38.
-
2 Hochberg MC, Smolen JO, Weinblatt ME, Weisman MH. Rheumatology. 3rd ed., Vol. 2., Ch. 132-135., Sec. 10. USA: Mosby; 2003. p. 1455-506.
-
3 Kane GC, Varga J, Conant EF, Spirn PW, Jimenez S, Fish JE. Lung involvement in systemic sclerosis (scleroderma): Relation to classification based on extent of skin involvement or autoantibody status. Respir Med 1996;90:223-30.
-
4 Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A, et al. Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis 2017;76:1897-905.
-
5 Witt C, Borges AC, John M, Fietze I, Baumann G, Krause A. Pulmonary involvement in diffuse cutaneous systemic sclerosis: Broncheoalveolar fluid granulocytosis predicts progression of fibrosing alveolitis. Ann Rheum Dis 1999;58:635-40.
-
6 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;64:519-24.
-
7 Nihtyanova SI, Schreiber BE, Ong VH, Rosenberg D, Moinzadeh P, Coghlan JG, et al. Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol 2014;66:1625-35.
-
8 Jaeger VK, Wirz EG, Allanore Y, Rossbach P, Riemekasten G, Hachulla E, et al. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: A longitudinal EUSTAR study. PLoS One 2016;11:e0163894.
-
9 Asano Y, Ihn H, Yamane K, Kubo M, Tamaki K. The prevalence and clinical significance of anti-U1 RNA antibodies in patients with systemic sclerosis. J Invest Dermatol 2003;120:204-10.
-
10 Man A, Davidyock T, Ferguson LT, Ieong M, Zhang Y, Simms RW. Changes in forced vital capacity over time in systemic sclerosis: Application of group-based trajectory modelling. Rheumatology (Oxford) 2015;54:1464-71.
-
11 Steen VD, Conte C, Owens GR, Medsger TA Jr. Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 1994;37:1283-9.
-
12 Morgan C, Knight C, Lunt M, Black CM, Silman AJ. Predictors of end stage lung disease in a cohort of patients with scleroderma. Ann Rheum Dis 2003;62:146-50.
-
13 van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: An American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 2013;65:2737-47.
-
14 Villalba WO, Sampaio-Barros PD, Pereira MC, Cerqueira EM, Leme CA Jr., Marques-Neto JF, et al. Six-minute walk test for the evaluation of pulmonary disease severity in scleroderma patients. Chest 2007;131:217-22.
-
15 Gilson M, Zerkak D, Wipff J, Dusser D, Dinh-Xuan AT, Abitbol V, et al. Prognostic factors for lung function in systemic sclerosis: Prospective study of 105 cases. Eur Respir J 2010;35:112-7.
-
16 Assassi S, Sharif R, Lasky RE, McNearney TA, Estrada-Y-Martin RM, Draeger H, et al. Predictors of interstitial lung disease in early systemic sclerosis: A prospective longitudinal study of the GENISOS cohort. Arthritis Res Ther 2010;12:R166.
-
17 Bergamasco A, Hartmann N, Wallace L, Verpillat P. Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol 2019;11:257-73.
-
18 Vonk MC, Broers B, Heijdra YF, Ton E, Snijder R, van Dijk AP, et al. Systemic sclerosis and its pulmonary complications in The Netherlands: An epidemiological study. Ann Rheum Dis 2009;68:961-5.
-
19 Cappelli S, Bellando Randone S, Camiciottoli G, de Paulis A, Guiducci S, Matucci-Cerinic M. Interstitial lung disease in systemic sclerosis: Where do we stand? Eur Respir Rev 2015;24:411-9.
-
20 Hafez EA, Hamza SH, Morad CS, Abd Alkader AA. Pulmonary manifestations in Egyptian patients with systemic sclerosis. The Egyptian Journal of Internal Medicine 2018;40:39-44.
-
21 Davie N, Haleen SJ, Upton PD, Polak JM, Yacoub MH, Morrell NW, et al. ET(A) and ET(B) receptors modulate the proliferation of human pulmonary artery smooth muscle cells. Am J Respir Crit Care Med 2002;165:398-405.
-
22 Wan ES, DeMeo DL, Hersh CP, Shapiro SD, Rosiello RA, Sama SR, et al. Clinical predictors of frequent exacerbations in subjects with severe chronic obstructive pulmonary disease (COPD). Respir Med 2011;105:588-94.
-
23 Ryerson CJ, O'Connor D, Dunne JV, Schooley F, Hague CJ, Murphy D, et al. Predicting mortality in systemic sclerosis-associated interstitial lung disease using risk prediction models derived from idiopathic pulmonary fibrosis. Chest 2015;148:1268-75.
-
24 Hao Y, Hudson M, Carreira P, Stevens W, Rabusa C, Tatibouet S, et al. Early mortality in Australian, Canadian and Spanish scleroderma patients: rationale for establishing a multi-national inception cohort of patients with systemic sclerosis [abstract]. Arthritis Rheum. 2014;66(Suppl 10):S316.
-
25 Whitacre CC. Sex differences in autoimmune disease. Nat Immunol 2001;2:777-80.
-
26 Solomon J, Olson A, Fischer A, Bull T, Brown K, Raghu G. Scleroderma lung disease. Eur Respir Rev 2013;22:6-19.
-
27 de Azevedo AB, Sampaio-Barros PD, Torres RM, Moreira C. Prevalence of pulmonary hypertension in systemic sclerosis. Clin Exp Rheumatol 2005;23:447-54.
-
28 Hachulla E, Launay D, Mouthon L, Sitbon O, Berezne A, Guillevin L, et al. Is pulmonary arterial hypertension really a late complication of systemic sclerosis? Chest 2009;136:1211-9.
-
29 Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 2000;43:2437-44.
-
30 Becker MO, Schohe A, Weinert K, Huscher D, Schneider U, Burmester GR, et al. Responders to cyclophosphamide: Results of a single-centre analysis among systemic sclerosis patients. Ann Rheum Dis 2012;71:2061-2.
-
31 Nannini C, West CP, Erwin PJ, Matteson EL. Effects of cyclophosphamide on pulmonary function in patients with scleroderma and interstitial lung disease: A systematic review and meta-analysis of randomized controlled trials and observational prospective cohort studies. Arthritis Res Ther 2008;10:R124.
-
32 Adler S, Huscher D, Siegert E, Allanore Y, Czirják L, DelGaldo F, et al. Systemic sclerosis associated interstitial lung disease – Individualized immunosuppressive therapy and course of lung function: Results of the EUSTAR group. Arthritis Res Ther 2018;20:17.
-
33 Bédard Méthot D, Leblanc É, Lacasse Y. Meta-analysis of gastroesophageal reflux disease and idiopathic pulmonary fibrosis. Chest 2019;155:33-43.