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Interstitial lung disease in South Africans with systemic sclerosis

  • Epidemiology of RMD
  • Published:
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Abstract

To investigate the frequency, severity and predictors of interstitial lung disease (ILD) in a cohort of South Africans with systemic sclerosis (SSc). Retrospective record review of SSc patients attending a tertiary Connective Tissue Diseases Clinic. Patients with ILD, defined by a combination of clinical findings, imaging, and lung function tests were compared to patients without ILD in terms of demographics, clinical features and autoantibodies. The majority (86.8%) of the 151 patients included were of Black ethnicity, 40% had ILD, of whom 39% had moderate–severe lung disease. Univariate predictors of ILD included: disease duration (OR 1.08, 95% CI 1.01–1.15); cough (OR 2.93, 95% CI 1.37–6.29); dyspnoea (OR 2.44, 95% CI 1.23–4.87); bibasal crackles (OR 7.58, 95% CI 3.31–17.37); diffuse cutaneous SSc (dcSSc) (OR 4.55, 95% CI 2.10–9.86) and a speckled anti-nuclear antibody (ANA) pattern (OR 2.47, 95% CI 1.25–4.90). Conversely, limited cutaneous disease (OR 0.22, 95% CI 0.09–0.50) and anti-centromere antibody (ACA) (OR 0.12, 95% CI 0.02–0.97) were protective. Independent predictors of ILD on multivariate analysis were bibasal crackles (OR 9.43, 95% CI 3.25–27.39), disease duration (OR 1.19, 95% CI 1.09–1.30) and speckled ANA (OR 2.95, 95% CI 1.22–7.15). Almost all (86.4%) patients received immunosuppressive treatment and the leading cause of death was related to ILD itself (44.4%). In this cohort of predominantly Black South Africans, SSc ILD was common and carried a poor prognosis. ILD occurred mainly, but not exclusively, in patients with dcSSc, especially those with a speckled ANA pattern. Conversely, the presence of ACA was protective against ILD.

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References

  1. Steen VD, Medsger TA (2007) Changes in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis 66(7):940–944

    Article  PubMed  PubMed Central  Google Scholar 

  2. Bussone G, Mouthon L (2011) Interstitial lung disease in systemic sclerosis. Autoimmun Rev 10(5):248–255

    Article  Google Scholar 

  3. Steele R, Hudson M, Lo E, Baron M (2012) Clinical decision rule to predict the presence of interstitial lung disease in systemic sclerosis. Arthritis Care Res (Hoboken) 64(4):519–524

    Article  Google Scholar 

  4. Meier FM, Frommer KW, Dinser R, Walker UA, Czirjak L, Denton CP et al (2012) Update on the profile of the EUSTAR cohort: an analysis of the EULAR scleroderma trials and research group database. Ann Rheum Dis 71(8):1355–1360

    Article  PubMed  Google Scholar 

  5. Steen VD, Conte C, Owens GR, Medsger TA Jr (1994) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37(9):1283–1289

    Article  CAS  PubMed  Google Scholar 

  6. Mayes MD, Lacey JV Jr, Beebe-Dimmer J, Gillespie BW, Cooper B, Laing TJ et al (2003) Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 48(8):2246–2255

    Article  PubMed  Google Scholar 

  7. Cowie RL, Dansey RD (1990) Features of systemic sclerosis (scleroderma) in South African goldminers. S Afr Med J 77(8):400–402

    CAS  PubMed  Google Scholar 

  8. Erasmus LD (1957) Scleroderma in goldminers on the Witwatersrand with particular reference to pulmonary manifestations. S Afr J Lab Clin Med 3(3):209–231

    Google Scholar 

  9. Cowie RL (1987) Silica-dust-exposed mine workers with scleroderma (systemic sclerosis). Chest 92(2):260–262

    Article  CAS  PubMed  Google Scholar 

  10. Gilson M, Zerkak D, Wipff J, Dusser D, Dinh-Xuan AT, Abitbol V et al (2010) Prognostic factors for lung function in systemic sclerosis: prospective study of 105 cases. Eur Respir J 35(1):112–117

    Article  CAS  PubMed  Google Scholar 

  11. Castelino FV, Varga J (2010) Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management. Arthritis Res Ther 12(4):213

    Article  PubMed  PubMed Central  Google Scholar 

  12. Ho KT, Reveille JD (2003) The clinical relevance of autoantibodies in scleroderma. Arthritis Res Ther 5(2):80–93

    Article  CAS  PubMed  Google Scholar 

  13. McNearney TA, Reveille JD, Fischbach M, Friedman AW, Lisse JR, Goel N et al (2007) Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum 57(2):318 – 26

    Article  PubMed  Google Scholar 

  14. Steen V, Domsic RT, Lucas M, Fertig N, Medsger TA Jr (2012) A clinical and serologic comparison of African American and Caucasian patients with systemic sclerosis. Arthritis Rheum 64(9):2986–2994

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Tangri VHC, Baron M, Bonner A, Fritzler M, Pope J (2013) Associations with organ involvement and autoantibodies in systemic sclerosis: results from the Canadian Scleroderma Research Group (CSRG). Open J Rheumatol Autoimmune Dis 3(2):113–118

    Article  Google Scholar 

  16. Tager RE, Tikly M (1999) Clinical and laboratory manifestations of systemic sclerosis (scleroderma) in Black South Africans. Rheumatology 38(5):397–400

    Article  CAS  PubMed  Google Scholar 

  17. Assassi S, Sharif R, Lasky RE, McNearney TA, Estrada YMRM., Draeger H et al (2010) Predictors of interstitial lung disease in early systemic sclerosis: a prospective longitudinal study of the GENISOS cohort. Arthritis Res Ther 12(5):R166

    Article  PubMed  PubMed Central  Google Scholar 

  18. Solomon JJ, Olson AL, Fischer A, Bull T, Brown KK, Raghu G (2013) Scleroderma lung disease. Eur Respir Rev 22(127):6–19

    Article  PubMed  PubMed Central  Google Scholar 

  19. Barnes J, Mayes MD (2012) Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr Opin Rheumatol 24(2):165 – 70

    Article  PubMed  Google Scholar 

  20. Theodore AC, Tseng CH, Li N, Elashoff RM, Tashkin DP (2012) Correlation of cough with disease activity and treatment with cyclophosphamide in scleroderma interstitial lung disease: findings from the Scleroderma Lung Study. Chest 142(3):614–621

    Article  Google Scholar 

  21. Muangchan C, Harding S, Khimdas S, Bonner A, Baron M, Pope J (2012) Association of C-reactive protein with high disease activity in systemic sclerosis: results from the Canadian Scleroderma Research Group. Arthritis Care Res (Hoboken) 64(9):1405–1414

    Article  CAS  Google Scholar 

  22. Roth MD, Tseng CH, Clements PJ, Furst DE, Tashkin DP, Goldin JG et al (2011) Predicting treatment outcomes and responder subsets in scleroderma-related interstitial lung disease. Arthritis Rheum 63(9):2797 – 808

    Article  PubMed  PubMed Central  Google Scholar 

  23. Masi AT, Rodnan GP, Medsger TA, Altman RD, D'Angelo WA, Fries JM et al (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 23(5):581–590

    Article  Google Scholar 

  24. Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM et al (2008) Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med 177(11):1248–1254

    Article  PubMed  Google Scholar 

  25. LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15(2):202–205

    CAS  PubMed  Google Scholar 

  26. Hair JF (2006) Multivariate data analysis. Pearson Prentice Hall, Upper Saddle River

    Google Scholar 

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Acknowledgements

Mr Manoj Chiba assisted with statistical analysis.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Faculty of Health Sciences, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa

    Philippa Ashmore, Mohammed Tikly & Claudia Ickinger

  2. Division of Pulmonology, Department of Medicine, Faculty of Health Sciences, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa

    Michelle Wong

Authors
  1. Philippa Ashmore
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  2. Mohammed Tikly
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  3. Michelle Wong
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  4. Claudia Ickinger
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Contributions

PA, MT, CI: Study conception and design. PA, MW: Acquisition of data. PA, MT, CI: Analysis and interpretation of data. PA, CI: Drafting of article. PA, MT, MW, CI: Critical revision of article. MT, CI: Supervision. PA, MT, MW, CI: Approval of version to be published.

Corresponding author

Correspondence to Claudia Ickinger.

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Funding

This was an unfunded study.

Conflict of interest

Philippa Ashmore, Mohammed Tikly, Michelle Wong, Claudia Ickinger declare that they have no conflict of interest.

Ethics approval

The study was approved by the Human Research Ethics Committee (Medical), University of the Witwatersrand (approval no. M120966, 2012-09-28), with waive of informed consent as this was a retrospective chart review. All procedures performed in studies involving participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Ashmore, P., Tikly, M., Wong, M. et al. Interstitial lung disease in South Africans with systemic sclerosis. Rheumatol Int 38, 657–662 (2018). https://doi.org/10.1007/s00296-017-3893-0

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  • DOI: https://doi.org/10.1007/s00296-017-3893-0

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