Chest
Original ResearchDiffuse Lung DiseasePredictors of Mortality and Progression in Scleroderma-Associated Interstitial Lung Disease: A Systematic Review
Section snippets
Data Sources and Search
We performed a systematic search of the literature using the MEDLINE, Embase, and Evidence-Based Medicine Reviews databases. The search was designed to capture all studies related to predictors of mortality and ILD progression in SSc-ILD. Two authors (T. A. W. and C. J. R.) reviewed citations independently using predefined criteria. Searches covered the period from the onset of the database to April 2013. Bibliographies from selected articles and major reviews were screened for additional
Search Results and Study Characteristics
The initial search identified 3,145 unique citations, and 167 were reviewed in full text. Twenty-seven descriptive studies met the eligibility criteria,5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31 including six abstracts (Fig 1, Table 1).7, 9, 21, 24, 30, 31 A total of 1,616 patients with SSc-ILD were included (range, 18-215 patients per study). The duration of follow-up was reported in 21 studies and ranged from 1 to 14 years. There was
Discussion
We conducted a rigorous systematic review of the literature to identify variables that predict mortality or ILD progression in patients with SSc-ILD. Several patient-specific, ILD-specific, and SSc-specific variables predicted mortality and ILD progression in SSc-ILD; however, included studies were of variable methodologic quality, and the predictors identified in this review should be considered within the context of these limited data. A minority of studies performed a multivariate analysis,
Conclusions
In summary, we found that several patient-specific, ILD-specific, and SSc-specific features predict worse prognosis in SSc-ILD. Dlco was the most consistent predictor of mortality and may help identify patients with a poor prognosis; however, more rigorous studies are needed to confirm and expand on these preliminary findings. A clearer understanding of how to determine prognosis with variables such as Dlco is required prior to incorporation of these findings into clinical practice. Additional
Acknowledgments
Author contributions: C. J. R. takes responsibility as the guarantor of the manuscript, including the data and analysis. T. A. W. and C. J. R. contributed to the design of the study, primary analysis and interpretation of the data, production of the initial draft of the manuscript, and approval of the final version and D. A., P. G. W., J. V. D., C. J. H., J. L., and H. R. C. contributed to the analysis and interpretation of the data, drafting of the manuscript, and approval of the final version.
References (38)
Introduction: diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines
Chest
(2004)Interstitial lung disease in scleroderma
Rheum Dis Clin North Am
(2003)- et al.
Determinants of morbidity and mortality of systemic sclerosis in Canada
Semin Arthritis Rheum
(2010) - et al.
Clinically significant interstitial lung disease in limited scleroderma: histopathology, clinical features, and survival
Chest
(2008) - et al.
Clinical characteristics and survival in systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease
Chest
(2011) - et al.
Association of gastroesophageal factors and progression of interstitial lung disease in the Canadian Scleroderma Research Group (CSRG), a large, multi-center database
J Rheumatol
(2012) - et al.
The natural course of progressive systemic sclerosis patients with interstitial lung involvement
Clin Rheumatol
(2007) - et al.
Changes in causes of death in systemic sclerosis, 1972-2002
Ann Rheum Dis
(2007) - et al.
T-889C IL-1alpha promoter polymorphism influences the response to oral cyclophosphamide in scleroderma patients with alveolitis
Clin Rheumatol
(2007) - et al.
Circulating T cell polarization is associated with respiratory decline in scleroderma patients with active interstitial lung disease [abstract]
Arthritis Rheum
(2010)
Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome
Am J Respir Crit Care Med
Serum markers of disease progression in idiopathic pulmonary fibrosis and in interstitial lung disease associated with systemic sclerosis
Am J Respir Crit Care Med
β-Thymosins and interstitial lung disease: study of a scleroderma cohort with a one-year follow-up
Respir Res
Bronchoalveolar lavage fluid and progression of scleroderma interstitial lung disease
Clin Respir J
Anti-U11/U12 RNP antibodies in systemic sclerosis: a new serologic marker associated with pulmonary fibrosis
Arthritis Rheum
Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis-associated interstitial lung disease are not predictive of disease progression
Arthritis Rheum
Interstitial lung disease in systemic sclerosis: a simple staging system
Am J Respir Crit Care Med
Increased epithelial permeability in pulmonary fibrosis in relation to disease progression
Eur Respir J
Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung Study Placebo Group
Arthritis Rheum
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