Journal ArticleDOI
Clinical decision rule to predict the presence of interstitial lung disease in systemic sclerosis.
TLDR
A clinical decision rule to predict the presence of interstitial lung disease in systemic sclerosis (SSc; scleroderma) and to estimate the prevalence of SSc‐ILD is developed.Abstract:
Objective
To develop a clinical decision rule to predict the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc; scleroderma) and to estimate the prevalence of SSc-ILD.
Methods
Patient data were extracted from the Canadian Scleroderma Research Group registry. Three algorithms for the clinical decision rule were considered based on lung auscultation, chest radiography (CXR), and % predicted forced vital capacity (FVC). High-resolution computed tomography (HRCT) scans were used as the gold standard to determine the diagnostic properties of the 3 algorithms. Multiple imputation was used to impute HRCT data when missing, thereby avoiding bias due to differential referral for HRCT.
Results
This study included 1,168 patients. Of the patients with HRCT scans, 65% had evidence of ILD, compared to 26% by physical examination and 22% by CXR. The FVC of those who did not have HRCT was 8.8% greater than those who did (95% confidence interval [95% CI] 6.0–11.6%). Algorithm A, which identified the presence of ILD based on crackles on lung auscultation and/or findings on CXR, had a likelihood ratio of 3.9, compared to 3.2 for Algorithm B (which included patients with FVC <70%) and 2.2 for Algorithm C (which included patients with FVC <80%). The prevalence of ILD in the cohort was estimated to be 52% (95% CI 46–59%).
Conclusion
We developed a simple clinical decision rule to predict SSc-ILD with good test characteristics. The prevalence of ILD in a large, unselected SSc cohort was estimated to be 52%.read more
Citations
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Journal ArticleDOI
Prediction of pulmonary complications and long-term survival in systemic sclerosis.
Svetlana I. Nihtyanova,Benjamin E. Schreiber,Voon H Ong,Daniel W. Rosenberg,Pia Moinzadeh,J. Gerrard Coghlan,Athol U. Wells,Christopher P. Denton +7 more
TL;DR: To assess survival and incidence of organ‐based complications in a large single‐center cohort of unselected systemic sclerosis patients, and to explore predictors of survival and clinically significant pulmonary fibrosis and pulmonary hypertension.
Journal ArticleDOI
Scleroderma lung disease
Joshua J. Solomon,Amy L. Olson,Amy L. Olson,Aryeh Fischer,Aryeh Fischer,Todd M. Bull,Todd M. Bull,Kevin K. Brown,Kevin K. Brown,Ganesh Raghu,Ganesh Raghu +10 more
TL;DR: The direct and indirect pulmonary manifestations of SSc are reviewed and recent therapeutic trials that have attempted to target these manifestations are reviewed.
Journal ArticleDOI
The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements
Anna-Maria Hoffmann-Vold,Toby M. Maher,Edward E Philpot,Ali Ashrafzadeh,Rafic Barake,Simone Barsotti,Cosimo Bruni,Paolo Carducci,Patricia Carreira,Ivan Castellví,Francesco Del Galdo,Jörg H W Distler,Ivan Foeldvari,Paolo Fraticelli,Peter M. George,Bridget Griffiths,Alfredo Guillén-Del-Castillo,Abdul Monem Hamid,Rudolf Horváth,Michael Hughes,Michael Kreuter,Florentine Moazedi-Fuerst,Jacek Olas,Suman Paul,Cinzia Rotondo,Manuel Rubio-Rivas,Andrei Seferian,Andrei Seferian,Michal Tomcik,Yurdagul Uzunhan,Ulrich A. Walker,Ewa Więsik-Szewczyk,Oliver Distler +32 more
TL;DR: The first evidence-based consensus statements were established on guidance for the identification and medical management of systemic sclerosis-associated ILD through a robust modified Delphi process developed by a diverse panel of experts.
Journal ArticleDOI
Systemic sclerosis-associated interstitial lung disease.
TL;DR: Systemic sclerosis-associated interstitial lung disease most commonly presents with dyspnoea, cough, and a non-specific interstitial pneumonia pattern on CT scan, with a minority of cases fulfilling the criteria for usual interstitial tuberculosis.
Journal ArticleDOI
Brief Report: Pulmonary Function Tests: High Rate of False-Negative Results in the Early Detection and Screening of Scleroderma-Related Interstitial Lung Disease.
Yossra A. Suliman,Rucsandra Dobrota,Dörte Huscher,Thi Dan Linh Nguyen-Kim,Britta Maurer,Suzana Jordan,Rudolf Speich,Thomas Frauenfelder,Oliver Distler +8 more
TL;DR: PFTs are evaluated compared with high‐resolution computed tomography of the chest for the detection of SSc‐related ILD in clinical practice, and predictors of lung involvement that is functionally occult but significant on HRCT are identified.
References
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Donald P. Tashkin,Robert Elashoff,Philip J. Clements,Jonathan G. Goldin,Michael D. Roth,Daniel E. Furst,Edgar Arriola,Richard M. Silver,Charlie Strange,Marcy B. Bolster,James R. Seibold,David J. Riley,Vivien Hsu,John Varga,Dean E. Schraufnagel,Arthur C. Theodore,Robert W. Simms,Robert A. Wise,Fredrick M. Wigley,Barbara White,Virginia D. Steen,Charles A. Read,Maureen D. Mayes,Ed Parsley,Kamal K. Mubarak,M. Kari Connolly,Jeffrey A. Golden,Mitchell A. Olman,B. J. Fessler,Naomi F. Rothfield,Mark L. Metersky +30 more
TL;DR: One year of oral cyclophosphamide in patients with symptomatic scleroderma-related interstitial lung disease had a significant but modest beneficial effect on lung function, dyspnea, thickening of the skin, and the health-related quality of life.
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