The DOI matches its Crossref record; 14 of the 14 references it cites were found in a registry or an index.
Severe restrictive lung disease in systemic sclerosis
Abstract
Objective. We sought to identify risk factors for developing severe restrictive lung disease and to determine the time of onset and rate of progression in patients with systemic sclerosis (SSc). Methods. Using the University of Pittsburgh Scleroderma Databank, we grouped patients according to their lowest forced vital capacity (FVC) value: <75% predicted, 50—75% predicted, and > 50% predicted. In patients with severe restrictive disease, we examined serial pulmonary function test (PFT) results to determine the rate of loss of lung volume over time. Results. Of 890 SSc patients, 60% (n = 531) never had an FVC ⩽75% predicted; 27% (n = 243) had moderate restrictive disease, with an FVC value of 50—75% predicted; and only 13% (n = 116) of the patients had severe restrictive disease, with FVC ⩽50% predicted. Black race, male sex, early disease, and primary cardiac involvement due to SSc were the features most frequently associated with severe restrictive lung disease (by multiple logistic regression). Fifty–five patients with severe restrictive lung disease had their first of at least 2 PFTs during the first 5 years after onset of any SSc (not pulmonary) symptoms. In 30 patients, the FVC declined by 32% per year in the first 2 years of illness, in 16 patients the annual loss was 12% in years 2—4 after disease onset, and in 9 patients annual loss was 3% during years 4—6 of disease (P < 0.005 by 1–way analysis of variance). Conclusion. In SSc patients, black men with early disease who have cardiac involvement are the most likely to have factors associated with the development of severe restrictive lung disease (which is increasingly becoming a major cause of death). Disease subtype (diffuse versus limited cutaneous) and serum antitopoisomerase I antibody do not differentiate between moderate and severe restrictive disease. Careful monitoring of pulmonary function early in the disease, when the greatest loss of lung function occurs, may help identify patients likely to respond to new therapy.
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Cites
Scleroderma (systemic sclerosis): classification, subsets and pathogenesis
(2001) · Classic Papers in Rheumatology · doi:10.3109/9780203214237-95 · Cited by 3,950
Steen, V. D., Powell, D. L., & Medsger, T. A. (1988) · Arthritis & Rheumatism · doi:10.1002/art.1780310207 · Cited by 573
Self-citationSteen, V. D., Costantino, J. P., Shapiro, A. P., & Medsger, T. A. (1990) · Annals of Internal Medicine · doi:10.7326/0003-4819-113-5-352 · Cited by 441
Self-citationD-Penicillamine Therapy in Progressive Systemic Sclerosis (Scleroderma): A Retrospective Analysis
STEEN, V. D., MEDSGER, T. A., & RODNAN, G. P. (1982) · Annals of Internal Medicine · doi:10.7326/0003-4819-97-5-652 · Cited by 322
Self-citationMEDSGER, T. A., MASI, A. T., RODNAN, G. P., BENEDEK, T. G., & ROBINSON, H. (1971) · Annals of Internal Medicine · doi:10.7326/0003-4819-75-3-369 · Cited by 321
Self-citationPredictors of survival in systemic sclerosis (Scleroderma)
Altman, R. D., Medsger, T. A., Bloch, D. A., & Michel, B. A. (1991) · Arthritis & Rheumatism · doi:10.1002/art.1780340405 · Cited by 298
Self-citationIsolated diffusing capacity reduction in systemic sclerosis
Steen, V. D., Graham, G., Conte, C., Owens, G., & Medsger, T. A. (1992) · Arthritis & Rheumatism · doi:10.1002/art.1780350709 · Cited by 241
Self-citationEvaluation and management of scleroderma lung disease using bronchoalveolar lavage
Silver, R. M., Miller, K. S., Kinsella, M. B., Smith, E. A., & Schabel, S. I. (1990) · The American Journal of Medicine · doi:10.1016/0002-9343(90)90425-d · Cited by 236
Pulmonary involvement in systemic sclerosis (scleroderma)
Steen, V. D., Owens, G. R., Fino, G. J., Rodnan, G. P., & Medsger, T. A. (1985) · Arthritis & Rheumatism · doi:10.1002/art.1780280706 · Cited by 168
Self-citationPulmonary Function in Progressive Systemic Sclerosis
Owens, G. R., Fino, G. J., Herbert, D. L., Steen, V. D., Medsger, T. A., Pennock, B. E., Cottrell, J. J., Rodnan, G. P., & Rogers, R. M. (1983) · CHEST Journal · doi:10.1378/chest.84.5.546 · Cited by 128
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Cyclophosphamide versus placebo in scleroderma lung disease
Tashkin, D. P., Elashoff, R., Clements, P. J., Goldin, J., Roth, M. D., Furst, D. E., Arriola, E., Silver, R., Strange, C., Bolster, M., et al. (2006) · New England Journal of Medicine · doi:10.1056/nejmoa055120 · Cited by 1,677
Self-citationInterstitial Lung Disease in Systemic Sclerosis: A Simple Staging System
Goh, N. S. L., Desai, S. R., Veeraraghavan, S., Hansell, D. M., Copley, S. J., Maher, T. M., Corte, T. J., Sander, C. R., Ratoff, J., Devaraj, A., et al. (2008) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200706-877oc · Cited by 1,230
Orens, J. B., Estenne, M., Arcasoy, S., Conte, J. V., Corris, P., Egan, J. J., Egan, T., Keshavjee, S., Knoop, C., Kotloff, R., et al. (2006) · The Journal of Heart and Lung Transplantation · doi:10.1016/j.healun.2006.03.011 · Cited by 1,073
Interstitial lung disease guideline
Wells, A. U., Hirani, N., & on behalf of the BTS Interstitial Lung Disease Guideline Group, A. S. O. T. B. T. S. S. O. C. C. (2008) · Thorax · doi:10.1136/thx.2008.101691 · Cited by 866
Burt, R. K., Shah, S. J., Dill, K., Grant, T., Gheorghiade, M., Schroeder, J., Craig, R., Hirano, I., Marshall, K., Ruderman, E., et al. (2011) · The Lancet · doi:10.1016/s0140-6736(11)60982-3 · Cited by 578
Metadata
- Authors
- Steen, V. D., Conte, C., Owens, G. R., & Medsger, T. A.
- Venue
- Arthritis & Rheumatism
- Publisher
- Wiley
- Published
- 1994-08
- Volume
- 37
- Issue
- 9
- Pages
- 1283-1289
- ISSN
- 0004-3591 (ISSN-L)
- 1529-0131
- OpenAlex
- W2042113729
- Cited by
- 526
- References
- 14
- FWCI
- 3.70
- Type
- journal article
- Language
- en
- Open access
- closed
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