EXTENDED REPORT

Changes in causes of death in systemic sclerosis, 1972–2002

Virginia D Steen¹, Thomas A Medsger²

¹ Georgetown University, Washington, DC, USA
² School of Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA

Correspondence to:
Dr V D Steen
Georgetown University, 3800 Reservoir Road, LL Kober Cogan, Washington, DC 20007, USA; steenv{at}georgetown.edu

Background: Survival of scleroderma has changed since the renal crisis treatment has become possible.

Aims: To document the changes in survival and organ system causes of mortality in systemic sclerosis (SSc) over the past 25 years in patients from a single medical centre.

Methods: Consecutive patients evaluated at the University of Pittsburgh, Pittsburgh, Pennsylvania, USA between 1 January 1972 and 31 December 1996 were studied. Survival was determined in five 5-year time periods between 1972 and 1997. Causes of death included scleroderma-related (scleroderma renal crisis, pulmonary arterial hypertension, pulmonary fibrosis (PF), gastrointestinal (GI), heart and multiorgan failure) and non-scleroderma-related (cancer, atherosclerotic cardiovascular or cerebrovascular disease, infection, sudden death, other and unknown) causes.

Results: The 10-year survival improved steadily from 54% to 66% during each of the time intervals. There was a significant improvement in survival for patients during 1982–91 compared with those during 1972–81 (p<0.001), even when patients with renal crisis were excluded (p<0.005). The frequency of deaths due to renal crisis significantly decreased over the 30-year time period, from 42% to 6% of scleroderma-related deaths (p<0.001), whereas the proportion of patients with scleroderma who died of PF increased from 6% to 33% (p<0.001). The frequency of pulmonary hypertension, independent of PF, also significantly increased during this time period (p<0.05). There were no changes in scleroderma GI- and heart-related deaths, nor in any of the non-scleroderma-related causes, although patients with scleroderma were less likely to die from scleroderma-related problems in the past 15 years.

Conclusion: The change in the pattern of scleroderma-related deaths over the past 30 years demonstrates that the lung (both pulmonary hypertension and PF) is the primary cause of scleroderma-related deaths today. It is important that aggressive searches continue to develop better therapies for these severe pulmonary complications of SSc.

Abbreviations: GI, gastrointestinal; PAH, pulmonary arterial hypertension; PF, pulmonary fibrosis; SRC, scleroderma renal crisis; SSc, systemic sclerosis

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

• Hachulla, E., Launay, D., Mouthon, L., Sitbon, O., Berezne, A., Guillevin, L., Hatron, P.-Y., Simonneau, G., Clerson, P., Humbert, M., for the French PAH-SSc Network, (2009). Is Pulmonary Arterial Hypertension Really a Late Complication of Systemic Sclerosis?. Chest 136: 1211-1219 [Abstract] [Full Text]  
• KHANNA, D., DISTLER, O., AVOUAC, J., BEHRENS, F., CLEMENTS, P. J., DENTON, C., FOELDVARI, I., GIANNINI, E., HUSCHER, D., KOWAL-BIELECKA, O., LOVELL, D., MATUCCI-CERINIC, M., MAYES, M., MERKEL, P. A., NASH, P., OPITZ, C. F., PITTROW, D., RUBIN, L., SEIBOLD, J. R., STEEN, V., STRAND, C. V., TUGWELL, P. S., VARGA, J., ZINK, A., FURST, D. E., for the Investigators in CRISS and EPOSS, (2009). Measures of Response in Clinical Trials of Systemic Sclerosis: The Combined Response Index for Systemic Sclerosis (CRISS) and Outcome Measures in Pulmonary Arterial Hypertension Related to Systemic Sclerosis (EPOSS). The Journal of Rheumatology 36: 2356-2361 [Abstract] [Full Text]  
• BADESCH, D. B., McGOON, M. D., BARST, R. J., TAPSON, V. F., RUBIN, L. J., WIGLEY, F. M., KRAL, K. M., RAPHIOU, I. H., CRATER, G. D. (2009). Longterm Survival Among Patients with Scleroderma-associated Pulmonary Arterial Hypertension Treated with Intravenous Epoprostenol. The Journal of Rheumatology 36: 2244-2249 [Abstract] [Full Text]  
• Vachiery, J-L., Coghlan, G. (2009). Screening for pulmonary arterial hypertension in systemic sclerosis. ERR 18: 162-169 [Abstract] [Full Text]  
• CODULLO, V., CAVAZZANA, I., BONINO, C., ALPINI, C., CAVAGNA, L., COZZI, F., PAPA, N. D., FRANCESCHINI, F., GUIDUCCI, S., MOROZZI, G., RUFFATTI, A., FERRI, C., GIACOMELLI, R., MATUCCI-CERINIC, M., VALENTINI, G., MONTECUCCO, C. (2009). Serologic Profile and Mortality Rates of Scleroderma Renal Crisis in Italy. The Journal of Rheumatology 36: 1464-1469 [Abstract] [Full Text]  
• LAUNAY, D., MARJANOVIC, Z., de BAZELAIRE, C., FLOREA, L., ZOHAR, S., KESHTMAND, H., DELIGNY, C., de RAIGNIAC, A., WELLS, A. U., FARGE, D. (2009). Autologous Hematopoietic Stem Cell Transplant in Systemic Sclerosis: Quantitative High Resolution Computed Tomography of the Chest Scoring. The Journal of Rheumatology 36: 1460-1463 [Abstract] [Full Text]  
• Matucci-Cerinic, M., Steen, V., Nash, P., Hachulla, E. (2009). The complexity of managing systemic sclerosis: screening and diagnosis. Rheumatology (Oxford) 48: iii8-iii13 [Abstract] [Full Text]  
• Black, C. M., Matucci-Cerinic, M., Guillevin, L. (2009). Progress in systemic sclerosis: a 10-year perspective. Rheumatology (Oxford) 48: iii1-iii2 [Full Text]  
• Denton, C. P., Lapadula, G., Mouthon, L., Muller-Ladner, U. (2009). Renal complications and scleroderma renal crisis. Rheumatology (Oxford) 48: iii32-iii35 [Abstract] [Full Text]  
• Guillevin, L. (2009). Translating ideas into progress in systemic sclerosis. Rheumatology (Oxford) 48: iii58-iii60 [Full Text]  
• Wells, A. U., Steen, V., Valentini, G. (2009). Pulmonary complications: one of the most challenging complications of systemic sclerosis. Rheumatology (Oxford) 48: iii40-iii44 [Abstract] [Full Text]  
• Humbert, M. (2009). Update in Pulmonary Hypertension 2008. Am. J. Respir. Crit. Care Med. 179: 650-656 [Full Text]  
• Humbert, M. (2009). More pressure on pulmonary hypertension. ERR 18: 1-3 [Full Text]  
• Hachulla, E., Carpentier, P., Gressin, V., Diot, E., Allanore, Y., Sibilia, J., Launay, D., Mouthon, L., Jego, P., Cabane, J., de Groote, P., Chabrol, A., Lazareth, I., Guillevin, L., Clerson, P., Humbert, M., the ItinerAIR-Sclerodermie Study Investigators, (2009). Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinerAIR-Sclerodermie study. Rheumatology (Oxford) 48: 304-308 [Abstract] [Full Text]  
• Hanitsch, L. G., Burmester, G.-R., Witt, C., Hunzelmann, N., Genth, E., Krieg, T., Lehmacher, W., Melchers, I., Meurer, M., Muller-Ladner, U., Schulze-Lohoff, E., Becker, M., Sunderkoetter, C., the DNSS centers, , Riemekasten, G. (2009). Skin sclerosis is only of limited value to identify SSc patients with severe manifestations--an analysis of a distinct patient subgroup of the German Systemic Sclerosis Network (DNSS) Register. Rheumatology (Oxford) 48: 70-73 [Abstract] [Full Text]  
• Aden, N., Shiwen, X., Aden, D., Black, C., Nuttall, A., Denton, C. P., Leask, A., Abraham, D., Stratton, R. (2008). Proteomic analysis of scleroderma lesional skin reveals activated wound healing phenotype of epidermal cell layer. Rheumatology (Oxford) 47: 1754-1760 [Abstract] [Full Text]  
• Mozaffarian, A., Brewer, A. W., Trueblood, E. S., Luzina, I. G., Todd, N. W., Atamas, S. P., Arnett, H. A. (2008). Mechanisms of Oncostatin M-Induced Pulmonary Inflammation and Fibrosis. J. Immunol. 181: 7243-7253 [Abstract] [Full Text]  
• Belch, J. J. F., McSwiggan, S., Lau, C. (2008). Macrovascular disease in systemic sclerosis: the tip of an iceberg?. Rheumatology (Oxford) 47: v16-v17 [Abstract] [Full Text]  
• Denton, C. P. (2008). Renal manifestations of systemic sclerosis--clinical features and outcome assessment. Rheumatology (Oxford) 47: v54-v56 [Abstract] [Full Text]  
• Seibold, J. R., Tyndall, A., Furst, D. E. (2008). Trial design: how must we move ahead?. Rheumatology (Oxford) 47: v57-v58 [Abstract] [Full Text]  
• Behr, J., Furst, D. E. (2008). Pulmonary function tests. Rheumatology (Oxford) 47: v65-v67 [Abstract] [Full Text]  
• Steen, V., Chou, M., Shanmugam, V., Mathias, M., Kuru, T., Morrissey, R. (2008). Exercise-Induced Pulmonary Arterial Hypertension in Patients With Systemic Sclerosis. Chest 134: 146-151 [Abstract] [Full Text]  
• Strange, C., Seibold, J. R. (2008). Scleroderma Lung Disease: "If You Don't Know Where You Are Going, Any Road Will Take You There". Am. J. Respir. Crit. Care Med. 177: 1178-1179 [Full Text]  
• Hsu, E., Feghali-Bostwick, C. A. (2008). Insulin-Like Growth Factor-II Is Increased in Systemic Sclerosis-Associated Pulmonary Fibrosis and Contributes to the Fibrotic Process via Jun N-Terminal Kinase- and Phosphatidylinositol-3 Kinase-Dependent Pathways. Am. J. Pathol. 172: 1580-1590 [Abstract] [Full Text]  
• Tourkina, E., Richard, M., Gooz, P., Bonner, M., Pannu, J., Harley, R., Bernatchez, P. N., Sessa, W. C., Silver, R. M., Hoffman, S. (2008). Antifibrotic properties of caveolin-1 scaffolding domain in vitro and in vivo. Am. J. Physiol. Lung Cell. Mol. Physiol. 294: L843-L861 [Abstract] [Full Text]  
• Teixeira, L, Mouthon, L, Mahr, A, Berezne, A, Agard, C, Mehrenberger, M, Noel, L-H, Trolliet, P, Frances, C, Cabane, J, Guillevin, L, for the Group Francais de Recherche sur le Sclerod, (2008). Mortality and risk factors of scleroderma renal crisis: a French retrospective study of 50 patients. Ann Rheum Dis 67: 110-116 [Abstract] [Full Text]  
• Chung, L., Krishnan, E., Chakravarty, E. F. (2007). Hospitalizations and mortality in systemic sclerosis: results from the Nationwide Inpatient Sample. Rheumatology (Oxford) 46: 1808-1813 [Abstract] [Full Text]