Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension

Lionel Schachna, Thomas A. Medsger, James H. Dauber, Fredrick M. Wigley, Neil A. Braunstein, Barbara White, Virginia D. Steen, John V. Conte, Stephen C. Yang, Kenneth R. McCurry, Marvin C. Borja, David E. Plaskon, Jonathan B. Orens, Allan C. Gelber

Research output: Contribution to journalArticle

127 Citations (Scopus)

Abstract

Objective. Lung transplantation is a viable, life-saving intervention for several primary pulmonary disorders complicated by severe lung dysfunction. This study was undertaken to evaluate whether patients with systemic sclerosis (scleroderma), a systemic autoimmune rheumatic disorder, would receive similar benefit from this intervention. Methods. Survival following tang transplantation was examined at 2 university medical centers among 29 patients with scleroderma as compared with 70 patients with idiopathic pulmonary fibrosis (IPF) and 38 with idiopathic pulmonary arterial hypertension (IPAH), the latter groups representing pathologically related primary pulmonary disorders. The end point was death from any cause. Risk of mortality in patients with scleroderma was compared with that in patients with IPF or IPAH, with adjustment for demographic and clinical parameters. Results. During 2 years of followup, 11 patients with scleroderma (38%), 23 with IPF (33%), and 14 with IPAH (37%) died. Cumulative survival at 6 months posttransplantation was 69% in the scleroderma group compared with 80% in the IPF group (log-rank P = 0.21) and 79% in the IPAH group (P = 0.38). The estimated risk of mortality at 6 months was increased in patients with scleroderma compared with those with IPF (relative risk [RR] 1.70, 95% confidence interval [95% CI] 0.74-3.93) and those with IPAH (RR 1.52,95% CI 0.59-3.96), but the differences were not statistically significant. Over the following 18 months, there was convergence in the survival rates such that cumulative survival at 2 years was comparable, at ∼64%, among all 3 groups. Conclusion. Patients with scleroderma who are recipients of lung transplantation experience similar rates of survival 2 years after the procedure compared with those with IPF or IPAH. Lung transplantation may represent a viable therapeutic option to consider for patients with end-stage lung disease due to scleroderma.

Original languageEnglish (US)
Pages (from-to)3954-3961
Number of pages8
JournalArthritis and rheumatism
Volume54
Issue number12
DOIs
StatePublished - Dec 1 2006

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Idiopathic Pulmonary Fibrosis
Lung Transplantation
Survival Rate
Systemic Scleroderma
Lung
Confidence Intervals
Familial Primary Pulmonary Hypertension
Survival
Mortality
Lung Diseases
Cause of Death
Transplantation
Demography

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Pharmacology (medical)

Cite this

Schachna, L., Medsger, T. A., Dauber, J. H., Wigley, F. M., Braunstein, N. A., White, B., ... Gelber, A. C. (2006). Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis and rheumatism, 54(12), 3954-3961. https://doi.org/10.1002/art.22264
Schachna, Lionel ; Medsger, Thomas A. ; Dauber, James H. ; Wigley, Fredrick M. ; Braunstein, Neil A. ; White, Barbara ; Steen, Virginia D. ; Conte, John V. ; Yang, Stephen C. ; McCurry, Kenneth R. ; Borja, Marvin C. ; Plaskon, David E. ; Orens, Jonathan B. ; Gelber, Allan C. / Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. In: Arthritis and rheumatism. 2006 ; Vol. 54, No. 12. pp. 3954-3961.
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abstract = "Objective. Lung transplantation is a viable, life-saving intervention for several primary pulmonary disorders complicated by severe lung dysfunction. This study was undertaken to evaluate whether patients with systemic sclerosis (scleroderma), a systemic autoimmune rheumatic disorder, would receive similar benefit from this intervention. Methods. Survival following tang transplantation was examined at 2 university medical centers among 29 patients with scleroderma as compared with 70 patients with idiopathic pulmonary fibrosis (IPF) and 38 with idiopathic pulmonary arterial hypertension (IPAH), the latter groups representing pathologically related primary pulmonary disorders. The end point was death from any cause. Risk of mortality in patients with scleroderma was compared with that in patients with IPF or IPAH, with adjustment for demographic and clinical parameters. Results. During 2 years of followup, 11 patients with scleroderma (38{\%}), 23 with IPF (33{\%}), and 14 with IPAH (37{\%}) died. Cumulative survival at 6 months posttransplantation was 69{\%} in the scleroderma group compared with 80{\%} in the IPF group (log-rank P = 0.21) and 79{\%} in the IPAH group (P = 0.38). The estimated risk of mortality at 6 months was increased in patients with scleroderma compared with those with IPF (relative risk [RR] 1.70, 95{\%} confidence interval [95{\%} CI] 0.74-3.93) and those with IPAH (RR 1.52,95{\%} CI 0.59-3.96), but the differences were not statistically significant. Over the following 18 months, there was convergence in the survival rates such that cumulative survival at 2 years was comparable, at ∼64{\%}, among all 3 groups. Conclusion. Patients with scleroderma who are recipients of lung transplantation experience similar rates of survival 2 years after the procedure compared with those with IPF or IPAH. Lung transplantation may represent a viable therapeutic option to consider for patients with end-stage lung disease due to scleroderma.",
author = "Lionel Schachna and Medsger, {Thomas A.} and Dauber, {James H.} and Wigley, {Fredrick M.} and Braunstein, {Neil A.} and Barbara White and Steen, {Virginia D.} and Conte, {John V.} and Yang, {Stephen C.} and McCurry, {Kenneth R.} and Borja, {Marvin C.} and Plaskon, {David E.} and Orens, {Jonathan B.} and Gelber, {Allan C.}",
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Schachna, L, Medsger, TA, Dauber, JH, Wigley, FM, Braunstein, NA, White, B, Steen, VD, Conte, JV, Yang, SC, McCurry, KR, Borja, MC, Plaskon, DE, Orens, JB & Gelber, AC 2006, 'Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension', Arthritis and rheumatism, vol. 54, no. 12, pp. 3954-3961. https://doi.org/10.1002/art.22264

Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. / Schachna, Lionel; Medsger, Thomas A.; Dauber, James H.; Wigley, Fredrick M.; Braunstein, Neil A.; White, Barbara; Steen, Virginia D.; Conte, John V.; Yang, Stephen C.; McCurry, Kenneth R.; Borja, Marvin C.; Plaskon, David E.; Orens, Jonathan B.; Gelber, Allan C.

In: Arthritis and rheumatism, Vol. 54, No. 12, 01.12.2006, p. 3954-3961.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension

AU - Schachna, Lionel

AU - Medsger, Thomas A.

AU - Dauber, James H.

AU - Wigley, Fredrick M.

AU - Braunstein, Neil A.

AU - White, Barbara

AU - Steen, Virginia D.

AU - Conte, John V.

AU - Yang, Stephen C.

AU - McCurry, Kenneth R.

AU - Borja, Marvin C.

AU - Plaskon, David E.

AU - Orens, Jonathan B.

AU - Gelber, Allan C.

PY - 2006/12/1

Y1 - 2006/12/1

N2 - Objective. Lung transplantation is a viable, life-saving intervention for several primary pulmonary disorders complicated by severe lung dysfunction. This study was undertaken to evaluate whether patients with systemic sclerosis (scleroderma), a systemic autoimmune rheumatic disorder, would receive similar benefit from this intervention. Methods. Survival following tang transplantation was examined at 2 university medical centers among 29 patients with scleroderma as compared with 70 patients with idiopathic pulmonary fibrosis (IPF) and 38 with idiopathic pulmonary arterial hypertension (IPAH), the latter groups representing pathologically related primary pulmonary disorders. The end point was death from any cause. Risk of mortality in patients with scleroderma was compared with that in patients with IPF or IPAH, with adjustment for demographic and clinical parameters. Results. During 2 years of followup, 11 patients with scleroderma (38%), 23 with IPF (33%), and 14 with IPAH (37%) died. Cumulative survival at 6 months posttransplantation was 69% in the scleroderma group compared with 80% in the IPF group (log-rank P = 0.21) and 79% in the IPAH group (P = 0.38). The estimated risk of mortality at 6 months was increased in patients with scleroderma compared with those with IPF (relative risk [RR] 1.70, 95% confidence interval [95% CI] 0.74-3.93) and those with IPAH (RR 1.52,95% CI 0.59-3.96), but the differences were not statistically significant. Over the following 18 months, there was convergence in the survival rates such that cumulative survival at 2 years was comparable, at ∼64%, among all 3 groups. Conclusion. Patients with scleroderma who are recipients of lung transplantation experience similar rates of survival 2 years after the procedure compared with those with IPF or IPAH. Lung transplantation may represent a viable therapeutic option to consider for patients with end-stage lung disease due to scleroderma.

AB - Objective. Lung transplantation is a viable, life-saving intervention for several primary pulmonary disorders complicated by severe lung dysfunction. This study was undertaken to evaluate whether patients with systemic sclerosis (scleroderma), a systemic autoimmune rheumatic disorder, would receive similar benefit from this intervention. Methods. Survival following tang transplantation was examined at 2 university medical centers among 29 patients with scleroderma as compared with 70 patients with idiopathic pulmonary fibrosis (IPF) and 38 with idiopathic pulmonary arterial hypertension (IPAH), the latter groups representing pathologically related primary pulmonary disorders. The end point was death from any cause. Risk of mortality in patients with scleroderma was compared with that in patients with IPF or IPAH, with adjustment for demographic and clinical parameters. Results. During 2 years of followup, 11 patients with scleroderma (38%), 23 with IPF (33%), and 14 with IPAH (37%) died. Cumulative survival at 6 months posttransplantation was 69% in the scleroderma group compared with 80% in the IPF group (log-rank P = 0.21) and 79% in the IPAH group (P = 0.38). The estimated risk of mortality at 6 months was increased in patients with scleroderma compared with those with IPF (relative risk [RR] 1.70, 95% confidence interval [95% CI] 0.74-3.93) and those with IPAH (RR 1.52,95% CI 0.59-3.96), but the differences were not statistically significant. Over the following 18 months, there was convergence in the survival rates such that cumulative survival at 2 years was comparable, at ∼64%, among all 3 groups. Conclusion. Patients with scleroderma who are recipients of lung transplantation experience similar rates of survival 2 years after the procedure compared with those with IPF or IPAH. Lung transplantation may represent a viable therapeutic option to consider for patients with end-stage lung disease due to scleroderma.

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