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An analysis of connective tissue disease-associated interstitial lung disease at a US Tertiary Care Center: better survival in patients with systemic sclerosis.
J Rheumatol. 2011 Apr; 38(4):693-701.JR

Abstract

OBJECTIVE

To compare survival of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) versus idiopathic pulmonary fibrosis (IPF) and patients with systemic sclerosis-associated ILD (SSc-ILD) versus other CTD-ILD followed at our center.

METHODS

We used the Stanford ILD database, which contains prospectively collected information on patients with ILD evaluated at our tertiary care center from 2002 to 2009. Survival at last followup from time of ILD diagnosis was calculated using the Kaplan-Meier estimator. Prognostic factors for survival in the overall cohort (IPF and CTD-ILD) and in the CTD-ILD group were identified with univariate and multivariate Cox regression models.

RESULTS

Of 427 patients with ILD, 148 (35%) had IPF and 76 (18%) had CTD-ILD at the baseline visit. The cumulative incidence of CTD was 4%. After a median followup of 4 years, 67 patients (36.4%) had died and 4 (2.2%) were lost to followup. Patients with IPF (n = 122) and CTD-ILD (n = 62) experienced similar survival rates (5-year survival about 50%). Patients with SSc-ILD (n = 24) experienced better survival than those with other CTD-ILD (n = 38), with 1-year, 3-year, and 5-year survival rates of 100%, 90%, and 77%, respectively, versus 78%, 42%, and 38% (p = 0.01). The presence of SSc in patients with CTD-ILD decreased the risk of death by > 80% even after correcting for age at ILD diagnosis, sex, and ethnicity (HR = 0.17, 95% CI 0.04-0.83).

CONCLUSION

Survival in patients with SSc-ILD was better than in patients with other CTD-ILD, potentially related to routine screening for and early detection of ILD in patients with SSc at our center.

Authors+Show Affiliations

Department of Medicine, Division of Immunology and Rheumatology, Division of Pulmonary and Critical Care, Stanford University School of Medicine, Palo Alto, CA, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

21285162

Citation

Su, Robert, et al. "An Analysis of Connective Tissue Disease-associated Interstitial Lung Disease at a US Tertiary Care Center: Better Survival in Patients With Systemic Sclerosis." The Journal of Rheumatology, vol. 38, no. 4, 2011, pp. 693-701.
Su R, Bennett M, Jacobs S, et al. An analysis of connective tissue disease-associated interstitial lung disease at a US Tertiary Care Center: better survival in patients with systemic sclerosis. J Rheumatol. 2011;38(4):693-701.
Su, R., Bennett, M., Jacobs, S., Hunter, T., Bailey, C., Krishnan, E., Rosen, G., & Chung, L. (2011). An analysis of connective tissue disease-associated interstitial lung disease at a US Tertiary Care Center: better survival in patients with systemic sclerosis. The Journal of Rheumatology, 38(4), 693-701. https://doi.org/10.3899/jrheum.100675
Su R, et al. An Analysis of Connective Tissue Disease-associated Interstitial Lung Disease at a US Tertiary Care Center: Better Survival in Patients With Systemic Sclerosis. J Rheumatol. 2011;38(4):693-701. PubMed PMID: 21285162.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - An analysis of connective tissue disease-associated interstitial lung disease at a US Tertiary Care Center: better survival in patients with systemic sclerosis. AU - Su,Robert, AU - Bennett,Mihoko, AU - Jacobs,Susan, AU - Hunter,Tessa, AU - Bailey,Carissa, AU - Krishnan,Eswar, AU - Rosen,Glenn, AU - Chung,Lorinda, Y1 - 2011/02/01/ PY - 2011/2/3/entrez PY - 2011/2/3/pubmed PY - 2011/7/21/medline SP - 693 EP - 701 JF - The Journal of rheumatology JO - J. Rheumatol. VL - 38 IS - 4 N2 - OBJECTIVE: To compare survival of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) versus idiopathic pulmonary fibrosis (IPF) and patients with systemic sclerosis-associated ILD (SSc-ILD) versus other CTD-ILD followed at our center. METHODS: We used the Stanford ILD database, which contains prospectively collected information on patients with ILD evaluated at our tertiary care center from 2002 to 2009. Survival at last followup from time of ILD diagnosis was calculated using the Kaplan-Meier estimator. Prognostic factors for survival in the overall cohort (IPF and CTD-ILD) and in the CTD-ILD group were identified with univariate and multivariate Cox regression models. RESULTS: Of 427 patients with ILD, 148 (35%) had IPF and 76 (18%) had CTD-ILD at the baseline visit. The cumulative incidence of CTD was 4%. After a median followup of 4 years, 67 patients (36.4%) had died and 4 (2.2%) were lost to followup. Patients with IPF (n = 122) and CTD-ILD (n = 62) experienced similar survival rates (5-year survival about 50%). Patients with SSc-ILD (n = 24) experienced better survival than those with other CTD-ILD (n = 38), with 1-year, 3-year, and 5-year survival rates of 100%, 90%, and 77%, respectively, versus 78%, 42%, and 38% (p = 0.01). The presence of SSc in patients with CTD-ILD decreased the risk of death by > 80% even after correcting for age at ILD diagnosis, sex, and ethnicity (HR = 0.17, 95% CI 0.04-0.83). CONCLUSION: Survival in patients with SSc-ILD was better than in patients with other CTD-ILD, potentially related to routine screening for and early detection of ILD in patients with SSc at our center. SN - 0315-162X UR - https://www.unboundmedicine.com/medline/citation/21285162/An_analysis_of_connective_tissue_disease_associated_interstitial_lung_disease_at_a_US_Tertiary_Care_Center:_better_survival_in_patients_with_systemic_sclerosis_ L2 - http://www.jrheum.org/cgi/pmidlookup?view=long&pmid=21285162 DB - PRIME DP - Unbound Medicine ER -