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Clinical Predictors of Survival in Lymphangioleiomyomatosis

Kinder, Brent W., M.D.
http://rave.ohiolink.edu/etdc/view?acc_num=ucin1298041138

Abstract Details

2011, MS, University of Cincinnati, Medicine: Clinical and Translational Research.
RATIONALE: Lymphangioleiomyomatosis (LAM) is a rare, progressive, cystic lung disease that almost exclusively affects women. Prognostic information in LAM has been limited by heterogeneous study methodology. OBJECTIVES: We sought to establish which clinical characteristics are associated with survival and to determine cause of death in patients with LAM. METHODS: The LAM Foundation maintains a population-based registry of self-identified LAM patients who completed a questionnaire with demographic and clinical data at enrollment. Vital status was obtained on all participants. Cox proportional hazard analysis evaluated the association of demographic and clinical features with survival. RESULTS: Among the 401 subjects, there were 50 deaths and 55 lung transplantations during a median of 10 years of observation time. The estimated median survival time for LAM patients in the US is 29 years from symptom onset and 23 years from diagnosis. Age at diagnosis (HR per decade 0.80, CI 0.64-0.99, p=0.04), supplemental oxygen use (HR 3.13, CI 1.90-5.18, p<0.001), and reported weight loss (HR 1.93, CI 1.23-3.04, p=0.004) were strong independent predictors of time to death or transplant. Neither mode of presentation, history of pregnancy, nor hormonal treatment was found to be associated with survival after adjustment for covariates. Among decedents, the most common cause of death was respiratory failure. CONCLUSIONS: Median survival in a population-based cohort of patients with LAM in the United States is longer than previously estimated. Demographic and clinical predictors are useful for prognostic determination. Patients with LAM frequently die from complications directly related to their lung disease.
Erin Nicole Haynes, DrPH (Committee Chair)
Francis McCormack, MD (Committee Member)
27 p.
Surgery
Lymphangioleiomyomatosis; epidemiology; Survival

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Citations

  • Kinder, M.D., B. W. (2011). Clinical Predictors of Survival in Lymphangioleiomyomatosis [Master's thesis, University of Cincinnati]. OhioLINK Electronic Theses and Dissertations Center. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1298041138

    APA Style (7th edition)

  • Kinder, M.D., Brent. Clinical Predictors of Survival in Lymphangioleiomyomatosis. 2011. University of Cincinnati, Master's thesis. OhioLINK Electronic Theses and Dissertations Center, http://rave.ohiolink.edu/etdc/view?acc_num=ucin1298041138.

    MLA Style (8th edition)

  • Kinder, M.D., Brent. "Clinical Predictors of Survival in Lymphangioleiomyomatosis." Master's thesis, University of Cincinnati, 2011. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1298041138

    Chicago Manual of Style (17th edition)

ucin1298041138
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© 2011, all rights reserved.
This open access ETD is published by University of Cincinnati and OhioLINK.