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Clinical InvestigationsPULMONARY FUNCTION TRAITSDecline in Lung Function in Patients With Lymphangioleiomyomatosis Treated With or Without Progesterone
Section snippets
Study Population
The study population consisted of 348 patients with LAM referred to the National Institutes of Health since 1996 for participation in a longitudinal study (National Heart, Lung, and Blood Institute [NHLBI] protocol 95-H-0186) approved by the NHLBI Institutional Review Board. In addition to self-referral or referral through individual physicians, subjects were informed of the study by the LAM Foundation and the Tuberous Sclerosis Alliance. All subjects gave informed consent before enrollment.
Patient Characteristics
The mean age of the 275 patients at the time of the first test was 42.3 ≤ 0.6 years (range, 19 to 77 years). Sixteen patients were Asian, 9 patients were African American, 2 patients were African, and the rest (248) were white. Twelve patients were smokers (10 ≤ 5 pack-years), 25 patients were ex-smokers (20 ≤ 2 pack-years), and the rest were nonsmokers. Mean age at the time of diagnosis was 41.0 ≤ 0.6 years. However, based on the history of LAM-related symptoms (pneumothorax, chylous
Discussion
We report here a retrospective analysis of the decline in lung function in a large population of patients with LAM, focusing on patients who were treated with progesterone by the po or IM route vs those who did not receive hormonal therapy. To quantify the decline in lung function, we selected the Dlco and FEV1 because these tests correlate well with disease severity defined by CT scans and LAM histology scores.101124 Initial Dlco and FEV1 and age were the most important predictors of rates of
ACKNOWLEDGMENT
We thank Dr. Martha Vaughan for discussions and critical review, and Xiaoling Chen for assistance in compilation and analysis of the data. We also thank the LAM Foundation and the Tuberous Sclerosis Alliance for their assistance in recruiting patients. This study would not have been possible without the cooperation of patients with LAM, who, in many cases, traveled great distances to participate in our clinical research protocols.
References (50)
Lymphangioleiomyomatosis: a review
Chest
(1998)- et al.
Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis
Chest
(1999) - et al.
High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex
Mayo Clin Proc
(2000) - et al.
Pregnancy exacerbating unsuspected mediastinal lymphangioleiomyomatosis and chylothorax
Int J Gynaecol Obstet
(1996) - et al.
Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous estrogens
Chest
(1987) - et al.
A report of two long-surviving cases of pulmonary lymphangioleiomyomatosis and the response to progesterone therapy
Br J Dis Chest
(1985) - et al.
Treatment of lymphangioleiomyomatosis: a meta-analysis
Chest
(1989) - et al.
Successful treatment of pulmonary lymphangioleiomyomatosis with progestins
Chest
(1999) Pharmacokinetics of progestational compounds
Maturitas
(1986)- et al.
Pharmacokinetic observations on MPA administered orally and intravaginally
Contraception
(1976)
Bioavailability of orally administered sex steroids used in oral contraception and hormone replacement therapy
Contraception
A preliminary pharmacokinetic and pharmacodynamic evaluation of depo-medroxyprogesterone acetate and norethisterone enantate
Fertil Steril
A study of the mechanism of weight gain in medroxyprogesterone acetate users
Contraception
Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors
Am J Respir Crit Care Med
Pulmonary lymphangioleiomyomatosis: a study of 69 patients
Medicine
Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex
Am J Respir Crit Care Med
Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis
Am J Respir Crit Care Med
The spectrum of mutations in TSC1 and TSC2 in women with tuberous sclerosis and lymphangioleiomyomatosis
Am J Respir Crit Care Med
Epidemiology of tuberous sclerosis
Ann NY Acad Sci
Reversible airflow obstruction, proliferation of abnormal smooth muscle cells and impairment of gas exchange as predictors of outcome in lymphangioleiomyomatosis
Am J Respir Crit Care Med
Maximal oxygen uptake and severity of disease in lymphangioleiomyomatosis
Am J Respir Crit Care Med
Pulmonary lymphangioleiomyomatosis complicated by pregnancy
Kansas Med
Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous oestrogen used for infertility treatment
Thorax
Pulmonary lymphangioleiomyomatosis (LAM): examining oral contraceptive pills and onset of disease
J Womens Health
Immunohistochemical detection of steroid receptors in a case of lymphangioleiomyomatosis
Am J Surg Pathol
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Lung transplantation for lymphangioleiomyomatosis
2023, Journal of Heart and Lung TransplantationA survey of use of mTOR inhibitors in patients with lymphangioleiomyomatosis listed for lung transplant
2022, Respiratory MedicineCitation Excerpt :Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease which affects women almost exclusively, in either a sporadic form or in association with tuberous sclerosis complex (TSC) [1–6]. LAM is a progressive lung disease, with a mean loss of forced expiratory volume in 1 s (FEV1) of 70–140 mL per year [3,7]. Progression of the disease, although typically gradual, can be life threatening, necessitating referral for lung transplantation.
Supported by the NHLBI, Division of Intramural Research.
Ms. Xiaoling Chen was supported in part by a grant from the LAM Foundation.