Am. J. Respir. Crit. Care Med., Volume 160, Number 2, August 1999, 628-633

Decline in Lung Function in Lymphangioleiomyomatosis
Relation to Menopause and Progesterone Treatment

SIMON R. JOHNSON and ANNE E. TATTERSFIELD

Division of Respiratory Medicine, University of Nottingham, City Hospital, Nottingham, United Kingdom

The progression of lymphangioleiomyomatosis, a rare lung disease in women, is thought to be influenced by hormonal factors. We studied the rate of decline in FEV₁ and carbon monoxide transfer factor (TL_CO) in a national cohort of patients with lymphangioleiomyomatosis in the United Kingdom and its relation to two factors that might influence the disease, menopausal status and progesterone treatment. We used retrospective data from hospital notes, and of the 50 patients identified 43 had suitable lung function data spanning at least 3 mo. Mean (SD) annual decline in FEV₁ was 118 (142) ml for all patients, and these figures changed little when only data spanning at least 2 and 3 yr were analyzed. There was considerable variation in the rate of decline between subjects, however, and although it tended to be less among postmenopausal women and those receiving progesterone, patient numbers were smaller and the findings were not significant. There was a significant reduction in decline in TL_CO in premenopausal patients receiving progesterone and in both FEV₁ and TL_CO after starting progesterone in six patients who had data before and after starting treatment. This study documents the rapid decline in lung function in lymphangioleiomyomatosis, confirms the wide variation between patients, and provides some support for the suggestion that disease progression may be reduced by progesterone. The data provide a basis for designing prospective studies of treatment for lymphangioleiomyomatosis.

This article has been cited by other articles:

				D. Clements, S. L Asprey, T. A McCulloch, T. A Morris, S. A Watson, and S. R Johnson Analysis of the oestrogen response in an angiomyolipoma derived xenograft model Endocr. Relat. Cancer, March 1, 2009; 16(1): 59 - 72. [Abstract] [Full Text] [PDF]

				F. X. McCormack Lymphangioleiomyomatosis: A Clinical Update Chest, February 1, 2008; 133(2): 507 - 516. [Abstract] [Full Text] [PDF]

				S. Harari, R. Cassandro, J. Chiodini, A. M. Taveira-DaSilva, and J. Moss Effect of a Gonadotrophin-Releasing Hormone Analogue on Lung Function in Lymphangioleiomyomatosis Chest, February 1, 2008; 133(2): 448 - 454. [Abstract] [Full Text] [PDF]

				G. P Cosgrove, S. K Frankel, and K. K Brown Challenges in pulmonary fibrosis {middle dot} 3: Cystic lung disease Thorax, September 1, 2007; 62(9): 820 - 829. [Abstract] [Full Text] [PDF]

				S. R. Johnson Lymphangioleiomyomatosis. Eur. Respir. J., May 1, 2006; 27(5): 1056 - 1065. [Abstract] [Full Text] [PDF]

				A. E. Tattersfield and M. K. Glassberg Lymphangioleiomyomatosis: A National Registry for a Rare Disease Am. J. Respir. Crit. Care Med., January 1, 2006; 173(1): 2 - 4. [Full Text] [PDF]

				J. H. Ryu, J. Moss, G. J. Beck, J.-C. Lee, K. K. Brown, J. T. Chapman, G. A. Finlay, E. J. Olson, S. J. Ruoss, J. R. Maurer, et al. The NHLBI Lymphangioleiomyomatosis Registry: Characteristics of 230 Patients at Enrollment Am. J. Respir. Crit. Care Med., January 1, 2006; 173(1): 105 - 111. [Abstract] [Full Text] [PDF]

				A. M. Taveira-DaSilva, M. P. Stylianou, C. J. Hedin, O. Hathaway, and J. Moss Decline in Lung Function in Patients With Lymphangioleiomyomatosis Treated With or Without Progesterone Chest, December 1, 2004; 126(6): 1867 - 1874. [Abstract] [Full Text] [PDF]

				S R Johnson, C I Whale, R B Hubbard, S A Lewis, and A E Tattersfield Survival and disease progression in UK patients with lymphangioleiomyomatosis Thorax, September 1, 2004; 59(9): 800 - 803. [Abstract] [Full Text] [PDF]

				C I Whale, S R Johnson, K G Phillips, S A Newton, S A Lewis, and A E Tattersfield Lymphangioleiomyomatosis: a case-control study of perinatal and early life events Thorax, November 1, 2003; 58(11): 979 - 982. [Abstract] [Full Text] [PDF]

				S. Halabi, R. Convery, and J. M. Curtis Progressive Dyspnea in a 49-Year-Old Woman With Long-standing Epilepsy* Chest, July 1, 2002; 122(1): 352 - 355. [Full Text] [PDF]

				A. M. TAVEIRA-DaSILVA, C. HEDIN, M. P. STYLIANOU, W. D. TRAVIS, K. MATSUI, V. J. FERRANS, and J. MOSS 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., September 15, 2001; 164(6): 1072 - 1076. [Abstract] [Full Text] [PDF]

				M. Demedts, A.U. Wells, J.M. Anto, U. Costabel, R. Hubbard, P. Cullinan, H. Slabbynck, G. Rizzato, V. Poletti, E.K. Verbeken, et al. Interstitial lung diseases: an epidemiological overview Eur. Respir. J., July 1, 2001; 18(32_suppl): 2S - 16s. [Abstract] [Full Text] [PDF]

				G.M. Verleden, R.M. du Bois, D. Bouros, M. Drent, A. Millar, J. Muller-Quernheim, G. Semenzato, S. Johnson, G. Sourvinos, D. Olivieri, et al. Genetic predisposition and pathogenetic mechanisms of interstitial lung diseases of unknown origin Eur. Respir. J., July 1, 2001; 18(32_suppl): 17S - 29s. [Abstract] [Full Text] [PDF]

				S R Johnson and A E Tattersfield Clinical experience of lymphangioleiomyomatosis in the UK Thorax, December 1, 2000; 55(12): 1052 - 1057. [Abstract] [Full Text]