Abstract
Background: Lymphangioleiomyomatosis (LAM) is a rare and progressive disease of young women with no effective treatment. Previous estimates of 10 year survival, based mostly on case series or patients from tertiary centres, have ranged from 40% to 79%; no data are available on the progression of respiratory disability. In order to provide data for patients and for planning intervention studies, we have looked at the time course of LAM using a national cohort.
Methods: Time to death, time to MRC dyspnoea grades 2–5, and need for oxygen in patients on the UK LAM database were analysed using Kaplan-Meier analysis and Cox regression.
Results: Fifty seven of 72 patients responded with a median duration of follow up of 12.6 years (range 2.3–37) from the onset of symptoms. Ten year survival was 91% from onset of symptoms but varied widely with 11 patients alive after 20 years. Median time to MRC grade 3 dyspnoea (breathless walking on the flat) was 9.3 years (95% CI 5.1 to 13.4) from onset of symptoms.
Conclusions: Survival from LAM appears to be better than that reported in early studies. These data should be helpful for patients and for planning clinical trials.
Full Text
The Full Text of this article is available as a PDF (86.9 KB).
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- BEHRMAN S. John LIZARS. Centenary of a forgotten pioneer of the surgery of trigeminal neuralgia. Br Med J. 1960 Dec 3;2(5213):1665–1666. [PMC free article] [PubMed] [Google Scholar]
- Bestall J. C., Paul E. A., Garrod R., Garnham R., Jones P. W., Wedzicha J. A. Usefulness of the Medical Research Council (MRC) dyspnoea scale as a measure of disability in patients with chronic obstructive pulmonary disease. Thorax. 1999 Jul;54(7):581–586. doi: 10.1136/thx.54.7.581. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Chu S. C., Horiba K., Usuki J., Avila N. A., Chen C. C., Travis W. D., Ferrans V. J., Moss J. Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis. Chest. 1999 Apr;115(4):1041–1052. doi: 10.1378/chest.115.4.1041. [DOI] [PubMed] [Google Scholar]
- Corrin B., Liebow A. A., Friedman P. J. Pulmonary lymphangiomyomatosis. A review. Am J Pathol. 1975 May;79(2):348–382. [PMC free article] [PubMed] [Google Scholar]
- Johnson S. R., Tattersfield A. E. Clinical experience of lymphangioleiomyomatosis in the UK. Thorax. 2000 Dec;55(12):1052–1057. doi: 10.1136/thorax.55.12.1052. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Johnson S. R., Tattersfield A. E. Decline in lung function in lymphangioleiomyomatosis: relation to menopause and progesterone treatment. Am J Respir Crit Care Med. 1999 Aug;160(2):628–633. doi: 10.1164/ajrccm.160.2.9901027. [DOI] [PubMed] [Google Scholar]
- Johnson S. Rare diseases. 1. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms. Thorax. 1999 Mar;54(3):254–264. doi: 10.1136/thx.54.3.254. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Kitaichi M., Nishimura K., Itoh H., Izumi T. Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors. Am J Respir Crit Care Med. 1995 Feb;151(2 Pt 1):527–533. doi: 10.1164/ajrccm.151.2.7842216. [DOI] [PubMed] [Google Scholar]
- McLoughlin L., Thomas G., Hasan K. Pregnancy and lymphangioleiomyomatosis: anaesthetic management. Int J Obstet Anesth. 2003 Jan;12(1):40–44. doi: 10.1016/s0959-289x(02)00138-3. [DOI] [PubMed] [Google Scholar]
- Silverstein E. F., Ellis K., Wolff M., Jaretzki A., 3rd Pulmonary lymphangiomyomatosis. Am J Roentgenol Radium Ther Nucl Med. 1974 Apr;120(4):832–850. doi: 10.2214/ajr.120.4.832. [DOI] [PubMed] [Google Scholar]
- Sullivan E. J. Lymphangioleiomyomatosis: a review. Chest. 1998 Dec;114(6):1689–1703. doi: 10.1378/chest.114.6.1689. [DOI] [PubMed] [Google Scholar]
- Taylor J. R., Ryu J., Colby T. V., Raffin T. A. Lymphangioleiomyomatosis. Clinical course in 32 patients. N Engl J Med. 1990 Nov 1;323(18):1254–1260. doi: 10.1056/NEJM199011013231807. [DOI] [PubMed] [Google Scholar]
- Urban T., Lazor R., Lacronique J., Murris M., Labrune S., Valeyre D., Cordier J. F. Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P). Medicine (Baltimore) 1999 Sep;78(5):321–337. doi: 10.1097/00005792-199909000-00004. [DOI] [PubMed] [Google Scholar]
- Whale C. I., Johnson S. R., Phillips K. G., Newton S. A., Lewis S. A., Tattersfield A. E. Lymphangioleiomyomatosis: a case-control study of perinatal and early life events. Thorax. 2003 Nov;58(11):979–982. doi: 10.1136/thorax.58.11.979. [DOI] [PMC free article] [PubMed] [Google Scholar]