ORPHAN LUNG DISEASE

Survival and disease progression in UK patients with lymphangioleiomyomatosis

S R Johnson¹, C I Whale², R B Hubbard², S A Lewis², A E Tattersfield²

¹ Division of Therapeutics and Molecular Medicine, University of Nottingham, Nottingham, UK
² Division of Respiratory Medicine, University of Nottingham, Nottingham, UK

Correspondence to:
Correspondence to:
Dr S R Johnson
Division of Therapeutics and Molecular Medicine, Queens Medical Centre, University of Nottingham, Nottingham NG7 2UH, UK; simon.johnson{at}nottingham.ac.uk

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.

Keywords: lymphangioleiomyomatosis; survival; MRC dyspnoea scale; Kaplan-Meier analysis

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

Relevant Article

Airwaves

Wisia Wedzicha
Thorax 2004 59: 729. [Extract] [Full Text] [PDF]

This article has been cited by other articles:

• McCartney, R., Facey, N., Chalmers, G., Mathers, A., Roberts, F., Irvine, G., Anderson, K. (2009). Multiple pneumothoraces during second and third trimesters as first presentation of lymphangioleiomyomatosis. Obstet Med 2: 84-86 [Abstract] [Full Text]  
• Brant, S., Parker, M. S., Brath, L. K., Grimes, M. M. (2009). A 47-Year-Old Woman With Progressive Dyspnea and Recurrent Pneumothoraces. Chest 135: 1389-1394 [Full Text]  
• Schmithorst, V. J., Altes, T. A., Young, L. R., Franz, D. N., Bissler, J. J., McCormack, F. X., Dardzinski, B. J., Brody, A. S. (2009). Automated Algorithm for Quantifying the Extent of Cystic Change on Volumetric Chest CT: Initial Results in Lymphangioleiomyomatosis. Am. J. Roentgenol. 192: 1037-1044 [Abstract] [Full Text]  
• Lynch, J. P. III (2009). Rare Interstitial Lung Diseases: Pulmonary Langerhans Cell Histiocytosis, Lymphangioleiomyomatosis, and Cryptogenic Organizing Pneumonia. ACCP Pulmonary Med Brd Rev 25: 585-618 [Full Text]  
• McCormack, F. X. (2008). Lymphangioleiomyomatosis*: A Clinical Update. Chest 133: 507-516 [Abstract] [Full Text]  
• Johnson, S. R. (2006). Lymphangioleiomyomatosis.. Eur Respir J 27: 1056-1065 [Abstract] [Full Text]  
• Jaffe, A (2005). Orphan lung diseases in childhood: still unadopted?. Thorax 60: 892-894 [Full Text]  
• Black, J. L., Ge, Q., Boustany, S., Johnson, P. R. A., Poniris, M. H., Glanville, A. R., Oliver, B. G. G., Moir, L. M., Burgess, J. K. (2005). In vitro studies of lymphangioleiomyomatosis. Eur Respir J 26: 569-576 [Abstract] [Full Text]  
• Ng, C. S. H., Wan, S., Lee, T. W., Yim, A. P. C. (2005). Cystic lesions of the lung: a forgotten menace. Eur Respir J 26: 748-749 [Full Text]  
• Abbott, G. F., Rosado-de-Christenson, M. L., Frazier, A. A., Franks, T. J., Pugatch, R. D., Galvin, J. R. (2005). From the Archives of the AFIP: Lymphangioleiomyomatosis: Radiologic-Pathologic Correlation. RadioGraphics 25: 803-828 [Abstract] [Full Text]