The epidemiology of lymphangioleiomyomatosis in Japan: A nationwide cross-sectional study of presenting features and prognostic factors

  1. Mie HAYASHIDA1,
  2. Kuniaki SEYAMA2,
  3. Yoshikazu INOUE3,
  4. Keisaku FUJIMOTO1,
  5. Keishi KUBO1,*,
  6. The Respiratory Failure Research Group of the Japanese Ministry of Health, Labor, and Welfare1

Article first published online: 6 JUN 2007

DOI: 10.1111/j.1440-1843.2007.01101.x

Issue

Respirology

Respirology

Volume 12, Issue 4, pages 523–530, July 2007

Additional Information

How to Cite

HAYASHIDA, M., SEYAMA, K., INOUE, Y., FUJIMOTO, K., KUBO, K. and The Respiratory Failure Research Group of the Japanese Ministry of Health, Labor, and Welfare (2007), The epidemiology of lymphangioleiomyomatosis in Japan: A nationwide cross-sectional study of presenting features and prognostic factors. Respirology, 12: 523–530. doi: 10.1111/j.1440-1843.2007.01101.x

Author Information

  1. 1

    Department of Internal Medicine, School of Medicine, Shinshu University, Matsumoto,

  2. 2

    Department of Respiratory Medicine, School of Medicine, Juntendo University, Tokyo and,

  3. 3

    Department of Diffuse Lung Diseases and Respiratory Failure, National Hospital Organization, Kinki-Chuo Chest Medical Center, Osaka, Japan

*Keishi Kubo, Department of Internal Medicine, Shinshu University School of Medicine, 3-1-1, Asahi, Matsumoto 390-8621, Japan. Email: keishik@hsp.md.shinshu-u.ac.jp

Publication History

  1. Issue published online: 6 JUN 2007
  2. Article first published online: 6 JUN 2007
  3. Received 2 July 2006; invited to revise 29 July and 6 October 2006; revised 31 August and 31 October 2006; accepted 29 November 2006 (Associate Editor: Philip Eng).

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Keywords:

  • lung function;
  • lymphangioleiomyomatosis;
  • phenotype;
  • presenting features;
  • prognosis

Background and objective:  To evaluate the characteristics and prognostic factors of Japanese patients with lymphangioleiomyomatosis (LAM).

Methods:  A nationwide survey to identify patients with LAM was conducted by questionnaire. Survival probability was estimated using the Kaplan–Meier method, and the prognostic factors were analysed by Cox regression.

Results:  Data were collected on 173 patients with pulmonary LAM. The major presenting features were pneumothorax (43%) and exertional dyspnoea (37%). The survival probabilities for patients presenting with exertional dyspnoea (Group A) were 85%, 60% and 47% after 5, 10 and 15 years, respectively, and for patients presenting with pneumothorax (Group B) were 95%, 89% and 89%, respectively. Although the age at symptom onset was higher among patients in Group A than in Group B, Cox regression revealed that the presenting feature was a prognostic factor independent of age at symptom onset (Group A/B hazard ratio = 5.732, P < 0.01). In the subgroup of patients whose initial FEV1 was >1000 mL, or FEV1/FVC >40%, or %DLCO >40%, the rate of deterioration in these tests was greater in Group A than in Group B (P < 0.01 for FEV1, P < 0.05 for FEV1/FVC and %DLCO).

Conclusions:  There are two possible subgroups of LAM patients. One subgroup that presented with pneumothorax, had onset of symptoms at a younger age and a more favourable prognosis; the other presented with exertional dyspnoea, had onset of symptoms at an older age and a poorer prognosis.

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