Clinico-epidemiological features of pulmonary histiocytosis X

R. Watanabe, K. Tatsumi, Shuji Hashimoto, A. Tamakoshi, T. Kuriyama

Research output: Contribution to journalArticle

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Abstract

Objective. To define the clinico-epidemiological features of pulmonary histiocytosis X in Japan. Methods. A nationwide survey was carried out in 1997 using two questionnaires. Results. The first questionnaire, which attempted to determine the number of patients during 1996, revealed that the number of patients treated at hospitals with 200 or more beds during the one-year period was estimated to be 160 (95% confidence interval: 140-180). The estimated crude prevalence among those aged 16 to 70 years was calculated as 0.27 and 0.07 per 100,000 population in males and females, respectively. The second questionnaire was concerned with the clinico-epidemiological features of the disease. Seventy-three histologically diagnosed patients were evaluated. It primarily afflicted younger adults, between the ages of 20 and 50, and showed a male predominance. Over 90% of the patients were smokers or ex-smokers and over 50% started smoking before 20 years of age, suggesting a strong association with cigarette smoking. Steroid therapy was applicable to 34% of the patients. In the patients who received steroid therapy, regression and stabilization were observed in 28% and deterioration in 36%. As for the patients for whom steroids were not required, remission occurred in 63% and progression in 10%. The ratio of remissions plus stabilization was higher in the patients who were not treated with steroids compared with those who required steroid therapy (p<0.05). Conclusion. In patients with pulmonary histiocytosis X therapeutic results obtained with steroids seemed not to be encouraging, although steroids are thought to be the most plausible treatment.

Original languageEnglish
Pages (from-to)998-1003
Number of pages6
JournalInternal Medicine
Volume40
Issue number10
DOIs
Publication statusPublished - 01-01-2001
Externally publishedYes

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Langerhans Cell Histiocytosis
Steroids
Smoking
Therapeutics
Young Adult
Japan
Confidence Intervals
Surveys and Questionnaires

All Science Journal Classification (ASJC) codes

  • Internal Medicine

Cite this

Watanabe, R. ; Tatsumi, K. ; Hashimoto, Shuji ; Tamakoshi, A. ; Kuriyama, T. / Clinico-epidemiological features of pulmonary histiocytosis X. In: Internal Medicine. 2001 ; Vol. 40, No. 10. pp. 998-1003.
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abstract = "Objective. To define the clinico-epidemiological features of pulmonary histiocytosis X in Japan. Methods. A nationwide survey was carried out in 1997 using two questionnaires. Results. The first questionnaire, which attempted to determine the number of patients during 1996, revealed that the number of patients treated at hospitals with 200 or more beds during the one-year period was estimated to be 160 (95{\%} confidence interval: 140-180). The estimated crude prevalence among those aged 16 to 70 years was calculated as 0.27 and 0.07 per 100,000 population in males and females, respectively. The second questionnaire was concerned with the clinico-epidemiological features of the disease. Seventy-three histologically diagnosed patients were evaluated. It primarily afflicted younger adults, between the ages of 20 and 50, and showed a male predominance. Over 90{\%} of the patients were smokers or ex-smokers and over 50{\%} started smoking before 20 years of age, suggesting a strong association with cigarette smoking. Steroid therapy was applicable to 34{\%} of the patients. In the patients who received steroid therapy, regression and stabilization were observed in 28{\%} and deterioration in 36{\%}. As for the patients for whom steroids were not required, remission occurred in 63{\%} and progression in 10{\%}. The ratio of remissions plus stabilization was higher in the patients who were not treated with steroids compared with those who required steroid therapy (p<0.05). Conclusion. In patients with pulmonary histiocytosis X therapeutic results obtained with steroids seemed not to be encouraging, although steroids are thought to be the most plausible treatment.",
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Watanabe, R, Tatsumi, K, Hashimoto, S, Tamakoshi, A & Kuriyama, T 2001, 'Clinico-epidemiological features of pulmonary histiocytosis X', Internal Medicine, vol. 40, no. 10, pp. 998-1003. https://doi.org/10.2169/internalmedicine.40.998

Clinico-epidemiological features of pulmonary histiocytosis X. / Watanabe, R.; Tatsumi, K.; Hashimoto, Shuji; Tamakoshi, A.; Kuriyama, T.

In: Internal Medicine, Vol. 40, No. 10, 01.01.2001, p. 998-1003.

Research output: Contribution to journalArticle

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