Reductions in pulmonary function detected in patients with lymphangioleiomyomatosis: An analysis of the Japanese National Research Project on Intractable Diseases database

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• 作者：Mie Hayashida ; MD ; PhD^a ; ^{mie@shinshu-u.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; Masanori Yasuo ; MD ; PhD^a ; ^{yasumasa@shinshu-u.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; Masayuki Hanaoka ; MD ; PhD^a ; ^{masayuki@shinshu-u.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; Kuniaki Seyama ; MD ; PhD^b ; ^{kseyama@juntendo.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; Yoshikazu Inoue ; MD ; PhD^c ; ^{giichi@kch.hosp.go.jp" class="auth_mail" title="E-mail the corresponding author} ; Koichiro Tatsumi ; MD ; PhD^d ; ^{tatsumi@faculty.chiba-u.jp" class="auth_mail" title="E-mail the corresponding author} ; Michiaki Mishima ; MD ; PhD^e ; ^{mishima@kuhp.kyoto-u.ac.jp" class="auth_mail" title="E-mail the corresponding author} ; the Respiratory Failure Research Group of the Ministry of Health ; Labour ; Welfare ; Japan
• 关键词：Database ; Disease progression ; Pulmonary function ; Lymphangioleiomyomatosis ; Rare lung disease
• 刊名：Respiratory Investigation
• 出版年：2016
• 出版时间：May 2016
• 年：2016
• 卷：54
• 期：3
• 页码：193-200
• 全文大小：690 K

文摘

In lymphangioleiomyomatosis (LAM), predicting lung disease progression is essential for treatment planning. However, no previous Japanese studies have attempted to predict the reductions in pulmonary function that occur in LAM patients.

Methods

The data for 89 LAM patients who had undergone ≥3 spirometry tests and whose data had been registered in the Japanese National Research Project on Intractable Diseases database between October 2009 and March 2014 were analyzed after excluding patients who had undergone (1) a lung transplant; (2) mTOR inhibitor treatment; or (3) thoracic drainage, pleurodesis, surgery, or thoracic duct ligation during the study period. The rates of change (slope) in pulmonary parameters were calculated, and their associations with clinical background factors were investigated.

Results

Among the whole study population, the median (quartiles) slope of forced expiratory volume in one second (FEV₁) was −46.7 (−95.2; −15.0) mL per year. Episodes of conservatively treated pneumothorax during the study period were found to be associated with rapid reductions in FEV₁ (% predicted). Pregnancy during the study period was associated with a reduction in FEV₁ (% predicted). When the patients were divided into those who exhibited initial FEV₁ (% predicted) values of >70% (Group A) and ≤70% (Group B), Group B displayed significantly faster reductions in FEV₁ (% predicted) than Group A.

Conclusions

LAM patients whose initial FEV₁ (% predicted) values are ≤70% subsequently exhibit rapid reductions in their FEV₁ values, and hence, require treatment. However, the FEV₁ reduction rate varies markedly among individuals and should be monitored in all cases.