LAM病人的尿中有一类物质，叫做MMP - 基质金属蛋白酶，与疾病的程度有一定相关性。多希环素是一种抗生素，但也具有抑制MMP的作用。在2006-6-15出版的新英格兰医学杂志上，有研究者报告了一例多希环素治疗LAM的结果。这是一个66岁女性病人，病史已有30年，肺功能在逐渐加重，医生采用多希环素成功地改善了病人的肺功能，FEV1从治疗前的21%提高到35%（占预计值比例），氧饱和度和活动耐力有明显改善。采用的剂量为20mg/日初始剂量，根据耐受性和尿MMP水平调整剂量，增加到每天100mg。该病例的成功治疗证明，随着对LAM发病机制认识的提高，一定会有一些新的、有效的治疗会用于LAM病人的治疗。（协和医院呼吸科 徐凯峰）
A 66-year-old woman was diagnosed with lymphangioleiomyomatosis
in 1975 after having undergone a partial nephrectomy for angiomyolipoma.
Since the diagnosis, the disease had progressed, with continuing
deterioration of pulmonary function. In February 2005, her forced
expiratory volume in one second (FEV
1) was 0.48 liter (21 percent
of the predicted value), and she was placed on the list for
lung transplantation. Before treatment with doxycycline, several
species of MMP were found to be markedly elevated in her urine,
including MMP-2, MMP-9, and a complex of MMP-9 and neutrophil
gelatinase–associated lipocalin (
Figure 1A). On the basis
of the elevated MMP levels, doxycycline was initiated at an
initial dose of 20 mg per day. Dose escalation (up to 100 mg
per day) was based on the results of monthly urinary MMP profiling
(
Figure 1A ) and on the patient's tolerance of the drug. The
patient's lung capacity (as measured on September 30, 2005)
was increased (FEV
1, 0.91 liter; 35 percent of the predicted
value), and enhanced oxygen saturation was observed (
Figure 1B).
Telegraphic speech, which had been present, resolved. The patient
has had a clear improvement in the quality of life and has been
taken off the waiting list for a lung transplant — an
outcome that was predicted by the decreasing urinary levels
of MMPs .
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