肺淋巴管平滑肌瘤病(PLAM)首发症状缺少特异性,临床上常易误诊或漏诊,而在诊断后的处理上缺乏可以参考的权威文献。目前其发病机制亦不清,单一的医学中心又很难募集到足够的病例数,更不用说临床试验,故临床上该病的循证医学证据较少。故本文将国内经病理证实的PLAM的临床资料进行回顾分析,旨在提高其诊治能力。 The first symptom of pulmonary lymphangioleiomyomatosis (PLAM) lacks specificity and is often misdiagnosed or missed as a clinical diagnosis. However, there is a lack of authoritative literature on the treatment after diagnosis. At present, its pathogenesis is also unclear. It is very difficult for a single medical center to raise sufficient numbers of cases, let alone clinical trials. Therefore, there is less evidence-based medical evidence for this disease in the clinic. Therefore, this paper will review the clinical data of pathologically confirmed PLAM in order to improve its diagnosis and treatment ability.