Division of Pulmonary and Critical Care Medicine, Gonda 18 South, Mayo Clinic, 200 First St. SW, Rochester, MN 55905, USA.
Respir Med. 2012 Nov;106(11):1586-90. doi: 10.1016/j.rmed.2012.07.007. Epub 2012 Aug 4.
To assess the frequency of cystic lung disease suggestive of pulmonary lymphangiomyomatosis in men with tuberous sclerosis complex.
Retrospective review of CT scans of the chest and abdomen on 29 men with tuberous sclerosis complex encountered during a 13-year period, 1998 to 2010.
Cystic lung disease (defined as ≥4 cysts) was seen in 11 of 29 men (38%) with tuberous sclerosis complex. The mean age of those with cystic lung disease was 46.3 ± 19.1 years. None of the patients had experienced pneumothorax or chylothorax and none had undergone a lung biopsy for evaluation of cystic lung disease. Lymphangiomyomatosis had been diagnosed in two of 11 patients with cystic lung disease. Renal angiomyolipomas were demonstrated in 14 of 25 patients (56%) with renal imaging available; all 14 had multiple bilateral renal involvement. In this limited cohort of patients, the presence of cystic lung disease did not correlate with age, smoking exposure or the presence of renal AMLs.
We conclude that tuberous sclerosis complex -related lymphangiomyomatosis may not be rare in men but is milder in severity.
评估男性结节性硬化症患者中疑似肺淋巴管肌瘤病的肺囊性疾病的频率。
回顾性分析了 1998 年至 2010 年期间 29 例结节性硬化症患者的胸部和腹部 CT 扫描。
在 29 例结节性硬化症患者中，有 11 例（38%）存在囊性肺病（定义为≥4 个囊肿）。有囊性肺病的患者平均年龄为 46.3±19.1 岁。所有患者均未发生气胸或乳糜胸，也未进行肺活检以评估囊性肺病。在有囊性肺病的 11 例患者中，有 2 例被诊断为淋巴管肌瘤病。有肾脏影像学检查结果的 25 例患者中有 14 例（56%）存在肾血管平滑肌脂肪瘤；所有 14 例患者均有多发性双侧肾受累。在这一有限的患者队列中，囊性肺病的存在与年龄、吸烟暴露或肾 AML 的存在均无相关性。
我们的结论是，结节性硬化症相关的淋巴管肌瘤病在男性中可能并不罕见，但病情较轻。
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