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著者: Yasuhito Sekimoto, Kazuhiro Suzuki, Makiko Okura, Takuo Hayashi, Hiroki Ebana, Toshio Kumasaka, Keiko Mitani, Koichi Nishino, Shouichi Okamoto, Etsuko Kobayashi, Kazuhisa Takahashi, Kuniaki Seyama
雑誌名: Sci Rep. 2021 Mar 30;11(1):7170. doi: 10.1038/s41598-021-85999-5. Epub 2021 Mar 30.
Abstract/Text
Lymphangioleiomyomatosis (LAM) is a rare destructive lung disease characterized by multiple thin-walled pulmonary cysts. The currently proposed diagnostic algorithm emphasizes the characteristic cystic appearance on high-resolution computed tomography (HRCT) so uncommon HRCT appearances present challenges to establishing the proper LAM diagnosis. The objective of this study is to accrue uncommon chest HRCT appearances, determine frequencies in both tuberous sclerosis complex (TSC)-associated LAM (TSC-LAM) and sporadic LAM (S-LAM) patients. 311 females referred to our hospital, including 272 S-LAM patients (mean age 39.2 years) and 39 TSC-LAM patients (mean age 38.3 years), were retrospectively evaluated. We found 2 types of radiologic findings likely to make HRCT cyst appearance atypical: characteristics of the cyst itself and uncommon findings in addition to cysts. We found that approximately 80% of LAM patients, whether TSC-associated or sporadic, showed typical HRCT appearance with mild to severe cystic destruction. The remaining 20% displayed unusual profiles in cyst appearance as well as additional findings aside from cyst: the former includes large cyst, thickened walls, and irregularly shaped whereas the latter includes ground glass attenuation and diffuse noncalcified nodules. It is important to be aware of various radiologic findings that make HRCT cystic appearance atypical of LAM.
PMID 33785773 Sci Rep. 2021 Mar 30;11(1):7170. doi: 10.1038/s41598-021-85999-5. Epub 2021 Mar 30.
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