LIMFANGIOLEIOMIOMATOSIS DALAM SPEKTRUM PENYAKIT PARU INTERSTISIAL : LAPORAN KASUS
DOI:
https://doi.org/10.31004/jkt.v7i1.54988Keywords:
interstitial lung disease, ILD, limfangioleiomiomatosis, LAMAbstract
Limfangioleiomiomatosis (LAM) merupakan salah satu bentuk Interstitial Lungs Disease (ILD), yang bersifat langka dan progresif yang ditandai oleh proliferasi sel otot polos atipikal dengan pembentukan kista paru multipel. Kelainan ini dapat menyebabkan gangguan pertukaran gas, sesak napas progresif, dan komplikasi pneumotoraks berulang. LAM banyak terjadi pada perempuan usia produktif. LAM juga berkaitan dengan mutasi gen TSC1 atau TSC2 yang menyebabkan proliferasi abnormal dari sel. Prevalensi LAM di tujuh negara di Amerika Serikat berkisar antara 3,4 hingga 7,8 per juta wanita dengan manifestasi klinis yang variatif. Belum ada laporan khusus yang menunjukan jumlah kejadian LAM di Indonesia. Laporan kasus ini disusun untuk memperluas pemahaman klinis tentang presentasi LAM, meningkatkan kewaspadaan terhadap diagnosis penyakit ini pada pasien dengan gejala paru kronik dan gambaran kistik paru. Seorang perempuan 41 tahun mengeluh sesak napas memberat sejak satu minggu disertai batuk berdahak kuning dan nyeri dada saat batuk; pasien memiliki riwayat sesak napas kronik sejak tiga tahun terakhir dengan riwayat hipertensi dan penyakit jantung, tanpa riwayat tuberkulosis. Pasien tinggal di lingkungan padat dengan paparan asap rokok dan asbes. Pemeriksaan fisik didapatkan ronki di seluruh lapang paru, sementara pemeriksaan laboratorium menunjukkan leukositosis (13.000/µL) dengan neutrofilia (93,2%) dan hiperglikemia (306 mg/dL). Foto toraks menunjukkan corakan bronkovaskular kasar dengan gambaran honeycomb dengan fluid level, sedangkan CT-scan toraks non-kontras memperlihatkan pelebaran bronkus berbentuk kistik di hampir seluruh lobus paru bilateral, konsisten dengan bronkiektasis kistik. Kasus ini menekankan bahwa pendekatan multidisiplin dengan pemahaman klinis dan radiologis yang komprehensif sangat penting untuk menegakkan diagnosis tepat pada penyakit paru kistik multipel serta mencegah progresivitas dan komplikasi melalui penatalaksanaan diniReferences
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