A Case Series of Lymphangioleiomyomatosis, a Rare Cystic Lung Disease

Sedef S., MUTLU P.

Respirology Case Reports, vol.14, no.3, 2026 (ESCI, Scopus) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 14 Issue: 3
  • Publication Date: 2026
  • Doi Number: 10.1002/rcr2.70535
  • Journal Name: Respirology Case Reports
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus
  • Keywords: pneumothorax, pulmonary lymphangioleiomyomatosis, sirolimus, tuberous sclerosis
  • Çanakkale Onsekiz Mart University Affiliated: Yes

Abstract

Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease that predominantly affects women of childbearing age. In this case series, we presented five cases diagnosed with LAM in our clinic in the last 2 years. All cases were female, and their ages ranged from 40 to 54 years. The primary symptoms of LAM seen in the cases were exertional dyspnea and cough. Radiological examination revealed multiple bilateral thin-walled air cysts on high-resolution computed tomography (HRCT) in all cases. In three cases, the diagnosis of LAM was made histopathologically by wedge resection. Awareness of this rare disease's clinical and radiological features is critical for early diagnosis and timely initiation of treatment.