Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare systematic neoplastic disease exclusively seen in middle-aged women with an incidence of 5-9 per million. They can occur sporadically or in association with tuberous sclerosis. Histopathological diagnosis is the gold standard. Median transplant-free survival from the time of diagnosis is 23 years. We here by present premenstrual female with history of recurrent dyspnea with differential diagnosis for various interstitial lung disease diagnosed to have LAM. She was managed with bronchodilator therapy and pulmonary rehabilitation as per European respiratory society guidelines.