Lymphangioleiomyomatosis associated with tuberous sclerosis complex

Description

This is a rare lung disease that results in a proliferation of disorderly smooth muscle growth (leiomyoma) throughout the lungs, in the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion). This diagnosis is associated with a rare multi-system genetic disease that causes non-malignant tumours to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin.

Exclusions from above levels

• 01 – Certain infectious or parasitic diseases
• 05 – Endocrine, nutritional or metabolic diseases
• 18 – Complications of pregnancy, childbirth and the puerperium