J84.81

HCC Category Mapping

What This Code Means

A rare lung disease where smooth muscle cells grow abnormally in the lungs and lymph vessels, primarily affecting women of childbearing age.

Coding Tips

• •Document gender and age when applicable, as this disease predominantly affects young women
• •Note any associated complications such as pneumothorax or chylothorax

Clinical Significance

Lymphangioleiomyomatosis is a rare, progressive cystic lung disease caused by proliferation of abnormal smooth muscle-like cells, occurring almost exclusively in women of childbearing age. It may be sporadic or associated with tuberous sclerosis complex. This condition requires specialized management including mTOR inhibitor therapy, transplant evaluation, and management of complications like pneumothorax and chylothorax.

Documentation Requirements

• ✓High-resolution computed tomography showing diffuse thin-walled cysts distributed throughout both lungs
• ✓Documentation of gender and age (predominantly affects women of reproductive age)
• ✓VEGF-D blood level or lung biopsy confirming LAM cells if performed
• ✓Assessment for tuberous sclerosis complex association (genetic testing, skin/renal evaluation)
• ✓Treatment plan including mTOR inhibitor therapy (sirolimus), pneumothorax management, and transplant evaluation if advanced

Commonly Confused Codes