Lymphangioleiomyomatosis

This code identifies lymphangioleiomyomatosis (LAM), a rare, progressive lung disease characterized by the abnormal proliferation of smooth muscle-like cells throughout the lungs, lymph nodes, and kidneys. This proliferation leads to the formation of cysts in the lung parenchyma, which can cause airflow obstruction, chylous effusions, and recurrent pneumothorax.

Assign this code for documented cases of LAM, whether sporadic or associated with tuberous sclerosis complex (TSC). Documentation should clearly indicate a diagnosis of LAM, often confirmed by imaging (e.g., CT scan showing diffuse thin-walled cysts) and/or lung biopsy.

• Differentiate from other interstitial lung diseases; LAM has distinct pathological and radiological features.
• If LAM is associated with tuberous sclerosis complex, code the underlying TSC first, followed by this code.
• Ensure documentation specifies the diagnosis of LAM rather than just symptoms like pneumothorax or chylothorax, which are manifestations.