Lymphangioleiomyomatosis

This code represents lymphangioleiomyomatosis (LAM), a rare, progressive lung disease characterized by the abnormal proliferation of smooth muscle-like cells in the lungs, lymphatic system, and kidneys. This proliferation leads to the formation of cysts in the lung parenchyma, causing airflow obstruction and often chylous effusions.

Assign this code for patients diagnosed with sporadic LAM or LAM associated with tuberous sclerosis complex. Documentation should clearly state the diagnosis of lymphangioleiomyomatosis, typically confirmed by imaging (CT scan showing characteristic lung cysts) and/or lung biopsy.

• Differentiate from other cystic lung diseases; LAM has distinct pathological features.
• Consider co-occurring conditions like tuberous sclerosis (237.2) if applicable, as LAM can be a manifestation.
• Be mindful of laterality for effusions if documented, though the primary diagnosis is systemic.