ICD-10 Code J84.81 – Lymphangioleiomyomatosis (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code J84.81 – Lymphangioleiomyomatosis
What it is
Lymphangioleiomyomatosis is a rare lung disease that causes abnormal smooth muscle-like cells to grow in the lungs and related structures. It can lead to progressive cystic changes and reduced lung function.
Clinical signs
Common findings include shortness of breath, recurrent pneumothorax, and diffuse thin-walled lung cysts on imaging. Clinical features vary; refer to documentation.
When to use this code
Use J84.81 when the provider documents lymphangioleiomyomatosis, including pulmonary LAM or tuberous sclerosis–associated LAM. Code it when the diagnosis is confirmed or clearly established in the record.
Do not assume the condition from nonspecific cystic lung disease alone. If the note only suggests LAM, check documentation for a final diagnosis.
Do not use for
Do not use this code for other interstitial lung diseases, emphysema, or unspecified pulmonary cysts. Check documentation if the record does not clearly name lymphangioleiomyomatosis.
Coding tip
Always code from the provider’s stated diagnosis, not imaging suspicion alone.