J84.82
BillableAdult pulmonary Langerhans cell histiocytosis
HCC Category Mapping
V28HCC 280 — Chronic Obstructive Pulmonary Disease
0.334V24HCC 112 — Fibrosis of Lung and Other Chronic Lung Disorders
0.268ESRDHCC 112 — Fibrosis of Lung and Other Chronic Lung Disorders
0.000RxHCCHCC 227 — Sarcoidosis, Pulmonary Alveolar Disorders
0.000What This Code Means
A rare lung disease in adults where abnormal immune cells accumulate in lung tissue, often related to smoking history.
Coding Tips
- •Confirm diagnosis through biopsy showing Langerhans cell infiltration
- •Document smoking status, as this is strongly associated with the condition
Clinical Significance
Adult pulmonary Langerhans cell histiocytosis is a rare smoking-related cystic and nodular lung disease caused by clonal proliferation of Langerhans cells in the lungs. This condition is strongly associated with cigarette smoking and requires aggressive smoking cessation, monitoring for disease progression, and potential immunosuppressive therapy. It can progress to respiratory failure and may require transplant evaluation.
Documentation Requirements
- ✓High-resolution computed tomography showing combination of nodules and cysts predominantly in upper and middle lung zones with costophrenic angle sparing
- ✓Bronchoalveolar lavage showing elevated CD1a+ cells (>5%) or lung biopsy confirming Langerhans cell infiltration
- ✓Documented smoking history (strong association with current/recent smoking)
- ✓Evaluation for extrapulmonary Langerhans cell histiocytosis involvement (bone, pituitary, skin)
- ✓Pulmonary function tests documenting the pattern and severity of lung impairment
Commonly Confused Codes
J84.81 — Lymphangioleiomyomatosis also causes cystic lung disease but in young women with different cyst morphologyJ43.9 — Emphysema unspecified can appear similar with upper lobe cystic changes in smokersC96.6 — Unifocal Langerhans cell histiocytosis is a different, more localized formJ84.89 — Other specified interstitial pulmonary diseases should not be used when PLCH is specifically diagnosed
Code Hierarchy
└J84Other interstitial pulmonary diseases└J84.8Other specified interstitial pulmonary diseases└J84.82Adult pulmonary Langerhans cell histiocytosis
└J84.82Adult pulmonary Langerhans cell histiocytosis