J84.81 Lymphangioleiomyomatosis

Billable ICD-10-CM code, FY2026. Acts as a Major Complication or Comorbidity (MCC) as a secondary diagnosis.

Applicable to

• Lymphangiomyomatosis

Applies from J84 Other interstitial pulmonary diseases

Excludes1 (never code together)

• drug-induced interstitial lung disorders (J70.2-J70.4)
• interstitial emphysema (J98.2)

Excludes2 (not included here)

• lung diseases due to external agents (J60-J70)

Code also

• , if applicable, associated condition

Applies from J84.8 Other specified interstitial pulmonary diseases

Excludes1 (never code together)

• exogenous lipoid pneumonia (J69.1)
• unspecified lipoid pneumonia (J69.1)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 280 — Chronic Obstructive Pulmonary Disease, Interstitial Lung Disorders, and Other Chronic Lung Disorders
• CMS-HCC V22: HCC 112 — Fibrosis of Lung and Other Chronic Lung Disorders
• RxHCC V08: HCC 227 — Pulmonary Fibrosis, Except Idiopathic
• ESRD V24: HCC 112

MS-DRG index

Defines the logic of DRG(s) 196-198 in MDC 04.

Approximate ICD-9-CM (GEMs)

• 516.4 Lymphangioleiomyomatosis

Code annotations containing back-references to J84.81

Back-references to J84.81

• Code First: J93.12 Secondary spontaneous pneumothorax ("lymphangioleiomyomatosis (J84.81)")

Back-references to J84

• Code Also: I27.23 Pulmonary hypertension due to lung diseases and hypoxia ("interstitial lung disease (J84.-)")

Diagnosis Index entries for J84.81

• Lymphangioleiomyomatosis
• Lymphangiomyomatosis

Nearby codes

• J84.17 Other interstitial pulmonary diseases with fibrosis in diseases classified elsewhere
• J84.170 Interstitial lung disease with progressive fibrotic phenotype in diseases classified elsewhere
• J84.178 Other interstitial pulmonary diseases with fibrosis in diseases classified elsewhere
• J84.2 Lymphoid interstitial pneumonia
• J84.8 Other specified interstitial pulmonary diseases
• J84.81 Lymphangioleiomyomatosis (this code)
• J84.82 Adult pulmonary Langerhans cell histiocytosis
• J84.83 Surfactant mutations of the lung
• J84.84 Other interstitial lung diseases of childhood
• J84.841 Neuroendocrine cell hyperplasia of infancy
• J84.842 Pulmonary interstitial glycogenosis