ICD-10-CM 2027
ICD-10 code for Lymphangioleiomyomatosis
From the official ICD-10-CM alphabetic index entry “Lymphangioleiomyomatosis”. Page updated September 29, 2026.
About coding Lymphangioleiomyomatosis
The ICD-10-CM code for Lymphangioleiomyomatosis is J84.81 (Lymphangioleiomyomatosis).
Within J84.8 (other specified interstitial pulmonary diseases), J84.81 is specifically for Lymphangioleiomyomatosis. Related codes cover adult pulmonary Langerhans cell histiocytosis (J84.82), Surfactant mutations of the lung (J84.83), other interstitial lung diseases of childhood (J84.84) and other specified interstitial pulmonary diseases (J84.89).
As a secondary diagnosis it is an MCC (major complication or comorbidity), which can move an inpatient stay into the highest-paying "with MCC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 196 (Interstitial Lung Disease with MCC, relative weight 1.8336), DRG 197 (Interstitial Lung Disease with CC, relative weight 0.9492) and DRG 198 (Interstitial Lung Disease without CC/MCC, relative weight 0.7259), in MDC 04 (Diseases and Disorders of the Respiratory System), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Lymphangioleiomyomatosis ICD-10 codes
What is the ICD-10 code for Lymphangioleiomyomatosis?
- J84.81 — Lymphangioleiomyomatosis.
Is J84.81 billable?
- Yes. J84.81 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can J84.81 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict J84.81 as a principal diagnosis.
Is J84.81 a CC or MCC?
- J84.81 is an MCC (major complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.