J84.81 maps to CMS-HCC V28 280. A source-labeled RAF reference is available. Confirm the documented diagnosis and applicable coding requirements. MEAT criteria · RAF Calculator · HCC Buddy coding tools

ICD-10-CM Code View

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Code lookupJ84.81

Effective periodFY2026 Apr Update (Apr 1-Sep 30)

FY 2026 Apr update / Diseases of the respiratory system (J00-J99) / Other respiratory diseases principally affecting the interstitium (J80-J84)

J84.81

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Lymphangioleiomyomatosis

A rare lung disease where smooth muscle cells grow abnormally in the lungs and lymph vessels, primarily affecting women of childbearing age.

Buddy Insight

Lymphangioleiomyomatosis is a rare, progressive cystic lung disease caused by proliferation of abnormal smooth muscle-like cells, occurring almost exclusively in women of childbearing age.

Overview Risk Models Code Book Documentation Audit Notes Edition comparison

CMS-HCC V28

Mapped

HCC 280

Chronic Obstructive Pulmonary...

Coefficient HCC 280: 0.319 (Community Non-Dual Aged (CNA))

CMS-HCC V24

Historical

Not used for standard CY2026 Medicare Advantage payment. Select a historical period to review V24.

Not used for CY2026 payment

ACA/HHS

Mapped

HCC 162

HHS-HCC 162 adult, RAF varies...

Code-level coefficient reference

ESRD/PACE

Mapped

HCC 112

Fibrosis of Lung and Other Ch...

Code-level coefficient reference

RXHCC

Mapped

HCC 227

Pulmonary Fibrosis, Except Id...

Code-level coefficient reference

Code Book Path

Official

J84Other interstitial pulmonary diseases J84.8Other specified interstitial pulmonary diseases J84.81Lymphangioleiomyomatosis

Inclusion Terms

Official

Lymphangiomyomatosis

Excludes 2

Official

certain conditions originating in the perinatal period (P04-P96)Inherited from J00-J99, J84
certain infectious and parasitic diseases (A00-B99)Inherited from J00-J99, J84
complications of pregnancy, childbirth and the puerperium (O00-O9A)Inherited from J00-J99, J84
congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)Inherited from J00-J99, J84
endocrine, nutritional and metabolic diseases (E00-E88)Inherited from J00-J99, J84
injury, poisoning and certain other consequences of external causes (S00-T88)Inherited from J00-J99, J84
neoplasms (C00-D49)Inherited from J00-J99, J84
smoke inhalation (T59.81-)Inherited from J00-J99, J84
symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)Inherited from J00-J99, J84
lung diseases due to external agents (J60-J70)Inherited from J00-J99, J84

Related Codes

Official

J84.82Adult pulmonary Langerhans cell histiocytosis J84.83Surfactant mutations of the lung J84.84Other interstitial lung diseases of childhood J84.89Other specified interstitial pulmonary diseases

Includes

Official

No Includes notes are included in this display for J84.81. Check the code and parent instructions in the Code Book.

Excludes 1

Official

drug-induced interstitial lung disorders (J70.2-J70.4)Inherited from J84, J84.8
interstitial emphysema (J98.2)Inherited from J84, J84.8
exogenous lipoid pneumonia (J69.1)Inherited from J84, J84.8
unspecified lipoid pneumonia (J69.1)Inherited from J84, J84.8

Code First

Official

No Code First sequencing instructions are included in this display for J84.81. Check the code and parent instructions in the Code Book.

Use Additional

Official

code, where applicable, to identify:Inherited from J00-J99
exposure to environmental tobacco smoke (Z77.22)Inherited from J00-J99
exposure to tobacco smoke in the perinatal period (P96.81)Inherited from J00-J99
history of tobacco dependence (Z87.891)Inherited from J00-J99
occupational exposure to environmental tobacco smoke (Z57.31)Inherited from J00-J99
tobacco dependence (F17.-)Inherited from J00-J99
tobacco use (Z72.0)Inherited from J00-J99

Code Also

Official

, if applicable, associated conditionInherited from J84

Buddy Documentation Tip

HCC Buddy guidance

High-resolution computed tomography showing diffuse thin-walled cysts distributed throughout both lungs

Documentation of gender and age (predominantly affects women of reproductive age)

VEGF-D blood level or lung biopsy confirming LAM cells if performed

Assessment for tuberous sclerosis complex association (genetic testing, skin/renal evaluation)

MEAT Support

HCC Buddy guidance

High-resolution computed tomography showing diffuse thin-walled cysts distributed throughout both lungs

Documentation of gender and age (predominantly affects women of reproductive age)

VEGF-D blood level or lung biopsy confirming LAM cells if performed

Assessment for tuberous sclerosis complex association (genetic testing, skin/renal evaluation)

Audit Caution

HCC Buddy guidance

Missing the association with tuberous sclerosis complex — always code Q85.1 separately if TSC is present

Confusing cystic lung changes of LAM with emphysema, especially in female patients who may not have smoking history

Failing to capture complications (pneumothorax, chylothorax, renal angiomyolipomas) as separate diagnoses

Using generic ILD codes when the specific LAM diagnosis is documented

Common Mistakes

HCC Buddy guidance

J43.9 — Emphysema unspecified can appear similar on imaging with cystic changes but has a different etiology and demographic

Q85.1 — Tuberous sclerosis may be associated with LAM and should be coded separately if present

J84.89 — Other specified interstitial pulmonary diseases should not be used when LAM is specifically documented

J93.11 — Primary spontaneous pneumothorax is a common complication of LAM that should be coded separately

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is J84.81 an HCC code?

Yes. J84.81 (Lymphangioleiomyomatosis) maps to HCC 280, Chronic Obstructive Pulmonary Disease, Interstitial Lung Disorders, and Other Chronic Lung Disorders under the CMS-HCC V28 risk adjustment model, with a source-labeled community non-dual aged reference coefficient of 0.319. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes. It is billable for payment year 2026.

Coder answer: J84.81 is billable and maps to V28 HCC 280, Chronic Obstructive Pulmonary Disease, Interstitial Lung Disorders, and Other Chronic Lung Disorders. Open it in the Code Book for the tabular path, RAF, and MEAT checklist.

Code: J84.81
Description: Lymphangioleiomyomatosis
HCC (V28): HCC 280 — Chronic Obstructive Pulmonary Disease, Interstitial Lung Disorders, and Other Chronic Lung Disorders
RAF reference coefficient: 0.319
Billable: Yes
Payment year: 2026

HCC Category Mapping

V28HCC 280, Chronic Obstructive Pulmonary Disease, Interstitial Lung Disorders, and Other Chronic Lung Disorders

0.319

ESRDHCC 112, Fibrosis of Lung and Other Chronic Lung Disorders

Not separately weighted

RxHCCHCC 227, Pulmonary Fibrosis, Except Idiopathic

Not separately weighted

These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. Weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.

Work J84.81 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT review for J84.81

For J84.81, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.

MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
EEvaluate: test results, medication response, or physical findings reviewed by the provider
AAssess: explicit mention in the assessment or plan with acknowledgment of status
TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Coder workflow notes

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What This Code Means

J84.81 is the ICD-10-CM diagnosis code for lymphangioleiomyomatosis. A rare lung disease where smooth muscle cells grow abnormally in the lungs and lymph vessels, primarily affecting women of childbearing age. J84.81 sits in the ICD-10-CM chapter for diseases of the respiratory system (j00-j99), within the section covering other respiratory diseases principally affecting the interstitium (j80-j84).

Under the CMS-HCC V28 risk adjustment model, J84.81 maps to Chronic Obstructive Pulmonary Disease, Interstitial Lung Disorders, and Other Chronic Lung Disorders (HCC 280) with a source-labeled community, non-dual, aged reference coefficient of 0.319. For CY2026 non-PACE Medicare Advantage, CMS uses 100% of the 2024 CMS-HCC model (V28). PACE uses a separate model blend. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes.

Document gender and age when applicable, as this disease predominantly affects young women. For J84.81, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for J84.81 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•Document gender and age when applicable, as this disease predominantly affects young women
•Note any associated complications such as pneumothorax or chylothorax

Clinical Significance

Lymphangioleiomyomatosis is a rare, progressive cystic lung disease caused by proliferation of abnormal smooth muscle-like cells, occurring almost exclusively in women of childbearing age. It may be sporadic or associated with tuberous sclerosis complex. This condition requires specialized management including mTOR inhibitor therapy, transplant evaluation, and management of complications like pneumothorax and chylothorax.

Documentation Requirements

✓High-resolution computed tomography showing diffuse thin-walled cysts distributed throughout both lungs
✓Documentation of gender and age (predominantly affects women of reproductive age)
✓VEGF-D blood level or lung biopsy confirming LAM cells if performed
✓Assessment for tuberous sclerosis complex association (genetic testing, skin/renal evaluation)
✓Treatment plan including mTOR inhibitor therapy (sirolimus), pneumothorax management, and transplant evaluation if advanced

Commonly Confused Codes

•J43.9: Emphysema unspecified can appear similar on imaging with cystic changes but has a different etiology and demographic
•Q85.1: Tuberous sclerosis may be associated with LAM and should be coded separately if present
•J84.89: Other specified interstitial pulmonary diseases should not be used when LAM is specifically documented
•J93.11: Primary spontaneous pneumothorax is a common complication of LAM that should be coded separately