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ICD-10-CM Code J84.81

Billable Code
Billable codes are sufficient justification for admission to an acute care hospital when used a principal diagnosis.
Female Only
Female Only
Code is only used for female patients.
| ICD-10 from 2011 - 2016

J84.81 is a billable ICD code used to specify a diagnosis of lymphangioleiomyomatosis. A 'billable code' is detailed enough to be used to specify a medical diagnosis.

The ICD code J8481 is used to code Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare, progressive, systemic disease that typically results in cystic lung destruction and predominantly affects women, especially during child bearing years. It occurs in more than 30% of women with tuberous sclerosis complex (TSC-LAM), a heritable syndrome that is associated with seizures, cognitive impairment and benign tumors in multiple tissues. Most LAM patients who present for medical evaluation have the sporadic form of the disease (S-LAM), however, which is not associated with other manifestations of tuberous sclerosis complex. Mild cystic changes consistent with LAM have been described in 10-15% of men with TSC, but symptomatic LAM in males is extremely rare. Sporadic LAM occurs exclusively in women, with one published exception to date. Both TSC-LAM and S-LAM are associated with mutations in tuberous sclerosis genes. Lung destruction in LAM is a consequence of diffuse infiltration by neoplastic smooth muscle-like cells, which invade all lung structures including the lymphatics, airway walls, blood vessels, and interstitial spaces. The consequence of obstruction of the vessels and airways include chylous fluid accumulations, hemoptysis, airflow obstruction and pneumothorax. The typical disease course is characterized by progressive dyspnea on exertion, punctuated by recurrent pneumothoraces and, in some patients, chylous pleural effusions or ascites. Modern estimates for median survival in LAM have varied from 10 to 30 years, based on whether hospital based or population based cohorts are studied. Most patients have dyspnea on exertion with daily activities by 10 years after symptom onset and many will require supplemental oxygen over that interval. An FDA approved drug for treatment of LAM, sirolimus, is now available for stabilization of lung function decline. Lung transplant remains the option of last resort for patients with advanced disease.

MeSH Code: D018192
ICD 9 Code: 516.4

Figure A shows the location of the lungs and airways in the body. The inset image shows a cross-section of a healthy lung. Figure B shows a view of the lungs with LAM and a collapsed lung (pneumothorax). The inset image shows a cross-section of a lung with LAM.

Source: Wikipedia

Coding Notes for J84.81 Info for medical coders on how to properly use this ICD-10 code

Inclusion Terms:
Inclusion Terms
Inclusion Terms are a list of concepts for which a specific code is used. The list of Inclusion Terms is useful for determining the correct code in some cases, but the list is not necessarily exhaustive.

MS-DRG Mapping

  • DRG Group #196-198 - Interstitial lung disease with MCC.
  • DRG Group #196-198 - Interstitial lung disease with CC.
  • DRG Group #196-198 - Interstitial lung disease without CC or MCC.

Related Concepts SNOMET-CT

  • Lymphangioleiomyomatosis due to tuberous sclerosis syndrome (disorder)

ICD-10-CM Alphabetical Index References for 'J84.81 - Lymphangioleiomyomatosis'

The ICD-10-CM Alphabetical Index links the below-listed medical terms to the ICD code J84.81. Click on any term below to browse the alphabetical index.

Lymphangioleiomyomatosis (J84.81)
Lymphangiomyomatosis (J84.81)


Lymphangioleiomyomatosis (exact match)

This is the official exact match mapping between ICD9 and ICD10, as provided by the General Equivalency mapping crosswalk. This means that in all cases where the ICD9 code 516.4 was previously used, J84.81 is the appropriate modern ICD10 code.

Parent Code: J84.8 - Other specified interstitial pulmonary diseases

** This Document Provided By ICD.Codes **
Source: http://icd.codes/icd10cm/J8481