2019 ICD-10-CM Diagnosis Code J84.81

Lymphangioleiomyomatosis

    2016 2017 2018 2019 Billable/Specific Code
  • J84.81 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2019 edition of ICD-10-CM J84.81 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of J84.81 - other international versions of ICD-10 J84.81 may differ.
Applicable To
  • Lymphangiomyomatosis
The following code(s) above J84.81 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to J84.81:
  • J00-J99
    2019 ICD-10-CM Range J00-J99

    Diseases of the respiratory system

    Note
    • When a respiratory condition is described as occurring in more than one site and is not specifically indexed, it should be classified to the lower anatomic site (e.g. tracheobronchitis to bronchitis in J40).
    Type 2 Excludes
    • certain conditions originating in the perinatal period (P04-P96)
    • certain infectious and parasitic diseases (A00-B99)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • smoke inhalation (T59.81-)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    Use Additional
    • code, where applicable, to identify:
    • exposure to environmental tobacco smoke (Z77.22)
    • exposure to tobacco smoke in the perinatal period (P96.81)
    • history of tobacco dependence (Z87.891)
    • occupational exposure to environmental tobacco smoke (Z57.31)
    • tobacco dependence (F17.-)
    • tobacco use (Z72.0)
    Diseases of the respiratory system
  • J84
    ICD-10-CM Diagnosis Code J84

    Other interstitial pulmonary diseases

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • drug-induced interstitial lung disorders (J70.2-J70.4)
    • interstitial emphysema (J98.2)
    Type 2 Excludes
    • lung diseases due to external agents (J60-J70)
    Other interstitial pulmonary diseases
  • J84.8
    ICD-10-CM Diagnosis Code J84.8

    Other specified interstitial pulmonary diseases

      2016 2017 2018 2019 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • exogenous lipoid pneumonia (J69.1)
    • unspecified lipoid pneumonia (J69.1)
    Other specified interstitial pulmonary diseases
Approximate Synonyms
  • Lymphangiomyomatosis
  • Pulmonary lymphangioleiomyomatosis
Clinical Information
  • A disease characterized by the progressive invasion of smooth muscle cells into the lymphatic vessels, and the blood vessels. The majority of the cases occur in the lungs of women of child-bearing age, eventually blocking the flow of air, blood, and lymph. The common symptom is shortness of breath (dyspnea).
  • A multifocal neoplasm with perivascular epithelioid cell differentiation affecting almost exclusively females of child-bearing age. It is characterized by the presence of smooth muscle and epithelioid cells and by the proliferation of lymphatic vessels. Sites of involvement include the lungs, mediastinum, and the retroperitoneum. It usually presents with chylous pleural effusion or ascites.
  • Rare lung disorder of unknown etiology mostly in women of childbearing age; proliferation of atypical smooth muscle cells in lungs, airways, blood and lymph vessels causing obstruction; symptoms include dyspnea and pneumothorax.
ICD-10-CM J84.81 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 196 Interstitial lung disease with mcc
  • 197 Interstitial lung disease with cc
  • 198 Interstitial lung disease without cc/mcc

Convert J84.81 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
Code annotations containing back-references to J84.81:
  • Code First: J93.12
    ICD-10-CM Diagnosis Code J93.12

    Secondary spontaneous pneumothorax

      2016 2017 2018 2019 Billable/Specific Code
    Code First
    • underlying condition, such as:
    • catamenial pneumothorax due to endometriosis (N80.8)
    • cystic fibrosis (E84.-)
    • eosinophilic pneumonia (J82)
    • lymphangioleiomyomatosis (J84.81)
    • malignant neoplasm of bronchus and lung (C34.-)
    • Marfan's syndrome (Q87.4)
    • pneumonia due to Pneumocystis carinii (B59)
    • secondary malignant neoplasm of lung (C78.0-)
    • spontaneous rupture of the esophagus (K22.3)

Diagnosis Index entries containing back-references to J84.81:
  • Lymphangioleiomyomatosis J84.81
  • Lymphangiomyomatosis J84.81

ICD-10-CM Codes Adjacent To J84.81
J84.111 …… not otherwise specified
J84.112 Idiopathic pulmonary fibrosis
J84.113 Idiopathic non-specific interstitial pneumonitis
J84.114 Acute interstitial pneumonitis
J84.115 Respiratory bronchiolitis interstitial lung disease
J84.116 Cryptogenic organizing pneumonia
J84.117 Desquamative interstitial pneumonia
J84.17 …… in diseases classified elsewhere
J84.2 Lymphoid interstitial pneumonia
J84.8 Other specified interstitial pulmonary diseases
J84.81 Lymphangioleiomyomatosis
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
J84.843 Alveolar capillary dysplasia with vein misalignment
J84.848 Other interstitial lung diseases of childhood
J84.89 Other specified interstitial pulmonary diseases
J84.9 Interstitial pulmonary disease, unspecified
J85 Abscess of lung and mediastinum

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.