Interstitial Lung Disease ICD-10-CM Codes

This guide provides an overview of ICD-10-CM codes commonly used for diagnosing and documenting interstitial lung diseases in 2025.

By Olivia Sayson on Apr 28, 2025.

Fact Checked by Gale Alagos.

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Interstitial Lung Disease ICD-10-CM Codes

What ICD-10 codes are used for interstitial lung disease?

Interstitial lung disease (ILD) refers to a broad category of lung disorders that affect the interstitial tissues of the lungs, which are the spaces around the air sacs. Accurate coding for interstitial lung disease is crucial for diagnosis, treatment planning, and medical billing. The ICD-10-CM system offers specific codes to help healthcare providers capture the nuances of this complex condition.

Here are some commonly used ICD-10-CM codes for interstitial lung diseases:

  • J84.1: Other interstitial pulmonary diseases with fibrosis: This code covers other specified forms of interstitial pulmonary diseases that lead to fibrosis.
  • J84.112: Idiopathic pulmonary fibrosis: This code explicitly addresses idiopathic pulmonary fibrosis, a form of ILD with an unknown cause.
  • J84.9: Interstitial lung disease, unspecified: This code is used for general cases of interstitial lung disease where the specific form is not indicated.
  • J70.9: Respiratory conditions due to unspecified external agents: This code is used for cases of interstitial lung disease believed to be caused by exposure to unspecified external agents like chemicals or dust.
  • J99: Respiratory disorders in diseases classified elsewhere: This code is for respiratory disorders, including ILD, due to diseases classified in other categories.

Which interstitial lung disease ICD codes are billable?

  • J84.112: This code is billable.
  • J84.9: This code is billable.
  • J70.9: This code is billable.
  • J99: This code is billable.

Clinical information

  • Interstitial lung diseases (ILDs) often present with symptoms like cough, shortness of breath, and fatigue.
  • Conditions such as lymphoid interstitial pneumonia and other interstitial lung diseases are part of the broad ILD spectrum.
  • Interstitial emphysema may occur in both adults. The history of lung diseases of childhood can also contribute, affecting breathing efficiency.
  • ILDs can develop secondary to systemic connective tissue disorders, including rheumatoid arthritis, leading to rheumatoid lung disease.
  • Some cases involve specified interstitial pulmonary diseases, while others remain classified under interstitial pulmonary disease, unspecified if no clear cause is found.
  • In many patients, ILDs progress into a progressive fibrotic phenotype, resulting in worsening lung function and potential respiratory failure.
  • Exposure to certain medications or substances can cause drug-induced interstitial lung conditions or induced interstitial lung disorders.
  • Diagnostic tests may include chest X-rays, CT scans, pulmonary function tests, and lung biopsies to evaluate disease extent and progression.
  • Treatment often includes anti-inflammatory drugs, immunosuppressants, and antifibrotic agents to slow disease progression.
  • Supportive care, such as oxygen therapy and pulmonary rehabilitation, is vital to maintain respiratory function and quality of life.

Synonyms include

  • Diffuse Parenchymal Lung Disease
  • Interstitial Pneumonitis
  • Fibrosing Alveolitis
  • Pulmonary Fibrosis
  • Chronic Interstitial Lung Disease

Frequently asked questions

What causes interstitial lung diseases aside from genetic factors?

Interstitial lung diseases can develop due to external agents such as environmental toxins, occupational exposures, or medications. Due to external factors, these diseases often require different management compared to inherited conditions.

How is lung involvement assessed in interstitial lung diseases?

Lung involvement is typically evaluated using imaging studies like CT scans and pulmonary function tests. In some cases, vein abnormalities, such as vein misalignment, can also be observed and contribute to breathing difficulties.

Can interstitial lung diseases occur alongside other conditions?

Yes, interstitial lung diseases may occur as part of an underlying disease, especially autoimmune disorders. Identifying the primary underlying disease is crucial for selecting the most effective treatment strategy.

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