1. Home
  2. ICD-10-CM Codes List
  3. J00–J99
  4. J80-J84
  5. Other interstitial pulmonary diseases (J84)

Other interstitial pulmonary diseases (J84)

ICD-10 code section J84 covers a group of other interstitial pulmonary diseases, which affect the lung's interstitial tissues. These codes are used specifically to classify various rare and complex lung conditions involving inflammation, scarring (fibrosis), or abnormal buildup in the lung structures, distinct from more common pulmonary diseases.

This category includes specific conditions such as alveolar proteinosis (J84.01), characterized by accumulation of proteins in the alveoli, also known by names like pulmonary alveolar proteinosis or infantile-onset pulmonary alveolar proteinosis. Chronic fibrosing conditions, including idiopathic pulmonary fibrosis (J84.112), are also included under J84 with synonyms like chronic interstitial lung disease and idiopathic pleuroparenchymal fibroelastosis. Other notable codes classify diseases like pulmonary alveolar microlithiasis (J84.02) and lymphangioleiomyomatosis (J84.81), a rare lung disease linked to tuberous sclerosis syndrome. This section also encompasses various childhood interstitial lung diseases and other specified interstitial pneumonias, helping healthcare professionals and coders accurately capture diagnoses for uncommon interstitial lung conditions requiring specialized treatment and documentation.

Instructional Notations

Code Also

A "code also" note instructs that two codes may be required to fully describe a condition, but this note does not provide sequencing direction.

  • , if applicable, associated condition

Type 1 Excludes

A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.

  • drug-induced interstitial lung disorders J70.2 J70.4
  • interstitial emphysema J98.2

Type 2 Excludes

A type 2 excludes note represents "Not included here". An excludes2 note indicates that the condition excluded is not part of the condition represented by the code, but a patient may have both conditions at the same time. When an Excludes2 note appears under a code, it is acceptable to use both the code and the excluded code together, when appropriate.

  • lung diseases due to external agents J60 J70

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

Asbestosis

A form of pneumoconiosis caused by inhalation of asbestos fibers which elicit potent inflammatory responses in the parenchyma of the lung. The disease is characterized by interstitial fibrosis of the lung, varying from scattered sites to extensive scarring of the alveolar interstitium.

Aspergillus

A genus of mitosporic fungi containing about 100 species and eleven different teleomorphs in the family Trichocomaceae.

Cryptogenic Organizing Pneumonia

An interstitial lung disease of unknown etiology, occurring between 21-80 years of age. It is characterized by a dramatic onset of a pneumonia-like illness with cough, fever, malaise, fatigue, and weight loss. Pathological features include prominent interstitial inflammation without collagen fibrosis, diffuse fibroblastic foci, and no microscopic honeycomb change. There is excessive proliferation of granulation tissue within small airways and alveolar ducts.

Dyspnea

Difficult or labored breathing.

Hemosiderosis

Conditions in which there is a generalized increase in the iron stores of body tissues, particularly of liver and the MONONUCLEAR PHAGOCYTE SYSTEM, without demonstrable tissue damage. The name refers to the presence of stainable iron in the tissue in the form of hemosiderin.

Hemosiderosis, Pulmonary

Iron deposition within the lung. Primary pulmonary hemosiderosis is characterized by HEMOPTYSIS; IRON-DEFICIENCY ANEMIA, and diffuse pulmonary hemorrhage as seen as transient pulmonary infiltrates on RADIOGRAPHY. Even though large amounts of iron are laid down in the lung, with normal or increased total body iron, anemia occurs because of inability of the erythron to use iron sequestered in pulmonary MACROPHAGES.

Idiopathic Pulmonary Fibrosis

A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.

Invasive Pulmonary Aspergillosis

Lung infections with the invasive forms of ASPERGILLUS, usually after surgery, transplantation, prolonged NEUTROPENIA or treatment with high-doses of CORTICOSTEROIDS. Invasive pulmonary aspergillosis can progress to CHRONIC NECROTIZING PULMONARY ASPERGILLOSIS or hematogenous spread to other organs.

Lymphangioleiomyomatosis

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).

Mononuclear Phagocyte System

Mononuclear cells with pronounced phagocytic ability that are distributed extensively in lymphoid and other organs. It includes MACROPHAGES and their precursors; PHAGOCYTES; KUPFFER CELLS; HISTIOCYTES; DENDRITIC CELLS; LANGERHANS CELLS; and MICROGLIA. The term mononuclear phagocyte system has replaced the former reticuloendothelial system, which also included less active phagocytic cells such as fibroblasts and endothelial cells. (From Illustrated Dictionary of Immunology, 2d ed.)

Pulmonary Alveolar Proteinosis

A PULMONARY ALVEOLI-filling disease, characterized by dense phospholipoproteinaceous deposits in the alveoli, cough, and DYSPNEA. This disease is often related to, congenital or acquired, impaired processing of PULMONARY SURFACTANTS by alveolar macrophages, a process dependent on GRANULOCYTE-MACROPHAGE COLONY-STIMULATING FACTOR.

Pulmonary Aspergillosis

Infections of the respiratory tract with fungi of the genus ASPERGILLUS.

Pulmonary Fibrosis

A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.