2026 ICD-10-CM Diagnosis Code J84.01Alveolar proteinosis

ICD-10-CM Codes›J00–J99›J80-J84›J84

ICD-10-CM J84.01
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

J84.01 is a billable ICD-10-CM diagnosis code for alveolar proteinosis. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 196 through 198. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified lower respiratory disease.

Code Identity

ICD-10-CM Code
J84.01
Billable Status
Yes — Valid for Submission
Code Describes
Alveolar proteinosis
Short Description
Alveolar proteinosis
Same as the full description in the CMS dataset.
Parent Code
J84.0
Alveolar and parieto-alveolar conditions

Code Classification

ChapterJ00–J99Diseases of the respiratory system
SectionJ80-J84Other respiratory diseases principally affecting the interstitium
CategoryJ84Other interstitial pulmonary diseases
This CodeJ84.01Alveolar proteinosis

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Autoimmune pulmonary alveolar proteinosis
  • Congenital pulmonary alveolar proteinosis
  • Hypogammaglobulinemia
  • Infantile-onset pulmonary alveolar proteinosis, hypogammaglobulinemia
  • Proteinosis
  • Pulmonary alveolar proteinosis
  • Secondary pulmonary alveolar proteinosis
  • Severe early-onset pulmonary alveolar proteinosis due to MARS deficiency

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Proteinosis
      • alveolar (pulmonary)

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR RSP016
Other specified and unspecified lower respiratory disease
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • 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 Alveolar Proteinosis

    a rare lung disorder characterized by the filling of the pulmonary alveoli with proteinaceous material which stains positive with periodic acid-schiff stain. it may be idiopathic or secondary due to hematologic malignancies or the inhalation of mineral dusts. signs and symptoms include dyspnea, cough and low grade fever.

  • Autoimmune Pulmonary Alveolar Proteinosis

    a type of pulmonary alveolar proteinosis associated with low concentrations of gm-csf.

Patient EducationClinical

Interstitial Lung Diseases

Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. The inflammation and scarring make it hard to get enough oxygen. The scarring is called pulmonary fibrosis.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert J84.01 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
516.0 Pul alveolar proteinosis
Exact Match The mapping is direct, with no qualifiers.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About J84.01Overview

Is J84.01 (Alveolar and parieto-alveolar conditions) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report alveolar proteinosis on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does J84.01 group to?

When alveolar proteinosis is the principal diagnosis on an inpatient stay, it groups to MS-DRG 196, 197, 198, with relative weights from 0.7162 to 1.8872 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of J84.01?

Under the General Equivalence Mappings, alveolar proteinosis converts to ICD-9-CM 516.0 (pul alveolar proteinosis). The mapping is a direct match.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.