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Pulmonology Coding Alert

ICD-10 Coding:
Pinpoint Diagnoses for These 3 Rare Lung Disorders

From LAM to PAP, learn the acronyms and the codes.

You may know the asthma and COPD diagnosis codes by heart, but that doesn't mean you're just as much of an ace with diagnoses that your pulmonologist sees less frequently. Get a handle on how to code these three rare lung disorders with a few quick tips.

1. Look for Confirmation When Coding LAM

Pulmonologists are increasingly seeing patients for LAM – an acronym for lymphangioleiomyomatosis, a condition that occurs mainly in women of child-bearing age and can lead to lung destruction and other serious symptoms. In most cases, patients don't know they have LAM until they experience pneumothorax, or a collapsed lung. When patients present with LAM symptoms but the pulmonologist hasn't yet diagnosed the condition, you should select the appropriate code from the documented symptoms, which may include the following, among others:

  • R05 - Cough
  • R07.89 - Other chest pain
  • J93.9 - Pneumothorax, unspecified
  • R53.83 - Other fatigue
  • R06.02 - Shortness of breath

If, however, the pulmonologist definitively diagnoses the patient with LAM, you'll instead bill the claim using J84.81 (Lymphangioleiomyomatosis), which is the sole diagnosis code in ICD-10 that describes the condition.

2. Goodpasture Syndrome Falls Under Rheumatology Codes

Pulmonologists are frequently called to diagnose and evaluate Goodpasture Syndrome, an autoimmune disorder that causes bleeding in the lungs and kidney failure, and is sometimes fatal if the lungs don't recover from the disease's acute phase.

If the pulmonologist is involved before the Goodpasture Syndrome diagnosis is made, you'll code the symptoms he treats, which can include R04.89 (Hemorrhage from other sites in respiratory passages), R07.89 (Other chest pain), R05 (Cough), R04.2 (Hemoptysis), and N18.9 (Chronic kidney disease, unspecified).

Once the patient is formally diagnosed with Goodpasture Syndrome, you'll report M31.0 (Hypersensitvitiy angiitis [Goodpasture's syndrome]), which is located in ICD10's "Diseases of the Musculoskeletal System and Connective Tissue" section.

3. See J84 Section for Alveolar Proteinosis

Patients with pulmonary alveolar proteinosis, often notated by physicians as "PAP," find it difficult to take oxygen from the air and exchange carbon dioxide from the blood due to a buildup of proteins and lipids in the patients' air sacs.

When patients present with this condition, they'll often complain of shortness of breath (R06.02), dyspnea (R06.00), or a cough (R05). You should code these symptoms if the physician hasn't yet diagnosed the patient with PAP.

Although your pulmonologist is likely to document whether the patient has primary or secondary alveolar proteinosis, ICD-10 includes just one code to describe this condition once the physician makes a diagnosis. Therefore, you'll report J84.01 (Alveolar proteinosis) for both primary or secondary presentations. However, in cases of secondary alveolar proteinosis, you'll also report the cause, such as a lung infection or inhalations of dust such as aluminum using the appropriate ICD-10 codes.