IDIOPATHIC PULMONARY FIBROSIS

ALTERNATE NAMES

Idiopathic Diffuse Interstitial Pulmonary Fibrosis; IPF; Pulmonary Fibrosis; Cryptogenic Fibrosing Alveolitis; CFA; Fibrosing Alveolitis; Usual Interstitial Pneumonitis; UIP; Diffuse Fibrosing Alveolitis; Familial Idiopathic Pulmonary Fibrosis (FIPF).

DESCRIPTION

Idiopathic Pulmonary Fibrosis (IPF) is a condition in which tissue deep in the lungs becomes thick and stiff, or scarred, over time. The development of the scarred tissue is called fibrosis. As the lung tissue becomes thickened the lungs lose their ability to transfer oxygen into the bloodstream. As a result, the brain and other organs do not get the oxygen they need to function properly. The condition is ‘idiopathic’ because there is no known cause for the disease but exposure to irritants, such as cigarette smoking, viral infections, exposure to environmental pollutants, certain side affects from antibiotics, heart and cancer medicines are suspected causes. Some people may be more likely to develop IPF because of their genes. This condition is called Familial Idiopathic Pulmonary Fibrosis (FIPF). The most common signs and symptoms are shortness of breath, a dry, hacking cough that does not get better, weight loss, fatigue or malaise, aching muscles and joints and clubbing of fingers or toes. IPF may lead to other medical conditions including collapsed lung, lung infections, blood clots in the lungs and lung cancer. The disease occurs most often in people over 50 years old. The prevalence is estimated to be in the order of 20 per 100,000 but is thought to be significantly higher in those aged 75 and older.

DIAGNOSTIC

TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Physical Findings: Physical exam with documentation of abnormal breath sounds (cackles) and in advanced disease, blue-colored skin (cyanosis) around the mouth or in the fingernails (due to low oxygen) and clubbing of the fingers and toes.

Diagnostic Testing: chest x-ray and HRCT scan, spirometry or bronchoscopy to determine the extent of lung damage and scarring. Pulse oximetry, arterial blood gas tests and EKG to measure the oxygen and carbon dioxide levels in the bloodstream. TB test to rule out tuberculosis. Surgical lung biopsy to confirm the diagnosis, determine the progression of the disease and to rule out other causes of the condition, such as sarcoidosis, cancer or infection.

ICD-9: 516.3

ONSET AND PROGRESSION

As IPF progresses it worsens and potentially life-threatening conditions develop such as respiratory failure, pulmonary hypertension and heart failure. Individuals with this disease usually die within 3 to 5 years of diagnosis.

TREATMENT

IPF is treated with anti-inflammatory medicines such as: Prednisone and immune system suppressants. Flu and pneumonia vaccines, cough medicines or oral codeine, Vitamin D, calcium and anti-reflux therapy to control gastroesophageal reflux disease (GERD) which is sometimes present, are also prescribed. Oxygen therapy, pulmonary rehabilitation and lung transplant are also used to manage the disease. The lung damage can not be reversed, but with treatment, more damage and scarring can be prevented and may improve the quality of life for these individuals.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation: Clinical evaluation with a description of symptoms, physical signs, biopsy reports, PFTs (Pulmonary Function Tests) including diffusing capacity (DLCO), spirometry and ABGs (Arterial Blood Gas Studies), and response, if any, to a regimen of treatment. Pulmonary hypertension may be apparent clinically and confirmed by echocardiography and/or right heart catheterization.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

3.02 A, B, or C,

3.09, 3.11

A description of findings establishing the diagnosis and response to treatment is needed when evaluating this condition. To meet listings 3.02 A, B or C, program compliant PFTs including diffusing capacity (DLCO), spirometry and/or ABGs documenting listing level severity would be needed. DLCO is particularly important as gas exchange is commonly impaired to a greater degree than ventilatory function.

Medical Equals

3.02 A, B, or C,

3.09

Review of the claim by a physician experienced in the management of patients with IPF would generally be required to determine if there was sufficient evidence to establish severity equal to the intent of the listing.