IDIOPATHIC PULMONARY FIBROSIS
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ALTERNATE NAMES
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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)
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: The diagnosis of IPF is made and supported by:
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Chest x-ray and HRCT scan;
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Spirometry or bronchoscopy to determine the extent of lung damage and scarring;
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Arterial blood gas tests to measure the oxygen and carbon dioxide levels in the bloodstream;
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TB test to rule out tuberculosis; and
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Surgical lung biopsy to confirm the diagnosis, determine the progression of the disease,
and rule out other causes of the condition, such as sarcoidosis, cancer or infection.
Physical findings: Symptoms of IPF include 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.
ICD-9: 516.3
ICD-10: J84.112
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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.
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TREATMENT
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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 cannot be
reversed, but with treatment, more damage and scarring may be prevented and may improve
the quality of life for these individuals.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Pulmonary Function Tests (PFTs) including diffusing capacity (DLCO), Spirometry, and
ABGs (Arterial Blood Gas Studies); and
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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3.02
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A description of findings establishing the diagnosis and response to treatment is
needed when evaluating this condition. A DLCO test is particularly important as gas
exchange is commonly impaired to a greater degree than ventilatory function.
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3.09
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3.11
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3.14
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Equals
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3.02
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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.
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3.09
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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