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idiopathic pulmonary fibrosis
ICD-10 Codes
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Description
Idiopathic Pulmonary Fibrosis (IPF) Description
Idiopathic pulmonary fibrosis, also known as IPF, is a serious and progressive lung disease that causes scarring and thickening of the tissue around and between the air sacs called alveoli in the lungs. This condition leads to breathing difficulties and can be life-threatening if left untreated.
Key Characteristics:
- Scarring and Thickening: The lungs become scarred and thickened, making it harder for oxygen to reach the bloodstream.
- Progressive Disease: IPF is a progressive disease, meaning that fibrosis builds up over time, gradually causing an increase in breathlessness.
- Unknown Cause: The exact cause of IPF is unknown, but it's believed to be related to genetic and environmental factors.
Symptoms:
- Breathing Difficulties: Shortness of breath, especially when performing physical activities or lying down.
- Coughing: A persistent cough that may produce mucus.
- Fatigue: Feeling tired or weak due to the body's reduced ability to take in oxygen.
References:
- [1] Pulmonary fibrosis is scarring and thickening of the tissue around and between the air sacs called alveoli in the lungs. These changes make it harder for oxygen to reach the bloodstream.
- [2] Idiopathic pulmonary fibrosis (IPF) is a condition in which the lungs become scarred and breathing becomes increasingly difficult.
- [3] Idiopathic pulmonary fibrosis (IPF) is the most common type of pulmonary fibrosis. It is a disease that causes scarring (fibrosis) of the lungs.
- [4] Idiopathic pulmonary fibrosis is a progressive disease, which means that fibrosis builds up over time, gradually causing an increase in breathlessness and the difficulty breathing.
- [6] Jul 25, 2024 — Idiopathic pulmonary fibrosis (IPF) is where the lungs become scarred and breathing becomes more difficult. It's not clear what causes it.
- [9] Jul 1, 2020 — Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. This condition causes scar tissue (fibrosis) to build up in the lungs.
Additional Characteristics
- fatigue
- coughing
- breathing difficulties
- scarring and thickening of the tissue around and between the air sacs called alveoli in the lungs
- shortness of breath, especially when performing physical activities or lying down
- persistent cough that may produce mucus
- feeling tired or weak due to the body's reduced ability to take in oxygen
Signs and Symptoms
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease characterized by scarring and inflammation in the lungs. The signs and symptoms of IPF can vary from person to person, but here are some common ones:
Common symptoms:
- Shortness of breath (dyspnea), including shortness of breath with everyday activities [1]
- A persistent dry cough [2][3]
- Tiredness (fatigue) [4][5]
- Unexplained weight loss [4][6]
- Clubbing of fingers and toes (swollen and rounded finger tips and toe tips) [7]
Other symptoms:
- Aching muscles and joints [8]
- Gradual, unintended weight loss [9]
- Feeling out of breath more than usual with everyday activities, like walking upstairs [10][11]
- A persistent cough every day and lasting for more than 8 weeks [10][12]
It's essential to note that some people may not experience any symptoms at first, but as the disease progresses, these symptoms can develop and worsen over time. If you're experiencing any of these symptoms, it's crucial to consult a doctor for proper diagnosis and treatment.
References:
[1] - Search result 11 [2] - Search result 3 [3] - Search result 7 [4] - Search result 5 [5] - Search result 9 [6] - Search result 14 [7] - Search result 8 [8] - Search result 4 [9] - Search result 13 [10] - Search result 11 [11] - Search result 12 [12] - Search result 5
Additional Symptoms
- Aching muscles and joints
- A persistent dry cough
- Tiredness (fatigue)
- Feeling out of breath more than usual with everyday activities
- A persistent cough every day and lasting for more than 8 weeks
- weight loss
- dyspnea
- clubbing of fingers
Diagnostic Tests
Idiopathic pulmonary fibrosis (IPF) is a chronic and ultimately fatal disease characterized by a progressive decline in lung function. Diagnostic tests for IPF are crucial to confirm the diagnosis and rule out other conditions that may cause similar symptoms.
Common diagnostic tests for IPF:
- High-resolution chest CT scan (HRCT): This imaging test can help diagnose IPF by showing scarring or fibrosis in the lungs [1]. HRCT is considered a sensitive and specific tool for diagnosing IPF, especially when compared to other imaging modalities like chest X-ray [5].
- Blood tests: Blood tests may be ordered to rule out other conditions that may cause similar symptoms. These tests can include antinuclear antibody (ANA), rheumatoid factor (RF), erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) [8, 9].
- Spirometry: Spirometry is a lung function test that measures the amount of air breathed in and out. It can help diagnose IPF by showing a decline in lung function over time [3].
- Chest X-ray and CT scan: These imaging tests can help identify scarring or fibrosis in the lungs, which is characteristic of IPF [4].
Other diagnostic tests:
- Pulse oximetry: This test measures the oxygen levels in the blood. It may be used to monitor the severity of IPF.
- Lung biopsy: In some cases, a lung biopsy may be performed to confirm the diagnosis of IPF.
It's essential to note that no single diagnostic test can definitively diagnose IPF. A combination of clinical evaluation, imaging tests, and blood tests is often necessary to establish a diagnosis.
References:
[1] Mar 24, 2022 — Diagnostic tests and procedures · High resolution chest CT scan, or HRCT: This is used to take pictures of the inside of your lungs and look for ...
[3] A commonly used lung function test is spirometry. During the test, you breathe into a mouthpiece that's attached to a monitor.
[4] Chest X-ray and CT scan. A chest ...
[5] Sep 25, 2024 — High-resolution computed tomography (HRCT) findings are significantly more sensitive and specific for the diagnosis of idiopathic pulmonary ...
[8] Sep 12, 2018 — Typical preliminary laboratory tests are antinuclear antibody, rheumatoid factor, erythrocyte sedimentation rate and C-reactive protein. Others ...
[9] The majority of panelists acknowledged routinely testing for CRP (C-reactive protein), erythrocyte sedimentation rate, antinuclear antibodies (by ...
Additional Diagnostic Tests
- Blood tests
- Spirometry
- Lung biopsy
- High-resolution chest CT scan (HRCT)
- Chest X-ray and CT scan
- Pulse oximetry
Treatment
Idiopathic pulmonary fibrosis (IPF) is a chronic and often fatal condition characterized by progressive scarring of the lungs. While there is no cure for IPF, various drug treatments have been approved to slow down disease progression and improve quality of life.
Approved Medications:
- Pirfenidone: An oral medication that has been shown to slow down lung function decline in patients with IPF [6]. It works by reducing inflammation and preventing further scarring.
- Nintedanib: Another oral medication approved for the treatment of IPF, which also slows down disease progression by inhibiting fibrosis [2].
- Acetylcysteine: A therapy
Recommended Medications
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Idiopathic Pulmonary Fibrosis (IPF) is a type of interstitial lung disease characterized by progressive and irreversible scarring of the lungs. The differential diagnosis of IPF involves identifying other conditions that may present with similar symptoms and radiographic findings.
Key Conditions to Consider
- Asthma: While asthma can cause chronic cough and shortness of breath, it is typically reversible with treatment and does not lead to fibrosis [4].
- Bronchiectasis: This condition involves permanent enlargement of the airways, which can mimic IPF symptoms but is often associated with recurrent infections and excessive sputum production [4].
- Chronic Obstructive Pulmonary Disease (COPD): COPD can cause chronic cough and shortness of breath, but it typically presents with a combination of airflow limitation and fibrosis [4].
- Sarcoidosis: This is an inflammatory disease that can affect the lungs and other organs, leading to symptoms similar to IPF. However, sarcoidosis often responds to corticosteroid treatment [4].
- Differential Interstitial Lung Diseases (ILDs): Other forms of ILDs, such as lymphangioleiomyomatosis or pneumoconiosis, can present with similar radiographic findings and require differential diagnosis [6].
Imaging and Histological Considerations
- Ground-glass opacities: These are characteristic of DIP (Desquamative Interstitial Pneumonia) but not typically seen in IPF. Imaging studies can help differentiate between these conditions [5].
- Reticular features: The presence or absence of reticular features on imaging studies can also aid in differential diagnosis, with certain patterns more suggestive of IPF than others [5].
Importance of Accurate Diagnosis
Accurate diagnosis is crucial for determining the best course of treatment and prognosis. Management strategies and prognoses differ among forms of fibrotic interstitial lung diseases (ILDs), making precise diagnosis essential [9].
Additional Information
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