Interstitial pulmonary fibrosis (IPF)
Interstitial pulmonary fibrosis (IPF) is a chronic and progressive lung disease characterized by scarring of the lung tissue, specifically the interstitium, which lies between the air sacs. This scarring can lead to significant respiratory issues, primarily manifesting as shortness of breath, chest pain, and a persistent dry cough. Various factors contribute to the development of IPF, including occupational exposure to harmful substances like silica or asbestos, certain medications, radiation, and underlying diseases such as sarcoidosis, lupus, and rheumatoid arthritis.
Symptoms typically begin subtly and worsen over time, affecting daily activities and potentially leading to severe complications such as respiratory or heart failure. Diagnosis involves a combination of imaging studies, lung function tests, and sometimes tissue examination. Treatment options focus on alleviating symptoms and slowing disease progression, with corticosteroids and other medications commonly prescribed. In advanced cases, lung transplantation may be considered. The disease predominantly affects individuals between the ages of 40 and 60 and is known for its challenging prognosis.
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Interstitial pulmonary fibrosis (IPF)
ANATOMY OR SYSTEM AFFECTED: Heart, lungs
DEFINITION: An inflammatory disease that results in the scarring and fibrosis of the lung alveolar tissue (air sacs)
CAUSES: Various; may include occupational exposure to silica or asbestos, drugs, radiation, diseases (sarcoidosis, lupus erythematosus, rheumatoid arthritis)
SYMPTOMS: Scarring of lung tissue, shortness of breath, chest pain, dry cough, clubbing of fingers, blue extremities; death may come from respiratory failure, heart failure, blood clot, stroke, heart attack
DURATION: Progressive, eventually fatal
TREATMENTS: Drugs (prednisone, cyclophosphamide), oxygen therapy, physical therapy and exercise, lung transplantation
Causes and Symptoms
Interstitial (IPF) is a chronic lung disease that causes scarring of the tissue between the air sacs (interstitium). It may be the result of a variety of causes, such as occupational exposure to silica or asbestos, drugs, radiation, and diseases such as or diseases (systemic erythematosus, rheumatoid arthritis). When no known cause can be ascertained, the disease is known as pulmonary fibrosis. It affects both sexes equally, with the highest incidence between the ages of forty and sixty.
The disease is characterized by an initial of the attributable to a known or unknown injury-causing agent, followed by healing by scarring and fibrosis of the interstitium. This results in a decreased transfer of oxygen to the blood, causing symptoms of increasing shortness of breath (dyspnea) and chest pain. The symptoms of the disease appear insidiously, with the patient noticing a dry cough and increasing shortness of breath, initially on exertion and eventually at rest. As the disease progresses, the patient is unable to perform daily activities and may require long-term oxygen therapy. Clubbing of the fingers and blueness of the extremities can be observed in these patients. Death is usually the result of respiratory failure, right-sided heart failure, a blood (embolism) in the lungs, stroke, or heart attack.
Diagnosis of IPF is made by correlating a chest x-ray, a computed tomography (CT) chest scan, lung function tests, bronchoscopy, and a measurement of blood oxygen content. Lung and microscopic examination of the tissue is the only confirmatory test that would also show the extent of damage and help determine the of the disease.
Treatment and Therapy
Treatment must be initiated as soon as the diagnosis is made. Because of the chronic nature of the disease, most patients require lifelong treatment. Treatment modalities differ with age and the stage of the disease, but all aim at reducing the inflammation and stopping the fibrosis. Drugs are the mainstay of treatment, with prednisone (a corticosteroid) and cyclophosphamide being the most commonly used ones. Oxygen therapy improves the blood oxygen level in patients with severe breathlessness, and physical therapy and exercise also help in improving muscle strength and breathing. Patients with severe fibrosis may require lung transplantation.
Bibliography
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