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Understanding IPF—Learn From the Experts

Understanding IPF—Learn From the Experts

Idiopathic Pulmonary Fibrosis is a complex disease with an unknown cause, most often affecting people over 50. Listen to physician specialists discuss disease development and progression.  

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IPF is an unpredictable, progressive disease in which damage to the smallest functioning units of the lungs advances until the lungs are no longer capable of sustaining adequate function. Injury happens to lung alveoli where the body’s blood passes to obtain fresh oxygen and release carbon dioxide. Since the injury does not heal properly, alveolar tissue becomes fibrotic and promotes a change in lung structure.

IPF patients typically exhibit cough, labored breathing, poor appetite and weight loss. More severe symptoms include finger clubbing and bluish toes and fingernails. Initially, clinicians may hear fine crackles at the lung bases during inspiration—a hallmark of IPF. As the disease worsens, right heart failure, pulmonary hypertension and edema can occur, along with honeycomb cysts or scars found adjacent to lung tissue that looks completely healthy but is likely also damaged. Because several other diseases have symptoms similar to IPF, definitive diagnosis is needed, requiring histological samples, high-resolution CT (HRCT) scans and lung biopsies. HRCT scans are especially useful since they show areas of lung opacity often seen in IPF.

Though all types of IPF are progressive, rate is very difficult to predict. Common progressions are slow or step-wise. Moreover, other diseases or illnesses can cause exacerbations of IPF. Regardless of the progression or comorbidities present, however, follow-up is advised every 4-6 months.

It is important to have a definitive diagnosis of IPF before choosing a treatment course, since other similar interstitial lung diseases involve treatments inadvisable for IPF. Furthermore, a definitive diagnosis will give the patient a realistic timeline of what to expect for better planning.

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