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Why Does Leg Swelling Develop in Patients with Idiopathic Pulmonary Fibrosis?
Early in IPF, common symptoms are cough and shortness of breath with vigorous exercise or walking up inclines. As IPF progresses, there is progressive loss of lung tissue including blood vessels in the lungs. Over time, this leads to increased work for the right side of your heart.
In response to having to work harder, the right side of the heart releases hormones that promote fluid retention. These hormones act on the kidneys to decrease the amount of salt and fluid that is removed from your body. Most people have similar swelling in both legs but some patients may have one leg that is a bit more swollen than the other. If there is a marked difference in the swelling in one leg compared to the other, we worry about a blood clot and may investigate further with an ultrasound exam of the more swollen leg.
Do I Need a Lung Biopsy to Diagnose IPF?
This is a very important question for patients with chronic fibrotic lung disease. I have written about this in detail (see section on role of lung biopsy). I will summarize a larger post on this topic. IPF may be diagnosed with confidence if a patient has the right demographics (age > 50, absence of connective tissue disease) and a high resolution CT scan of the lungs that has the characteristic findings.
However, many patients have CT scans of their lungs that are not typical. These patients should consider lung biopsy, especially if they have early disease. Patients with advanced scarring or who are very ill with respiratory failure may not be able to undergo lung biopsy due to risks of the procedure. For less ill patients, video assisted lung biopsy is a low risk procedure.
If I Don’t Tolerate one IPF Medicine, Does this Mean that I Won’t Tolerate the Other?
It is not uncommon for a patient to have side effects with either Esbriet (Pirefenidone) or OFEV (Nintedanib). The good news is that the two drugs are completely unrelated and intolerance to one does not predict intolerance to the other. For example, if a patient has severe diarrhea with Esbriet, this does not predict that they will have severe diarrhea with OFEV and vice versa.
Pets and Pulmonary Fibrosis
A reader asked if a dog allergy can lead to worsening of IPF.
There is no data that suggests that allergies to dogs can lead to pulmonary fibrosis or exacerbate IPF. Enjoy your canine companions!