What are the symptoms?
The most common symptoms in UIP, as in all forms of IPF, include excessive shortness of breath when trying to exercise and a dry, hacking cough. Some people may also notice flu-like feelings such as fatigue, mild fever, weight loss and muscle and joint aches. However, fever is generally uncommon in UIP, occurring in only 15% of patients. Symptoms usually develop gradually over months or years, and this chronic nature is what distinguishes IPF in general from diseases with similar symptoms such as the common cold or flu.
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Average duration
of symptoms before diagnosis
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Schwartz. Am J Resp Crit Care Med 1994; 149:450 |
On examination by a physician, fine "crackles" like the sound of Velcro may be heard in the lungs upon inspiration. These crackles are usually heard in the lower lobes of both lungs. In addition, about half of patients may have "clubbing" of the fingertips. Clubbing results from the loss of the normal 15º angle formed by the fingernail with the cuticle; enlargement or bulging of the fingertip; and a downward curvature of the fingernails. This usually indicates more advanced disease when present. None of these symptoms or signs is specific for UIP or even IPF in general. That is, they may occur in many other lung diseases as well. In addition, many patients may have shortness of breath but completely normal examinations. Therefore, if your doctor suspects that IPF is the cause of your symptoms and signs, a number of tests must be done to confirm the diagnosis.
In those with NSIP, the most common symptoms are again shortness of breath and dry cough. Fever has also been found in about 20% of patients. Like UIP, the symptoms develop gradually over months. However, the average duration of symptoms before diagnosis is shorter than UIP at 8 months.
RBILD has more mild symptoms, but it also presents primarily with shortness of breath and dry cough. Clubbing has specifically been noted to be absent in all patients with RBILD. DIP, which is thought to simply represent a more severe form of RBILD, results in the same symptoms of shortness of breath and dry cough developing gradually over months. However, clubbing has been noted in about half of patients with DIP.
AIP is significantly different from the other forms of IPF in that symptoms develop rapidly over just a few weeks. Often there is a period of flu-like symptoms followed by rapidly progressive and severe shortness of breath. Nearly half of patients have a fever.
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References