Usual interstitial pneumonia is a form of lung disease characterized by progressive scarring of both lungs. The scarring (fibrosis) involves the supporting framework (interstitium) of the lung. UIP is thus classified as a form of interstitial lung disease. The term "usual" refers to the fact that UIP is the most common form of interstitial fibrosis.
The cause of the scarring in UIP may be known or unknown. The latter situation is more common. Since the medical term for conditions of unknown cause is "idiopathic", the clinical term for UIP of unknown cause is idiopathic pulmonary fibrosis (IPF). Examples of known causes of UIP include systemic sclerosis/scleroderma, rheumatoid arthritis, asbestosis and chronic nitrofurantoin toxicity.
The typical symptoms of UIP are progressive shortness of breath and cough for a period of months. In some patients, UIP is diagnosed only when a more acute disease supervenes and brings the patient to medical attention.
Regardless of etiology, UIP is relentlessly progressive, usually leading to respiratory failure and death. Some patients do well for a prolonged period of time, but then deteriorate rapidly because of a superimposed acute illness (so-called "accelerated UIP"). The outlook for long-term survival is poor. In most studies, the median survival is 3 to 4 years. Patients with UIP in the setting of rheumatoid arthritis have a slightly better prognosis than UIP without a known cause (IPF).
I am posting this because I have a 42-year old friend, mother of 2 DDs, who approx 6 months was healty, vibrant, working and playing. Around that time she had a cough and was just not feeling up to par. She was diagnosed with "bronchitis" (first diagnosis), which developed into pneumonia (2nd diagnosis) and today it is UIP. When she was first diagnosed (bronchitis), she was given the usual treatment of antibiotics. At 2nd diagnosis (pnuemonia, maybe 4-6 weeks later), she was so gravely ill that she was put into a medically induced coma for almost 2 months to allow her body and lungs to try to heal. Which to some extent they did, as she did get better. She was released from the hosp right before Xmas, on 24/7 oxygen and respiratory therapy. She seemed OK for about a month, but never really seemed to gain any strength back. And she coughed constantly. In mid-Jan, things started to go south again and lung biopsies were done. Last weekend, she was put back into the hospital, and back into the coma, as her lungs are no longer capable of functioning. Two days ago, she was diagnosed w/ UPI. The doctors say that her lungs are akin to brittle tissue paper. And her only hope is a lung transplant. However, she will need to be driven 350 miles to the nearest transplant-capable hospital and "put on the transplant list". To wait. But at this time, she cannot even be shifted in bed without a lung collapsing or tearing. IF she is ever able to get a lung transplant, the doctors told her it might extend her life 2-4 years.
There is no cure for this disease.
I had never heard of this disease until 2 days ago. And I am mortified by the rapid onslaught of this disease and how it seems to be destroying my young friend. In reading about this, it appears there can be many causes, as described above. And if I am understanding the "causes", one can also "have" this disease, undetected for many years, and then another acute illness (perhaps such as bronchitis or pneumonia) can cause the disease to accelerate. OR it can be caused by pulmonary toxicity due to the use of the antibiotic Nitrofurantoin (used to treat UTI - http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0000640/).
Has anyone else ever heard of this, or known anyone who had such?