Fibrosis means scarring, thus pulmonary fibrosis means scarring in the lungs. Pulmonary fibrosis can be caused by many things. Including: infections, environmental agents like asbestos and silica, chronic inflammatory processes such as sarcoidosis, and Wegener’s granulomatosis, lupus, rheumatoid arthritis, exposure to radiation, and certain kinds of medications.
Pulmonary fibrosis is recognized by a history of shortness of breath with physical action. During an assessment of the lungs with a stethoscope, the doctor can hear sounds like crackling in the chest. The chest x-ray may or may not be atypical, but an x-ray test called a high resolution CAT scan will often demonstrate abnormalities. Lung function testing is markedly abnormal.
The diagnosis can be established by a lung biopsy. An open surgical biopsy, which means that the chest cavity must be cut open under general anesthesia to eliminate a portion of lung tissue, may be needed to obtain enough tissue to make an precise diagnosis. The removed tissue is examined microscopically by a pathologist to confirm the presence of fibrosis.
The treatment choices for idiopathic pulmonary fibrosis are few. There is no proof that any medications can help this disorder, since scarring is permanent once it has started. Lung transplantation is the only curative option available. At times, this identification can be difficult to make even with tissue biopsies examined by pathologists with specialized knowledge in this field. Research experiments using diverse drugs that may decrease fibrous scarring are long-term. Since selected types of lung fibrosis can react to corticosteroids (such as Prednisone) and/or other medications that hold back the body's immune system, this category of drugs are sometimes given in an attempt to reduce the progression that leads to fibrosis.
The immune system is thought to play a vital role in the progression of many forms of pulmonary fibrosis. The aim of treatment with immune suppressive agents such as corticosteroids is to decrease lung inflammation and resulting scarring. Reactions to treatment are inconsistent. Once scarring has begun, it is permanent. Those whose situations improve with immune suppressive treatment most likely do not have idiopathic pulmonary fibrosis.
The toxicity and side effects of treatments can be somber. Therefore, patients with pulmonary fibrosis should be followed by a lung specialist knowledgeable in this condition. The lung specialist will ascertain the need for treatment, the length of treatment, and will keep an eye on the reaction to therapy along with any side effects. Only a small number of patients respond to corticosteroids alone, so other immune-suppressing medications are used in accumulation to corticosteroids. These include gamma-interferon, cyclophosphamide, azathioprine, methotrexate, penicillamine, and cyclosporine. The anti-inflammatory medication colchicine has also been used with partial success. Ongoing trials are ongoing using innovative drugs such as gamma interferon, mycophenolate mofetil (Cellcept), and pirfenidone.
Pulmonary fibrosis can cause reduced oxygen levels in the blood. A decline in blood oxygen level (hypoxia) can lead to increased pressure in the pulmonary artery (the vessel that carries blood from the heart to the lungs to receive oxygen), a condition known as pulmonary hypertension, which can in turn lead to breakdown of the right ventricle of the heart. Therefore, patients with pulmonary fibrosis are commonly treated with supplemental oxygen to avert pulmonary hypertension.
There is also substantiation that patients suffering from pulmonary fibrosis may be at amplified risk for blood clots that move to the lung (pulmonary emboli), and therefore anticoagulation (blood thinning) therapy may be needed.
1 - 1 of 1 <<previous | next>>
Ipratropium inhalation is used to prevent bronchospasm, or narrowing airways in the lungs, in people with bronchitis, emphysema, or COPD (chronic obstructive pulmonary disease). This medication will not treat bronchospasm while it is happening but more of more...
1 - 1 of 1 <<previous | next>>