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Fibrosing alveolitis is known as an idiopathic systemic fibrosing disorder, which is a chronic lung disease. It is characterized by inflammation of the alveoli caused by thickening and fibrosis along the alveoli walls. The exact cause of fibrosing alveolitis (FA) is yet to be determined, but is thought largely contributed by environmental changes and exposure to hazards, such as asbestos and other airborne agents. Another major common risk factor is in those who smoke.
Who Gets Fibrosing Alveolitis
FA is most common in individuals who are 50 and older. Men are marginally more predisposed to the disease than women. Much of this has to do with the type of occupation a man holds, such as those exposed to heavy metals, asbestos, and silica on a regular basis.
While genetics does not seem to play a major role in predisposition of FA, there have been reported cases of familial episodes. A disturbing fact to researchers is that the occurrence of the disease is rising gradually, and without a defined etiology, it is a complex disease to understand. Some speculation surrounds the fact that the general population is increasing and perhaps that the disease is becoming more widely recognized, whereas it had been contributed to other diseases in the past.
Diagnosing Fibrosing Alveolitis
The common signs and symptoms of FA are easily recognized, but have often been blamed on different diseases in the past. Doctors are now able to correctly diagnosis FA based on a patient’s presentation and physical exam more easily than before. Some of these signs and symptoms include, but are not limited to:
- Shortness of breath
- Dry cough
- Fatigue
- Weight loss
- Flu-like symptoms
- Muscle pains
- Skin rash
- Skin color changes
- Right heart failure in later stages
Patients presenting with the above signs and symptoms might also have a differential diagnosis of heart failure, chronic obstructive pulmonary disease, sarcoidosis, and pulmonary embolism. For this reason, a series of tests are performed to ensure the proper diagnosis of FA (Fibrosing Alveolitis, PatientPlus article, Patient UK.com). These tests include:
- ESR, usually elevated in patients with FA
- CPR, usually elevated in patients with FA
- ANA, elevated in 1/3 of patients with FA
- Chest x-ray, showing abnormalities in 90% of patients
- CT scan of the lungs
- Lung biopsy, definitive measure for diagnosing FA
FA is often associated with other diseases, such as lupus, thyroid disease, rheumatoid arthritis, and autoimmune liver disease. Having one of these existing conditions can greatly raise the possibility of FA when presenting with the above signs and symptoms.
Treatment Options for Fibrosing Alveolitis
Patient with FA are often treated differently, depending on the exact signs and symptoms, and preexisting medical conditions that they are experiencing. Oxygen therapy is generally a treatment of choice, as well as the encouragement of light exercise. If a patient is a smoker, cessation is highly recommended.
Steroids are the first line of drugs used in patients diagnosed with FA. This helps reduce the amount of inflammation in the lungs. Immunosuppressive drugs may help in patients who do not respond to steroidal treatments. The worst-case scenario is the need for a lung transplant when all non-drug and drug-related treatments fail.
FA is a serious and rare disease, so patient-doctor communication is absolutely necessary to help further studies on how to treat the disease effectively. Prognosis can greatly improve if new treatment options can be made available to patients. Clinical trials are ongoing for patients who would like to participate (ClinicalTrials.gov – U.S. National Institutes of Health).
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