| LUNG (PULMONARY): MAIN MENU |
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| Pulmonary Fibrosis |
| This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer. |
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| Overview of Pulmonary Fibrosis |
| Pulmonary (lung) fibrosis, is a scarring of the lungs, and is the consequence of untreated pulmonary inflammation (alveolitis). It is often also referred to as interstitial lung disease. |
| What is pulmonary fibrosis? Pulmonary fibrosis is scarring throughout the lungs. Pulmonary fibrosis can be caused by many conditions such as sarcoidosis, hypersensitivity pneumonitis, asbestosis, certain medications, etc. MedicineNet |
| About 70% of patients with diffuse scleroderma develop some degree of pulmonary fibrosis, which is the most common cause of death directly related to scleroderma. Therefore, prompt diagnosis and aggressive treatment of pulmonary fibrosis is very important. |
| Pulmonary fibrosis is also one of the three minor criteria (1) for the classification of limited systemic scleroderma. (See Types of Scleroderma.) |
| Ask The Mayo Clinic: Causes and treatments for pulmonary fibrosis. I've had numerous bouts of pneumonia over the years. Now I've been told that I have pulmonary fibrosis. Can you tell me what to expect and is there anything I can do? SeattlePI.com. 06/08/08. |
| Pulmonary Fibrosis Research Enhancement Act (PFREA) Of 2009 Introduced In 111th U.S. Congress. The PFREA would expand the research, prevention, and awareness activities of the Centers for Disease Control and Prevention and the National Institutes of Health with respect to pulmonary fibrosis. Medical News Today. 02/20/09. |
| Deterioration of lung function is associated with presence of IgM rheumatoid factor (RF) and smoking in patients with systemic sclerosis (SSc). The presence of IgM RF in smoking SSc patients is associated with deteriorating lung function. Berit Broholm. (SpringerLink) Clinical Rheumatology. 06/05/08. (Also see: Quit Smoking). |
| First different black/white mechanism in pulmonary fibrosis/scleroderma identified. Malfunction in c-Met receptor parallels increased prevalence, doubled prognosis suffered by African-Americans in group of lung disease. Of the more than 40,000 persons who die each year in the U.S. from pulmonary fibrosis, the mortality rate among African-Americans is twice as high as Caucasians. EurekAlert! 04/04/06. |
| The natural course of progressive systemic sclerosis patients with interstitial lung involvement. Pulmonary symptoms tend to appear at a mean of 7 years after the onset of disease.The first sign of interstitial pulmonary involvement is highest during the first 15 years. Immunosuppressive treatment is not effective in preventing the development of pulmonary involvement. However, it delays the manifestation of pulmonary symptoms for nearly 4 years. PubMed. Clin Rheumatol. 2006 Apr 25. |
| Mortality and Prognosis |
| Exercise Peripheral Oxygen Saturation (SpO2) Accurately Reflects Arterial Oxygen Saturation (SaO2) and Predicts Mortality in Systemic Sclerosis (SSc). Among patients with SSc interstitial lung disease (ILD), SpO2 is an adequate reflection of SaO2, and radial arterial lines need not be inserted during cardiopulmonary exercise test in these patients. Jeffrey J Swigris Thorax. 8 April 2009. |
| New Prognostic Indicator For Patients With Idiopathic Pulmonary Fibrosis (IPF). There may be a new way to predict mortality in patients with (IPF), a devastating disease that slowly petrifies the lungs. Most patients live only three years after diagnosis on average; however, some remain stable for many years, while for others, the disease progresses more rapidly. Medical News Today. 02/22/09. |
| Risk Factors For Mortality In Patients With Systemic Sclerosis (SSc) And Interstitial lung disease (ILD). Mortality in patients with SSc and ILD is increased in those with an early and severe impairment of pulmonary function, concomitant cardiac involvement and elevated ESR (Sed rate), but not in Scl-70 positive patients. Beatriz E. Joven. 6/6. ACR 2007. (Also see: Systemic: Mortality and Prognosis) |
| Symptoms |
| Symptoms of pulmonary fibrosis include shortness of breath (dyspnea) on exertion. In treating scleroderma, its best to identify pulmonary fibrosis before it is so advanced that it shows up on x-ray. That is why your doctor may order an echocardiogram, or other tests. (Also see Pulmonary Tests) ISN. |
| Correlation of Pulmonary Fibrosis with Other Complications |
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| Diagnosis |
| Diagnosing Pulmonary Fibrosis. Pulmonary fibrosis is one of many interstitial lung diseases that scar lung tissue. Scarring and stiffening of the alveoli decrease transport of oxygen across the alveolar membrane. Advance News Magazine for Physician Assistants. |
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| Living with Pulmonary Fibrosis |
| Living with Pulmonary Fibrosis requires a constant awareness of your symptoms, your breathing, your surroundings, and your overall well-being. Work closely with your doctors on treatments, diet and exercise. If you require oxygen therapy, use it. It protects your heart as well as improves the quality of your life. ISN. |
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| Treatments |
| Treatments for Pulmonary Fibrosis include oxygen therapy, oral and IV cyclophosphamide (cytoxan), biologic agents, Mycophenolate Mofetil (cellcept), and lung transplants. There are current clinical trials that are studying the effectiveness of various treatments for scleroderma. Some of these trials are using the treatment's effect on the patient's pulmonary fibrosis as a measurement criterion. ISN. |
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| Pulmonary Fibrosis Research |
| Interstitial lung disease and sleep: What is known? There are a number of distinctive changes in sleep architecture associated with pulmonary fibrosis including decreased REM (rapid eye movement) sleep and increased sleep fragmentation. In addition, there is suggestion of possible sleep disturbances in this population including obstructive sleep apnea, although the frequency of such sleep abnormalities as well as predictors of these abnormalities remain uncertain. Shikhar Agarwal Sleep Medicine. October 2009. (Also see: Sleep Disorders) |
| Healing process found to backfire in lung patients. A mechanism in the body which typically helps a person heal from an injury, may actually be causing patients with idiopathic pulmonary fibrosis (IPF) to get worse. EurekAlert! 10/27/08. |
| Histopathology and bronchoalveolar lavage. Studies employing lung biopsy material and BAL fluid (BALF) have provided insight into the pathogenesis of scleroderma-associated interstitial lung disease (SSc-ILD). (UnBound MedLine) Silver RM. Rheumatology (Oxford) 2008 Oct.:v62-4. |
| Study Sheds Light On Deadly Lung Disease. Researchers generated a transgenic mouse strain, which develops ubiquitous skin and sporadic lung scar tissue, characteristics similar to humans with SSc and pulmonary fibrosis. Medical News Today. 04/15/08. |
| References |
| (1) Reference: Subcommittee for Scleroderma criteria of the American Rheumatism Association diagnostic and therapeutic criteria committee. 1980. Preliminary criteria for the classification of systemic sclerosis (Scleroderma). Arthritis Rheum. 23,581:590. |
