| LUNG (PULMONARY): MAIN MENU |
| |
| Pulmonary Fibrosis |
| This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer. |
| |
| 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. |
| 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. |
| Among a Group of Patients with Dyspnea and Alveolitis Entering the Scleroderma Lung Study (SLS), a Surprising Proportion have Limited SSC (lSSc). In the SLS, lSSc patients with systemic sclerosis of short duration and dyspnea had Interstitial lung disease (ILD) and alveolitis that was not distinguishable from the diffuse scleroderma patients.Physicians need to be aware of this in evaluating dyspnea in the lSSc population. Daniel Eric Furst. 1064/444. ACR 2004. |
| Organ Involvement in Systemic Sclerosis - The Lung. Interstitial fibrosis strikes up to 90% of patients with systemic sclerosis (scleroderma), causes restrictive lung disease in 30-50% of patients and progresses to significant lung damage in about 15% of patients. B. White. SP0013 EULAR 2003. |
| Mortality and Prognosis |
| 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 ) |
| Outcome of patients with scleroderma admitted to intensive care unit. A report of nine cases. The outcome of scleroderma patients admitted to the ICU was extremely poor. Infectious complication was the most common cause of death in our patients. PubMed. Clin Exp Rheumatol. 2006 Jul-Aug;24(4):380-6. (Also see: Systemic: Mortality and Prognosis ) |
| Changes in Causes of Death in Systemic Sclerosis Over the Past 30 Years. Throughout the past 30 years, the frequency of deaths from RC (renal crisis) has dramatically decreased and at the same time the frequency of PF (pulmonary fibrosis) increased. However, only 10% of SSc patients surviving RC have died of PF, which may be because patients with the highest frequency of RC have a low frequency of PF. Over the past 10 years, SSc patients have had improved survival, longer disease duration at the time they die and are less likely to die from scleroderma related complications. Pulmonary hypertension and pulmonary fibrosis now account for 50% of SSc related deaths and 25% of all causes of death in SSc patients. Virginia Steen. 1052/432. ACR 2004. (Also see: Renal Involvement, Pulmonary Hypertension, and Types of Scleroderma: Prognosis and Mortality) |
| 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 |
| |
| 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. |
| |
| 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. |
| |
| 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. |
| |
| Pulmonary Fibrosis Research |
| 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. |
| Molecular Pathway Appears Crucial In Development Of Pulmonary Fibrosis.The investigators found that a specific molecular pathway appears responsible for key aspects of the scarring of lung tissue that characterizes Idiopathic Pulmonary Fibrosis. Medical News Today. 12/16/07. |
| Impairment of the antifibrotic effect of hepatocyte growth factor (HGF) in lung fibroblasts from African Americans: Possible role in systemic sclerosis. Reduced levels of HGF as well as a deficiency in c-Met receptor function appear to be present in African American patients with SSc. These findings may explain in part the greater disease severity and worse prognosis observed in African Americans with SSc. Arthritis and Rheumatism. Volume 56, Issue 7, Pages 2432 - 2442. (Also see: Scleroderma Prognosis ) |
| Scientist Discovers Clue In Mysterious Lung Disease. Even though it affects more than 5 million people world-wide, pulmonary fibrosis remains a mysterious and incurable disease. A recent discovery could someday change that. Researchers have uncovered a clue as to how this devastating lung disease is formed, and how they might try to stop it. Medical News Today. 10/31/07. |
| 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. |
