| 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.) |
| 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. |
