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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.
Pulmonary fibrosis (PF) can occur by itself or as seconday to some autoimmune diseases, including systemic sclerosis (SSc, scleroderma).
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.)
Key roles for interferon- and TGF-beta-regulated genes, and macrophage activation in progressive lung fibrosis associated with Systemic Sclerosis. This highlights major pathogenic pathways relevant to progressive pulmonary fibrosis in SSc-ILD: macrophage activation, and upregulation of TGF-beta- and IFN-regulated genes. PubMed, Arthritis Rheum, 2013 Nov 27.
Good outcome of interstitial lung disease in patients with scleroderma associated to anti-PM/Scl antibody. Several features and prognosis of ILD in SSc may be modified depending on the identified immunological profile. Seminars in Arthritis and Rheumatism, 07/15/2014. (Also see Antibodies in Systemic Scleroderma)
Proteome-wide Analysis and CXCL4 as a Biomarker in Systemic Sclerosis. Levels of CXCL4 were elevated in patients with systemic sclerosis and correlated with the presence and progression of complications, such as lung fibrosis and pulmonary arterial hypertension. The New England Journal of Medicine, 12/18/2013. (Also see Biomarkers and Pulmonary Hypertension)
Skin gene expression correlates of severity of interstitial lung disease (ILD) in systemic sclerosis. A limited number of skin transcripts including genes involved in extravasation and adhesion of inflammatory cells correlate with severity of ILD. American College of Rheumatology, 07/30/2013.
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. ISN.
Diagnosis of Pulmonary Fibrosis. All systemic sclerosis patients should have regular screening for both pulmonary fibrosis and pulmonary hypertension. ISN.
| Overview High Resolution Computed Tomography Induced Sputum and Bronchoalveolar Lavage Lung Biopsy |
Pulmonary Function Tests Ultrasound Walking and Stress Tests Pulmonary Fibrosis Antibodies |
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.
| Overview Air Quality Exercises |
Oxygen Therapy Dangers of Oxygen Support Groups |
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.
Reconciling Healthcare Professional and Patient Perspectives in the Development of Disease Activity and Response Criteria in Connective Tissue Disease-related Interstitial Lung Diseases (CTD-ILD). Efforts described here demonstrate unequivocally the value and influence of patient involvement on core set development. PubMed, J Rheumatol, 2014 Feb 1. (Also see Connective Tissue Disease)
Combined Pulmonary Fibrosis and Emphysema (CPFE) Among Patients With Scleroderma (SSc)-Associated Pulmonary Hypertension (PH). There appears to be no difference in the severity of PH or in pulmonary function between SSc-PH patients with and without CPFE. Chest, 2013;144. (Also see Pulmonary Hypertension)
Pulmonary Fibrosis Preceding Skin Involvement in Scleroderma. Pulmonary fibrosis can be the initial manifestation of scleroderma, preceding skin involvement. Chest, 2013;14.
Targeting Focal Adhesion Kinase (FAK) in Fibrotic Diseases. Recent findings suggest that FAK plays a key role in development of fibrotic disorders, and it appears to be an attractive target for antifibrotic therapy and has potential as a future therapeutic target to counteract fibrosis. PubMed, BioDrugs, 2012 Nov 27. (Also see Pulmonary Fibrosis Research and Clinical Trials)
(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.
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