| 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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| Pulmonary Fibrosis Diagnosis |
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| Diagnosis Overview |
| 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. |
| Medical Tests: Pulmonary. Diagnostic tests for pulmonary fibrosis include CAT scan, echocardiogram, pulmonary function test, chest x-ray, bronchoscopy, and bronchoalveolar lavage. ISN. |
| Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease. In the presentation of an idiopathic interstitial pneumonia, the presence of a nucleolar-staining ANA, telangiectasia, Raynaud phenomenon with abnormal capillaroscopy findings, gastroesophageal reflux, or pericardial disease suggests underlying systemic sclerosis.. PubMed. Chest. 2006 Oct;130(4):976-81. (Also see: Diffuse Without Skin Involvement and Pulmonary Involvement ) |
| Dyspnea Due to Pulmonary Hypertension and Interstitial Lung Disease in Scleroderma: Room for Improvement in Diagnosis and Management. Rigorous assessment for pulmonary disease in patients with scleroderma, will reliably define the cause of dyspnea and exercise intolerance in the majority of patients. J Rheumatol 2006 September;33:1723. Editorial: H. Racz, S. Mehta. (Also see: Pulmonary Hypertension ) |
| Clinical Features of Scleroderma Sine Scleroderma-Associated Progressive Interstitial lung disease (ILD). Scleroderma may present as progressive ILD without the characteristic skin-tightening features of scleroderma. Scleroderma sine scleroderma-associated progressive ILD is associated with a high prevalence of telangiectasias, Raynaud’s with abnormal nailfold capillaroscopy, esophageal dysmotility, nucleolar-staining ANA, anti-Th/To-antibody positivity, echocardiographically-defined pulmonary hypertension, and asymptomatic pericardial disease. Aryeh Fischer. 1574/350. ACR 2005. (Also see: Diffuse Scleroderma) |
| New Developments in Scleroderma Interstitial Lung Disease. Basic and clinical studies of systemic sclerosis patients with interstitial lung disease are yielding promising data that ultimately will be translated in to more effective diagnostic and therapeutic strategies. PubMed. Curr Opin Rheumatol. 2005 Nov;17(6):737-745. |
| Fibrosing alveolitis in systemic sclerosis: the need for early screening and treatment. Screening of patients recently diagnosed with systemic sclerosis (SSc) by pulmonary function tests and the performance of high resolution computed tomography when physiological abnormalities are identified has resulted in the identification of significant numbers of patients with early, asymptomatic FASSc (Fibrosing alveolitis in SSc). PubMed. Intern Med J. 2004 Nov;34(11):626-638. |
| High Resolution Computed Tomography (HRCT) |
| Significance of Ground-glass Opacity on HRCT in Long-term Follow-up of Patients With Systemic Sclerosis. In systemic sclerosis, ground-glass opacity is most commonly associated with irreversible disease. Disease progression or improvement could not be predicted by the presence of ground-glass opacity. Journal of Thoracic Imaging. 22(2):120-124, May 2007. |
| High Resolution Computed Tomography (HRCT) in Fibrosing Alveolitis Associated with Systemic Sclerosis (FA-SSc). Patients with FA-SSc with abnormal HRCT experienced progressive replacement of ground-glass opacities by honeycombing and/or traction bronchiectasis/bronchiolectasis. Ground-glass opacity is probably the first step of lung fibrosis in SSc, and treatment should be discussed even at this early stage. J Rheumatol 2006 September;33:1789-801. |
| CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Interstitial lung disease in patients with SSc is less extensive, less coarse, and characterized by a greater proportion of ground-glass opacification than that in patients with IPF. The CT features of lung disease in patients with SSc closely resemble those in patients with idiopathic NSIP. PubMed. Radiology. 2004 Aug;232(2):560-7. |
| Scleroderma, Thoracic Patients with suggested or known diagnosis of systemic sclerosis should undergo a CAT Scan. eMedicine Journal |
| Sequential Evaluation of Pulmonary Involvement in 90 Patients with Systemic Sclerosis by High-Resolution CT. For patients with abnormal HRCT, there could be a natural history of the lung fibrosis with a spatial extension of the fibrosis progressively replacing the ground-glass lesions, especially in the male patients. A decrease of the diffusing capacity appears to be associated with the progression of the fibrosis. David Launay. ACR Conference Oct. 2003. |
| Interstitial Lung disease in Systemic Sclerosis Qualitative HRCT (high-resolution CT) is able to evaluate inflammation and fibrosis, showing important relationships with diffusion capacity and lung volume, respectively. PubMed. Acta Radiol 2003 May;44(3):258-64. |
| Induced Sputum and Bronchoalveolar Lavage Tests |
| A Vascular endothelial growth factor (Vegf) Deficiency Characterizes Scleroderma Interstitial Lung Disease. Scleroderma lung disease is characterized by a VEGF deficiency. Lower bronchoalveolar lavage fluid VEGF levels were found in the patients with a worse lung involvement and with the progression of pulmonary disease. M. De Santis. FRI0258. EULAR 2008. |
| Role of bronchoalveolar lavage in diagnosis of interstitial lung disease. Bronchoalveolar lavage (BAL) is a minimally invasive diagnostic technique that yields insights into immunologic, inflammatory, and infectious processes occurring at the alveolar level. UpToDate for Patients. Janunary, 2008. |
| Prognostic Significance of Bronchoalveolar Lavage Cellular Analysis in Scleroderma Lung Disease. Analysis of data from the Scleroderma Lung Study found that abnormal bronchoalveolar lavage (BAL) cellularity was associated with more advanced Interstitial lung disease (ILD), but was of no benefit in predicting disease progression or treatment response. Red Orbit. 06/03/08. |
| Induced Sputum (IS): A Non-Invasive Tool for Evaluation of Lung Involvement and Alveolar Inflammation in Systemic Sclerosis. IS method supplied information comparable to bronchoalveolar lavage (BAL) findings in patients with SSc, indicating that invasive BAL technique could be replaced by noninvasive IS method in assessing lung disease in SSc. N. S. Damjanov. FRI0339 EULAR 2006. |
| Beta thromboglobulin and platelet factor 4 in bronchoalveolar lavage fluid of patients with systemic sclerosis. The study provides evidence that activation of blood platelets takes place within the lungs of patients with scleroderma lung disease (SLD) and may contribute to the development of lung fibrosis. PubMed. Ann Rheum Dis. 2005 Mar;64(3):484-6. |
| Regional differences in bronchoalveolar lavage and thoracic high-resolution computed tomography results in dyspneic patients with systemic sclerosis. These data suggest that, in addition to HRCT, BAL with lavage, differential cell counting, and culture from at least 2 segments of lung be performed for diagnosing SSc alveolitis. PubMed. Arthritis Rheum. 2004 Jun;50(6):1909-17. |
| Lung Biopsy |
| Surgical lung biopsy for diffuse pulmonary disease: Experience of 196 patients. Surgical lung biopsy is a safe and accurate diagnostic tool for diffuse pulmonary disease. For a large proportion of the patients, change of therapy and then clinical improvement can be achieved after surgical lung biopsy. Surgical lung biopsy should be considered earlier in patients with undiagnosed diffuse pulmonary disease, especially when the respiratory condition is deteriorating. J Thorac Cardiovasc Surg 2005;129:984-990. |
| Pulmonary FunctionTests |
| Pulmonary Function Tests. Pulmonary function tests are tests performed to make measurements of how your lungs and airways function. Results from pulmonary function tests enable your physician to evaluate your breathing, make diagnosis, recommend treatment and follow your progress. This article tells you how to prepare for the test and how it is conducted. National Jewish Medical and Research Center. |
| Scleroderma lung: Initial forced vital capacity as predictor of pulmonary function decline. Measured within the first 3 years from disease onset, baseline FVC (percent predicted) may predict deterioration of pulmonary function in patients with scleroderma. PubMed. Arthritis Rheum. 2006 Jul 27;55(4):598-602. (Also see: Pulmonary Involvement ) |
| Predicting 24 Month Pulmonary Function Test, Modified Rodnan Skin Score (mRSS) and Functional Results from Baseline Measurements in the Cyclophosphamide versus Placebo Trial of Systemic Sclerosis (SSc) Alveolitis. This study suggests that 12 months of cyclophosphamide results in continued benefit at 24 months. Daniel E. Furst. 1130/389. ACR 2006. (Also see:Skin Fibrosis, and Clinical Trials: Cyclophosphamide and Scleroderma Lung Disease ) |
| Walking and Stress Tests |
| Six-minute walk test for the evaluation of pulmonary disease severity in scleroderma patients. Hemoglobin desaturation during a 6 minute walk test (MWT) provides additional information regarding severity of disease in scleroderma patients with pulmonary manifestations. PubMed. Chest. 2007 Jan;131(1):217-22. (Also see: Pulmonary Hyptertension ) |
| Submaximal exercise testing in the assessment of interstitial lung disease secondary to systemic sclerosis: reproducibility and correlations of the 6-min walk test (6MWT). The lack of correlation of 6MWT with standard physiological parameters of ILD suggests a multifactorial basis for limited exercise capacity in patients with SSc and calls into question the utility of the 6MWT as a measure of outcome in future studies on SSc-ILD. Annals of the Rheumatic Diseases 2007;66:169-173. |
| Pulmonary Fibrosis Antibodies |
| Comprehensive investigation of novel serum markers of pulmonary fibrosis associated with systemic sclerosis and dermato/polymyositis. KL-6, SP-D, vWF and ES (specific serum levels) are good surrogate factors of pulmonary fibrosis but can not replace conventional diagnostic procedures. However, these markers are suitable for the assessment of progression and severity of pulmonary fibrosis in systemic autoimmune disorders once the diagnosis is established. Clin Exp Rheumatol 2008; 26: 414-420. |
| 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 ) |
| The role of anti-endothelial cell antibody-mediated microvascular injury in the evolution of pulmonary fibrosis in the setting of collagen vascular disease. Pulmonary fibrosis, a recognized complication of systemic connective tissue disease, develops in connective tissue disease syndromes with pathogenetically established immune-based microvascular injury at other sites. A similar mechanism of antibody-mediated endothelial cell injury may be the basis of the tissue injury and fibrosing reparative response. PubMed. Am J Clin Pathol. 2007 Feb;127(2):237-47. |
| Rice University breakthrough could prevent multiple fibrotic diseases. A protein, which is called serum amyloid P (or SAP), has proven effective at preventing fibrotic disease from developing in the hearts and lungs of lab animals, and researchers hope it will eventually save thousands of lives once it is developed for human use. EurekAlert! 01/18/07. (Also see: Skin Fibrosis ) |
| Anti-lipoprotein lipase antibody in systemic sclerosis: association with elevated serum triglyceride concentrations. The presence of IgG anti-LPL antibody was associated with elevated serum triglyceride levels, greater extent of skin fibrosis, and more frequent presence of lung fibrosis, heart involvement, and anti-topoisomerase I antibodies. Our results suggest that anti-LPL autoantibody contributes to elevated serum triglyceride levels by inhibiting LPL enzyme activity in patients with SSc. J Rheumatol. 2005 Apr;32(4):629-36. (Also see: Antibodies, Skin Fibrosis, and Cardiac Involvement) |
| Curcumin-Induced Apoptosis in Scleroderma Lung Fibroblasts: Role of Protein Kinase C {epsilon}. These observations suggest that curcumin may have therapeutic value in treating scleroderma, just as it has already been shown to protect rats from lung fibrosis induced by a variety of agents. PubMed. Am J Respir Cell Mol Biol. 2004 Jan 23. (Also see: Pulmonary Fibrosis) |
| Direct and indirect immunofluorescence as a diagnostic adjunct in the interpretation of nonneoplastic medical lung disease. With respect to the septal capillary pattern, endothelial cell decoration was seen with scleroderma, mixed connective tissue disease, anti-Ro-associated lupus erythematosus, dermatomyositis, humoral allograft rejection, and patients with isolated pulmonary fibrosis in whom autoantibodies were established, including antiphospholipid antibodies. PubMed. Am J Clin Pathol 2003 Feb;119(2):279-89. |
