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Diagnosis of Scleroderma Pulmonary Involvement |
| Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer |
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Overview of Diagnosis of Scleroderma (Systemic Sclerosis) Pulmonary Involvement |
| "Pulmonary function testing is recommended for all systemic sclerosis (SSc) patients." |
| The management of pulmonary manifestations in scleroderma. Aggressive screening and specialist-care involvement are crucial for early detection of the pulmonary disease long before it is clinically apparent. Combined rheumatology/respiratory clinics have been shown to be an effective way of providing a streamlined approach to patient care, enabling potentially life-threatening pulmonary complications to be diagnosed early and treated. Dr. Fahd Adeeb. Irish Medical Times. 01/20/11. |
| Scleroderma lung disease, variation in screening, diagnosis and treatment practices between rheumatologists and respiratory physicians. The heterogeneity of responses revealed in this study raises the importance of screening, diagnosis and treatment algorithms in the management of this potentially life-threatening disease. P Mangat. (PubMed) Intern Med J. 2010 Jul;40(7):494-502. |
| Pulmonary functional abnormalities in asymptomatic patients with systemic sclerosis. A functional defect, mainly of the restrictive type, was observed in the majority of asymptomatic patients with systemic sclerosis (SSc). Pulmonary function testing is recommended for all systemic sclerosis (SSc) patients. PubMed. Eur J Intern Med. 2003 May;14(3): 162-165. (Also see: What is Scleroderma? and Types of Scleroderma) |
| Non-invasive diagnostic and functional evaluation of cardiac and pulmonary involvement in systemic sclerosis (SSc). Pulmonary involvement occurs both in limited and in diffuse cutaneous SSc patients and develops, in 83% of the cases, without any regional lung perfusion abnormality. Cardiac involvement is detected in 65% of the cases. Lung perfusion MRI should be considered as a complementary diagnostic method for the functional evaluation of these symptoms in systemic sclerosis. PubMed. In Vivo. 2004 Mar-Apr;18(2):229-35. (Also see: Cardiac Involvement) |
Tests for Scleroderma Pulmonary Involvement |
| Scleroderma Care and Research Journal: Spring 2004. This free online PDF medical journal issue includes articles on Diagnostic and Management Preferences for Lung Disease in Scleroderma for medical professionals. SCTC 6-2-04. |
| Bronchoalveolar Lavage |
| Bronchoalveolar lavage (BAL) and lung function in patients with systemic sclerosis and subsequent pulmonary impairment. The investigation by BAL appears to be a reliable tool for the assessment of the pulmonary impairment related to systemic sclerosis. PubMed. Pneumologia 2002 Jul-Sep;51(3):203-5 |
| Gene Expressions |
| Lung tissues in systemic sclerosis (SSc) have gene expression patterns unique to pulmonary fibrosis and pulmonary hypertension Using microarray analysis on carefully phenotyped SSc and comparator lung tissues, we demonstrated distinct molecular profiles in tissues and fibroblasts of patients with SSc-associated lung disease compared to idiopathic forms of lung disease. Unique molecular signatures were generated that are disease- (SSc) and phenotype- (PF vs PAH) specific. Eileen Hsu (PubMed) Arthritis and Rheumatism, Nov. 19, 2010. (Also see: Genetics) |
| Nail Abnormalities |
| Pulmonary Disorders. Clubbing is enlargement of the fingertips (or toes) due to proliferation of connective tissue between the fingernail and the bone. Clubbing is most commonly observed in lung cancer but is an important sign of chronic pulmonary disease, such as cystic fibrosis and idiopathic pulmonary fibrosis. The Merck Manuals. November 2005. (Also see: Fingernails) |
| Nail Abnormalities: Clues to Systemic Disease. The visual appearance of the fingernails and toenails may suggest an underlying systemic disease. Clubbing of the nails often suggests pulmonary disease or inflammatory bowel disease. R. S. Fawcett, M.D., M.S. American Family Physician. March 15. 2004.(Also see: Fingernails) |
| Echocardiography |
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| High Resolution CT |
| Imaging Lung Disease in Systemic Sclerosis. Interstitial lung disease and pulmonary hypertension (PH) are the most common cardiopulmonary findings in patients with systemic sclerosis High-resolution CT scanning is a key method for evaluating chest involvement. Diane Strollo. (MetaPress) Current Rheumatology Reports. Volume 12, Number 2 / April, 2010. |
| Nailfold Capillary Microscopy |
| Nailfold Capillary Microscopy Can Suggest Pulmonary Disease Activity in Systemic Sclerosis (SSc). The severity of NCM abnormalities is associated with lung disease activity in SSc, particularly when the disease duration is relatively short. J Rheumatol No. 2 Feb. 2004;31:286-94. |
| Nitric Oxide |
| Alveolar Concentration Of Nitric Oxide Predicts The Occurrence Of Pulmonary Function Worsening In Patients With Systemic Sclerosis (SSc). Increased concentration of nitric oxide identified accurately SSc patients with high risk to develop lung function deterioration, and may help to initiate early appropriate treatment. K.P. Tiev. EULAR 2011 OPO 235. Ann Rheum Dis 2011;70(Suppl3):148. |
| Overnight Pulse Oximetry Monitoring |
| Variations in Physician Interpretation of Overnight Pulse Oximetry Monitoring. The variation in physician interpretation, use, and opinions regarding overnight pulse oximetry calls into question its clinical utility and underscores a need for standardization of presentation, training, and interpretation. Chest, doi:10.1378/chest.07-0312. (Also see: Sleep Disorders) |
| Pulmonary Function Test (PFT) |
| 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 Fibrosis) |
| Right Heart Catheterization (RHC) |
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| Six-Minute Walk Test (6MWT) |
| The 6-minute walk test in scleroderma--how measuring everything measures nothing. Never fully validated in scleroderma, a variety of data suggest that many non-pulmonary aspects of scleroderma contribute to its results thereby blunting the ability of the 6MWT to measure change in lung function. (UnBound MedLine) Impens AJ. Rheumatology (Oxford) 2008 Oct.:v68-9. (Also see: Pulmonary Fibrosis) |
Diagnosis of Pulmonary Fibrosis |
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Diagnosis of Pulmonary Hypertension |
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Research on Diagnosis of Scleroderma Pulmonary Involvement |
| Serum interleukin-15 (IL-15) in patients with early systemic sclerosis (SSc) - a potential novel marker of lung disease. We conclude that IL-15 is associated with fibrotic as well as vascular lung disease and vasculopathy in early SSc. IL-15 may contribute to the pathogenesis of SSc. Arthritis Research & Therapy 2007, 9:R85. |
| Thoracic Manifestations of Inflammatory Bowel Disease (IBD) . Respiratory symptoms and diagnosed respiratory system disorders are more common among patients with IBD than generally appreciated. Chest. 2007; 131:524-532. (Also see: IBD) |
| The Effects of Body Mass Index (BMI) on Lung Volumes. BMI has significant effects on all of the lung volumes, and the greatest effects were on FRC (functional residual capacity) and ERV (expiratory reserve volume), which occurred at BMI values < 30 kg/m2. Chest. 2006;130:827-833. |
