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Diagnosis of Scleroderma Pulmonary Involvement
This page was written by Shelley Ensz, and has not yet been medically edited. Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See Disclaimer.
Overview of Diagnosis of Scleroderma Pulmonary Involvement
Tests for Pulmonary Involvement
Diagnosis of Pulmonary Fibrosis
Diagnosis of Pulmonary Hypertension
Research on Diagnosis of Pulmonary Involvement
Overview of Diagnosis of Scleroderma (Systemic Sclerosis) Pulmonary Involvement
"Pulmonary function testing is recommended for all systemic sclerosis (SSc) patients."
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.
Diagnosis and management of pulmonary disorders requires a history, a physical examination, and usually chest x-rays. Pulmonary function testing, arterial blood gas analysis, chemical or microbiologic tests, or special studies (eg, endoscopy, bronchoalveolar lavage, biopsy, radionuclide scanning) may be needed. Merck Manual.
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
Cytokine Levels In Bronchoalveolar Lavage Fluid (Balf) In Patients With Systemic Sclerosis (SSc). In comparison to other diseases, SSc patients present a specific cytokine pattern in BALF. The detection of high MCP-1 levels in patients with fibrosis and in patients with decreased DLCO after one year of treatment suggests a key function of this cytokine as potential therapeutic target in SSc lung involvement. G. Riemekasten THU0318 EULAR 2007. (Also see: Causes of Scleroderma: Cytokines)
Echocardiography
“Echocardiography cannot be relied upon to exclude pulmonary hypertension when pre-test probability is high.” Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. The positive predictive accuracy of currently used non-invasive tests are adequate for the diagnosis of advanced PAH provided sufficiently high thresholds (TG 45 mmHg or DLCO < 55% predicted) are used. These tests cannot be relied upon to exclude pulmonary hypertension where pre-test probability is high. PubMed. Rheumatology (Oxford). 2004 Apr;43(4):461-6. (Also see: Pulmonary Hypertension Diagnosis)
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.
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)
Interpretation of Pulmonary Function Test (PFTs). A PFT report provides a wealth of measurements; however, in the final analysis, only a few of these measurements are necessary to make a pretty good assessment of the patient's condition. James Allen, MD.
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)

How is PH/PAH Diagnosed? Pulmonary function tests can be helpful. Blood pressure in the lung can be estimated and the size and function of the right ventricle assessed by Doppler echocardiography. However, Doppler echocardiograms are notoriously inaccurate when the PH is mild or when there is simultaneous presence of lung scarring. PH cannot be diagnosed without right heart catheterization. James R. Seibold, MD, International Scleroderma Network Newsletter. 09-30-07. (Also see: Pulmonary Hypertension Diagnosis)
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)
Six-Minute Walk Test for the Evaluation of Pulmonary Involvement in 110 Scleroderma Patients. Hemoglobin desaturation during a 6MWT provides additional information regarding the severity of disease in scleroderma patients with pulmonary manifestations. W. O. Villalba. SAT0232 EULAR 2006.
Diagnosis of Pulmonary Fibrosis
Diagnosis Overview
High Resolution Computed Tomography
Induced Sputum and Bronchoalveolar Lavage
Lung Biopsy
Pulmonary Function Tests
Walking and Stress Tests
Pulmonary Fibrosis Antibodies
Diagnosis of Pulmonary Hypertension
Overview of Diagnosis of PH/PAH in Scleroderma
Diagnostic Algorithm
DLCO in PH
Echocardiograms
Exercise Echocardiograph
Functional Classification
High Resolution Chest CT
Markers for PH in Scleroderma
P-Wave Amplitude
Right Heart Catheterization (RHC)
Risk Factors for PH/PAH
Six-Minute Walk
TAPSE as Survival Predictor
Research on Diagnosis of Scleroderma Pulmonary Involvement
Device Could Crack Case on Pulmonary Diseases. A lung-on-a-chip that re-creates respiratory crackles might help doctors understand whether the crackling is a contributor to lung damage or just a symptom of a disease. Medical Device Link (R&D Digest) February, 2008.
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.
Pulmonary-renal vasculitic disorders: differential diagnosis and management. Pulmonary-renal syndrome is not a single entity and is caused by a variety of conditions, including Goodpastures syndrome associated with autoantibodies to the glomerular and alveolar basement membranes, various forms of primary systemic vasculitis associated with serum positivity for antineutrophil cytoplasmic antibodies (ANCA), cryoglobulinemia, systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, environmental factors, and drugs. PubMed. Curr Rheumatol Rep 2003 Apr;5(2):107-15 (Also see: Renal Involvement)
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