Diagnosis of Scleroderma Pulmonary Involvement

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Tests for Pulmonary Involvement
Diagnosis of Pulmonary Fibrosis
Diagnosis of Pulmonary Hypertension

Overview of Diagnosis of Scleroderma (Systemic Sclerosis) Pulmonary Involvement

"Pulmonary function testing is recommended for all systemic sclerosis (SSc) patients."

Treatment and prognosis of interstitial lung disease in systemic sclerosis (scleroderma). The clinical presentation and diagnosis of scleroderma lung disease and treatments are reviewed here. Uptodate. (Also see What is Scleroderma?)

Tests for Scleroderma Pulmonary Involvement

Bronchoalveolar Lavage

Biomarkers from bronchoalveolar lavage fluid (BALF) in systemic sclerosis (SSc) patients with interstitial lung disease (ILD) relate to severity of lung fibrosis. In SSc related ILD, biomarkers from BALF and serum correlate to findings on HRCT suggesting usefulness as markers of presence and extent of lung fibrosis. PubMed, Respir Med. (Also see Pulmonary Fibrosis Research and Induced Sputum and Bronchoalveolar Lavage Tests)

Gene Expressions

Lung tissues in systemic sclerosis (SSc) have gene expression patterns unique to pulmonary fibrosis and pulmonary hypertension 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

Nail Abnormalities. Disorders that affect the amount of oxygen in the blood (such as abnormal heart anatomy and lung diseases including cancer or infection) may cause clubbing. MedLine Plus.


“Echocardiography cannot be relied upon to exclude pulmonary hypertension when pre-test probability is high.”

Clinical and echocardiographic correlations of exercise-induced pulmonary hypertension in systemic sclerosis: a multicenter study. Exercise-induced increase in PASP occurs in almost one-half of patients with systemic sclerosis (SSc) with normal resting pulmonary artery systolic pressure (PASP). Am Heart J.

High Resolution CT

Quantitative chest computed tomography analysis in patients with systemic sclerosis before and after autologous stem cell transplantation. CT quantification of lung volume and parenchymal attenuation in SSc patients presenting with alveolitis and fibrosis that undergo autologous SCT yields parameters that match well with those of pulmonary function and even clinical tests. PubMed, Rheumatology (Oxford), 06/21/2016.

Quantitative volumetric assessment of pulmonary involvement in patients with systemic sclerosis (SSc). The percentage of lower lobe volume (PLLV) of the right lung may decrease in SSc patients with interstitial lung disease and the PLLV may be a quantitative parameter indicating damage in the lung. Quantitative Imaging in Medicine and Surgery, 03/09/2016.

Multimodality Thoracic Imaging of Juvenile Systemic Sclerosis: Emphasis on Clinical Correlation and High-Resolution CT of Pulmonary Fibrosis. Common thoracic findings in juvenile systemic sclerosis and treatment decisions and prognostic assessment are directly related to imaging findings along with clinical evaluation. PubMed, AJR Am J Roentgenol, 2015 Feb;204(2):408-22. (Also see Juvenile Scleroderma)

Lung structure and function relation in systemic sclerosis (SSc): Application of lung densitometry Perc85 is an optimal lung density parameter confirming a lung parenchymal structure-function relation in SSc. European Journal of Radiology, 01/22/2015.

Nailfold Capillary Microscopy

Capillary loss on nailfold capillary microscopy is associated with mortality in systemic sclerosis (SSc). The avascular score was associated with higher risk of death and may be a useful tool in prognostic evaluation of SSc. PubMed, Clin Rheumatol, 10/10/2017.

Nitric Oxide

Application of nitric oxide measurements in clinical conditions beyond asthma. So far, application of FeNO measurement in the management of systemic sclerosis (SSc) and interstitial lung disease (ILD) patients is not supported by data, whereas increased CalvNO concentrations could be used to non-invasively assess the extent of ILD in conditions including SSc. Eur Clin Respir Jv, 2015.

Pulmonary Function Test (PFT)

Pulmonary function tests. Pulmonary function tests are a group of tests that measure how well the lungs take in and release air and how well they move gases such as oxygen from the atmosphere into the body's circulation. MedLine Plus.

Right Heart Catheterization (RHC)

"PH cannot be diagnosed without right heart catheterization.”

The early symptoms of pulmonary arterial hypertension (PAH), such as dyspnoea, dizziness, and fatigue, are often mild and are common to many other conditions. At rest there are often no symptoms and no apparent signs of illness.

Six-Minute Walk Test (6MWT)

Factors relating to impaired stroke volume during the 6–minute walk test in patients with systemic sclerosis. Impaired stroke volume in patients with systemic sclerosis was observed at rest and during exercise, and the factors relating to the cardiac response seemed to be pulmonary function and the extent of pulmonary hypertension. PubMed, Clin Exp Rheumatol, 07/22/2016. (Also see Pulmonary Hypertension Diagnosis)

Six-Minute Walk Test in Scleroderma-Associated Pulmonary Arterial Hypertension: Are We Counting What Counts? The above limitations need to be resolved through further study before the routine use of 6MWT can be recommended for the management of SSc-PAH. The Journal of Rheumatology vol. 36 no. 2 216-218.

Diagnosis of Pulmonary Fibrosis

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

Video Overview
Diagnostic Algorithm
Exercise Echo
Functional Classification
Markers for PH in Scleroderma
P-Wave Amplitude
Right Heart Catheterization
Risk Factors
Six-Minute Walk
Go to Oxygen Therapy

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