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

Bronchoalveolar Lavage (BAL) in Systemic Sclerosis (SSC) Patients: a Systematic Review. Alveolitis and the increase of some cytokines/chemokines in BAL were related to more severe pulmonary disease in SSc in the majority of the studies. PubMed, Curr Rheumatol Rev, 11/12/2020. (Also see Induced Sputum and Bronchoalveolar Lavage Tests)

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. (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

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. (Also see Juvenile Scleroderma)

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, 2018 Feb;37(2):475-481.

Nitric Oxide

Rationale underlying the measurement of fractional exhaled nitric oxide (FeNO) in systemic sclerosis (SSc) patients. The findings concerning the usefulness of FeNO measurements in SSc patients are discordant, but the available papers suggest that airway/alveolar NO concentration (ANOC) is a more accurate indicator of progressive lung dysfunction. PubMed, Clin Exp Rheumatol, 02/27/2019.

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)

Usefulness of Six-Minute Walk Test (6MWT) in Systemic Sclerosis. Recent studies suggest two alternatives to the 6MWT: maximal cardiopulmonary exercise testing and DLCO testing during effort. PubMed, Clin Exp Rheumatol, Jul-Aug 2018;36 Suppl 113(4):161-167. (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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