Pulmonary Fibrosis Diagnosis

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
High Resolution Computed Tomography
Induced Sputum and Bronchoalveolar Lavage
Lung Biopsy
Pulmonary Function Tests
Walking and Stress Tests
Pulmonary Fibrosis Antibodies


All systemic sclerosis patients should have regular screening for both pulmonary fibrosis and pulmonary hypertension. Exams may include pulmonary function test (PFT), Doppler ECHO, high-resolution computed tomography (HRCT), right heart catheterization (RHC), and six-minute walk test. (Also see What is Scleroderma?, Pulmonary Fibrosis, and Pulmonary Hypertension)

Is It Idiopathic Pulmonary Fibrosis or Not? The early and correct diagnosis is hampered by mimickers that include nonspecific interstitial pneumonitis, chronic hypersensitivity pneumonitis, and fibrotic sarcoidosis. PubMed Journals, J Am Board Fam Med, 31 (1), 151-162. 1 2018 2018 Jan-Feb.

Difficulties in diagnosis of systemic sclerosis-related interstitial lung disease (SSc-ILD). The diagnosis of SSc-ILD could maybe be made sooner if a capillaroscopy was performed earlier, according to the current criteria of early SSc. PMC, Respirol Case Rep, 2015 Sep; 3(3): 99–101. (Also see Nailfold Capillaroscopy)

Investigation of Lung Involvement in Connective Tissue Disorders (CTD). The investigation of lung involvement in CTDs requires adequate skills in the ambit of a multidisciplinary approach and an extended spectrum of diagnostic tools. PubMed, Respiration, 06/26/2015. (Also see Connective Tissue Disease)

Interstitial Lung Disease in Scleroderma. The diagnosis of interstitial lung disease hinges on careful clinical evaluation and pulmonary function tests and high-resolution computed tomography. Rheumatic Disease Clinics of North America, 03/05/2015.

Medical Tests: Pulmonary. Diagnostic tests for pulmonary fibrosis include CAT scan, echocardiogram, pulmonary function test, chest x-ray, bronchoscopy, and bronchoalveolar lavage. ISN.

High Resolution Computed Tomography (HRCT)

Interstitial lung disease (ILD) in systemic sclerosis: progress in screening and early diagnosis. Prospective cohort data indicated poor performance of pulmonary function tests as a stand–alone method for ILD screening, although lung HRCT appeared promising, radiation is an issue. PubMed, Curr Opin Rheumatol, 2016 Nov;28(6):613-8.

High False Negative Rate in Detecting Scleroderma (SSc) Lung Disease Associated With Pulmonary Function Tests. These findings indicate that high–resolution computed tomography scans, even when conducted by the most skilled radiologists, may lead to false negatives in detecting SSc of the lung. Scleroderma News, 09/21/2015.

Thoracic Scleroderma Imaging. Patients with suggested or known diagnosis of systemic sclerosis should undergo a CAT Scan. Medscape.

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)

Lung Biopsy

Open lung biopsy. An open lung biopsy is surgery to remove a small piece of tissue from the lung. The sample is then examined for cancer, infection, or lung disease. MedLinePlus.

Histopathology of Lung Disease in the Connective Tissue Diseases (CTD). This article will cover the pulmonary pathologies seen in rheumatoid arthritis, systemic sclerosis, myositis, systemic lupus erythematosus, Sjögren syndrome, and mixed CTD. Rheumatic Disease Clinics of North America, 03/06/2015. (Also see Connective Tissue Disease)

Pulmonary Function Tests

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.


Ultrasound signs of pulmonary fibrosis (PF) in systemic sclerosis as timely indicators for chest computed tomography. Thoracic ultrasound imaging of pleural/subpleural structures can detect ultrasonographic signs of initial PF prior to the onset of respiratory symptoms and function test abnormalities. PubMed, Scand J Rheumatol, 2015;44(5):389-98.

Walking and Stress Tests

Six Minute Walk Test (6MWT). The original purpose of the six minute walk was to test exercise tolerance in chronic respiratory disease and heart failure. The test has since been used as a performance-based measure of functional exercise capacity in other populations including healthy older adults, people undergoing knee or hip arthroplasty, fibromyalgia, and scleroderma. American College of Rheumatology.

Factors associated with the 6–minute walk distance (6MWD) in patients with systemic sclerosis (SSc). During SSc, the 6MWD is independently associated with initial HR and HR variation; suggesting that pulmonary vasculopathy may have a greater impact than parenchymal involvement on functional limitation. PubMed, Arthritis Res Ther, 2017 Dec 15;19(1):279.

Pulmonary Fibrosis Antibodies and Biomarkers

Interstitial lung disease (ILD) in systemic sclerosis (SSc): progress in screening and early diagnosis. Lung HRCT appeared promising, but radiation is an issue. Promising biomarker data indicate the possibility of new ILD screening algorithms in SSc. PubMed, Curr Opin Rheumatol, 07/06/2016.

New Potential Serum Biomarker for Interstitial Lung Disease (ILD) Associated With Systemic Sclerosis. The research team concluded that CC16 serum levels can be considered a potential biomarker for ILD in systemic sclerosis patients. Scleroderma News, 08/12/2015.

Interstitial lung disease: is interstitial lung disease the same as scleroderma lung disease? Despite differences in clinical presentation and response to treatment, similarities are noted in proposed pathogenesis and putative biomarkers. Curr Opin Rheumatol.

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