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)

Nailfold Videocapillaroscopy (NVC) is a Useful Tool to Recognize Definite Forms of Systemic Sclerosis (SSc) and Idiopathic Inflammatory Myositis in Interstitial Lung Disease Patients (ILD). NVC should be considered in the diagnostic assessment of ILD patients regardless of the presence of Raynaud’s Phenomenon. PubMed, Diagnostics (Basel), 2020 Apr 25;10(5). (Also see Nailfold Videocapillaroscopy)

Defining genetic risk factors for scleroderma-associated interstitial lung disease (SSc-ILD) : IRF5 and STAT4 gene variants are associated with scleroderma while STAT4 is protective against scleroderma-associated interstitial lung disease. Further work is required to understand the genetic basis of lung fibrosis in association with scleroderma. PubMed, Clin Rheumatol, 01/08/2020. (Also see Genetics)

Genetic Interactions Affect Lung Function in Patients with Systemic Sclerosis. We identified a three-gene network comprising WNT5A, RBMS3 and MSI2, which in combination influenced multiple pulmonary pathology measures. PubMed, G3 (Bethesda), 11/06/2019. (Also see Genetics)

Detection and classification of systemic sclerosis-related interstitial lung disease: a review. Classification of SSc-ILD has shifted to a personalized approach that considers an individual patient's probability of progressive disease through identification of risk factors. PubMed, Curr Opin Rheumatol, 08/13/2019.

Augmented concentrations of CX3CL1 are associated with interstitial lung disease in systemic sclerosis (SSc–ILD). The CX3CR1/CX3CL1-biological axis may be involved in recruiting antibody secreting plasma cells to SSc lungs, thereby contributing to the immune–mediated pathobiology of SSc–ILD. PubMed, PLoS One, 2018 Nov 20;13(11):e0206545.

Interstitial lung disease (ILD) in patients with mixed connective tissue disease (MCTD): pilot study on predictors of lung involvement. The association of easily elicited historical and clinical features of MCTD with subtle, but worrisome, pulmonary pathology carries the promise of sensitising the unsuspecting clinician about the entity of ILD in MCTD. PubMed, Clin Exp Rheumatol. 2018 Jul-Aug;36(4):648-651. (Also see Connective Tissue Disease)

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.

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)

High-resolution computed tomography (HRCT) Has Key Role Detecting Lung Disease in Systemic Sclerosis. The first evidence-based consensus statements for systemic sclerosis-associated interstitial lung disease recommend use of HRCT for screening and diagnosis. Medscape, 02/04/2020.

Performance of a new quantitative computed tomography index for interstitial lung disease (ILD) assessment in systemic sclerosis (SSc). This new composite CT index for SSc-ILD assessment could be sufficiently sensitive for capturing early lung density changes in visually ILD-free patients. PubMed, Sci Rep, 2019 Jul 1;9(1):9468.

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.

Histology of Pulmonary and Bronchiolar Disorders in Connective Tissue Diseases (CTD). In this review we describe the patterns of lung disease associated with common CTDs, lung disease in pediatric CTD patients, and newly recognized conditions. PubMed, Semin Respir Crit Care Med. 2019 Apr;40(2):147-158. (Also see Connective Tissue Disease)

Pulmonary Function Tests

Increased prevalence of small airways dysfunction in patients with systemic sclerosis (SSc) as determined by impulse oscillometry. A full assessment of lung function in SSc patients should include impulse oscillometry as a complementary technique, due to potential clinical and therapeutic implications. PubMed, Rheumatology (Oxford), 08/22/2019.

Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease (SSc-ILD). Despite the current preference for forced vital capacity, available evidence suggests that DLCO and total lung capacity should not yet be discounted as potential surrogate markers for SSc-ILD progression. PubMed, Eur Respir Rev, 2018 May 15;27(148).

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.


Development and Assessment of a Novel Lung Ultrasound (LUS) Interpretation Criteria for the Detection of Interstitial Lung Disease in Systemic Sclerosis (SSC-ILD). We developed a novel LUS examination technique and interpretation criteria that are highly sensitive and specific for SSc-ILD detection in an SSc cohort. PubMed, Arthritis Care Res (Hoboken), 05/31/2020.

A simplified lung ultrasound (LUS) for the diagnosis of interstitial lung disease in connective tissue disease: a meta–analysis. We found a modified and simplified method of LUS, by scanning 14 lung intercostal spaces in a short time, which had a very high sensitivity and specificity. PubMed, Arthritis Res Ther, 2019 Apr 11;21(1):93. (Also see Connective Tissue Disease)

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

Biomarkers in Systemic Sclerosis-Associated Interstitial Lung Disease: Review of the Literature. The purpose of this article is to describe the main biomarkers thought to be of interest with clinical value in the diagnosis and prognosis of SSc-interstitial lung disease. PubMed, Rheumatology (Oxford), 2019 Sep 1;58(9):1534-1546.

Systemic Sclerosis Associated Interstitial Lung Disease: A Comprehensive Overview. This review aims to provide a comprehensive overview of all key aspects of SSc-ILD. PubMed, Semin Respir Crit Care Med, 2019 Apr;40(2):208-226.

High Baseline Serum Clara Cell 16 kDa Predicts Subsequent Lung Disease Worsening in Systemic Sclerosis (SSc). High baseline serum CC16 predicts lung disease worsening in patients with SSc. PubMed, J Rheumatol, 2018 Feb;45(2):242-247.

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