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)

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.

Diagnosis and treatment of connective tissue disease (CTD)-associated interstitial lung disease (ILD). A careful and systematic approach to diagnosis in patients with ILD may reveal an unrecognized CTD or evidence of autoimmunity in those previously believed to have idiopathic ILD. PubMed, Chest, 2013 Mar; 143(3):814-24.

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 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.

Screening for interstitial lung disease in systemic sclerosis: performance of high-resolution CT (HRCT) with limited number of slices: a prospective study. This HRCT protocol reliably detects even mild SSc-ILD in clinical practice, with a much lower radiation dose compared with standard whole-chest HRCT. PubMed, Ann Rheum Dis, 2014 Dec;73(12):2069-73.

Enteric-coated mycophenolate sodium for progressive systemic sclerosis (Ssc)-a prospective open-label study with CT histography for monitoring of pulmonary fibrosis. CT histography appears to be a sensitive method for the detection of progression of pulmonary fibrosis and therefore should be considered for further studies. PubMed, Clin Rheumatol, 2013 Jan 10.

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, 2013 May 6. (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.


Sonographic assessment of interstitial lung disease in patients with rheumatoid arthritis, systemic sclerosis and systemic lupus erythematosus. Transthoracic ultrasound of the lung might be a sensitive non-invasive tool to observe early stage interstitial lung disease in rheumatic diseases. Clinical and Experimental Rheumatology, 01/29/2015.

Ultrasound (US) in systemic sclerosis (SSc). A multi-target approach from joint to lung. Discussion of the current evidence supporting ultrasound relevance and applications in daily clinical practice. PubMed, Clin Rheumatol, 02/18/2014.

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.

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, 2012 Sep 5.

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