scholarly journals Assessment of recent evidence for the management of patients with systemic sclerosis-associated interstitial lung disease: a systematic review

2021 ◽  
Vol 7 (1) ◽  
pp. 00235-2020
Author(s):  
Anna-Maria Hoffmann-Vold ◽  
Toby M. Maher ◽  
Edward E. Philpot ◽  
Ali Ashrafzadeh ◽  
Oliver Distler
Keyword(s):  
Systematic Review ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Disease Progression ◽  
Immunosuppressive Therapy ◽  
Pulmonary Function Tests ◽  
Function Tests

This systematic review summarises current evidence to help guide treatment decisions for patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD). A systematic search of the literature (January 2012 to April 2018), including grey literature (searched between 1992 and 2011), was conducted using multiple electronic databases. Guidelines, meta-analyses, randomised controlled trials and observational studies reporting on risk stratification, screening, diagnosis, treatment and management outcomes for patients with SSc-ILD were included. A quality assessment of the included evidence was undertaken.In total, 2464 publications were identified and 280 included. Multiple independent risk factors for ILD in patients with SSc were identified, including older age, male sex and baseline pulmonary function. High-resolution computed tomography (HRCT) has been used for characterising ILD in patients with SSc, and pulmonary function tests are a key adjunctive component in the diagnostic and monitoring pathway. The clinical value of biomarkers relating to SSc-ILD diagnosis or assessment for disease progression is unknown at present. Immunosuppressive therapy (monotherapy or combined therapy) is the current standard of care for SSc-ILD; long-term evidence for effective and safe treatment of SSc-ILD is limited.Identification of patients at risk for SSc-ILD remains challenging. HRCT and pulmonary function tests are key to diagnosing and monitoring for disease progression. Although immunosuppressive therapy is considered current first-line treatment, it is partly associated with adverse effects and long-term follow-up evidence is limited. Novel therapies and biomarkers should be further explored in well-controlled clinical studies.

scholarly journals Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease

2018 ◽  
Vol 27 (148) ◽  
pp. 170102 ◽  
Author(s):  
Melissa Caron ◽  
Sabrina Hoa ◽  
Marie Hudson ◽  
Kevin Schwartzman ◽  
Russell Steele
Keyword(s):  
Systematic Review ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Validation Studies ◽  
Pulmonary Function Tests ◽  
Outcome Studies ◽  
Surrogate Markers ◽  
Function Tests

Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). We performed a systematic review to characterise the use and validation of pulmonary function tests (PFTs) as surrogate markers for systemic sclerosis-associated interstitial lung disease (SSc-ILD) progression.Five electronic databases were searched to identify all relevant studies. Included studies either used at least one PFT measure as a longitudinal outcome for SSc-ILD progression (i.e. outcome studies) and/or reported at least one classical measure of validity for the PFTs in SSc-ILD (i.e. validation studies).This systematic review included 169 outcome studies and 50 validation studies. Diffusing capacity of the lung for carbon monoxide (DLCO) was cumulatively the most commonly used outcome until 2010 when it was surpassed by forced vital capacity (FVC). FVC (% predicted) was the primary endpoint in 70.4% of studies, compared to 11.3% for % predicted DLCO. Only five studies specifically aimed to validate the PFTs: two concluded that DLCO was the best measure of SSc-ILD extent, while the others did not favour any PFT. These studies also showed respectable validity measures for total lung capacity (TLC).Despite the current preference for FVC, available evidence suggests that DLCO and TLC should not yet be discounted as potential surrogate markers for SSc-ILD progression.

Pulmonary Function Tests and 5-Year Survival in Patients Systemic Sclerosis and Interstitial Lung Disease (ILD)

CHEST Journal ◽  
2012 ◽  
Vol 142 (4) ◽  
pp. 433A
Author(s):  
Maria Otaola ◽  
Silvia Quadrelli ◽  
Gabriela Tabaj ◽  
Raquel Aguirre ◽  
Luciana Molinari ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Function Tests

Interstitial lung disease in systemic sclerosis quantification of disease classification and progression with high-resolution computed tomography: An observational study

2021 ◽  
pp. 239719832098537
Author(s):  
Johan Clukers ◽  
Maarten Lanclus ◽  
Dennis Belmans ◽  
Cedric Van Holsbeke ◽  
Wilfried De Backer ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Severe Disease ◽  
Quantitative Computed Tomography ◽  
High Resolution Computed Tomography ◽  
Function Tests

Introduction: Systemic sclerosis–associated interstitial lung disease accounts for up to 20% of mortality in these patients and has a highly variable prognosis. Functional respiratory imaging, a quantitative computed tomography imaging technique which allows mapping of regional information, can provide a detailed view of lung structures. It thereby shows potential to better characterize this disease. Purpose: To evaluate the use of functional respiratory imaging quantitative computed tomography in systemic sclerosis–associated interstitial lung disease staging, as well as the relationship between short-term changes in pulmonary function tests and functional respiratory imaging quantitative computed tomography with respect to disease severity. Materials and methods: An observational cohort of 35 patients with systemic sclerosis was retrospectively studied by comparing serial pulmonary function tests and in- and expiratory high-resolution computed tomography over 1.5-year interval. After classification into moderate to severe lung disease and limited lung disease (using a hybrid method integrating quantitative computed tomography and pulmonary function tests), post hoc analysis was performed using mixed-effects models and estimated marginal means in terms of functional respiratory imaging parameters. Results: At follow-up, relative mean forced vital capacity percentage change was not significantly different in the limited (6.37%; N = 13; p = 0.053) and moderate to severe disease (−3.54%; N = 16; p = 0.102) groups, respectively. Specific airway resistance decreased from baseline for both groups. (Least square mean changes −25.11% predicted ( p = 0.006) and −14.02% predicted ( p = 0.001) for limited and moderate to severe diseases.) In contrast to limited disease from baseline, specific airway radius increased in moderate to severe disease by 8.57% predicted ( p = 0.011) with decline of lower lobe volumes of 2.97% predicted ( p = 0.031). Conclusion: Functional respiratory imaging is able to differentiate moderate to severe disease versus limited disease and to detect disease progression in systemic sclerosis.

scholarly journals Lung ultrasound B-lines and serum KL-6 correlate with the severity of idiopathic inflammatory myositis-associated interstitial lung disease

Rheumatology ◽  
2019 ◽  
Vol 59 (8) ◽  
pp. 2024-2029 ◽  
Author(s):  
Yukai Wang ◽  
Shaoqi Chen ◽  
Jianqun Lin ◽  
Xuezhen Xie ◽  
Shijian Hu ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Lung Ultrasound ◽  
Pulmonary Function Tests ◽  
Forced Expiratory Volume ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Function Tests ◽  
B Lines

Abstract Objective Idiopathic inflammatory myositis-associated interstitial lung disease (IIM-ILD) significantly increases morbidity and mortality. Lung ultrasound B-lines and Krebs von den Lungen-6 (KL-6) are identified as new sonographic and serum markers of ILD, respectively. The aim of our work was to assess the role of B-lines and KL-6 as markers of the severity of IIM-ILD. For this purpose, the correlation among B-lines score, serum KL-6 levels, high-resolution CT (HRCT) score, and pulmonary function tests were investigated in IIM-ILD patients. Methods Thirty-eight patients with IIM-ILD underwent chest HRCT scans, lung ultrasound and pulmonary function tests (independently performed within 1 week) examination. To assess severity and extent of ILD at HRCT, the Warrick score was used. The B-lines score denoting the extension of ILD was calculated by summing the number of B-lines on a total of 50 scanning sites. Serum KL-6 levels (U/ml) was measured by chemiluminescent enzyme immunoassay. Results A significant correlation was found between the B-lines score and serum KL-6 levels (r = 0.43, P < 0.01), and between the Warrick score and serum KL-6 levels (r = 0.45, P < 0.01). A positive correlation between B-lines score and the Warrick score (r = 0.87, P < 0.0001) was also confirmed. Both B-lines score and KL-6 levels inversely correlated to diffusion capacity for carbon monoxide (r = −0.77, P < 0.0001 and r = −0.42, P < 0.05, respectively) and total lung capacity (r = −0.73, P < 0.0001 and r = −0.36, P < 0.05, respectively). Moreover, B-lines correlated inversely with forced vital capacity (r = −0.73, P < 0.0001), forced expiratory volume in 1 s (r = −0.69, P < 0.0001). Conclusion B-lines score and serum KL-6 levels correlate with HRCT findings and pulmonary function tests, supporting their use as measures of IIM-ILD severity.

scholarly journals EP29 An evaluation of the use of baseline pulmonary function tests in the detection of interstitial lung disease in patients newly diagnosed with rheumatoid arthritis

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Ashraful Haque ◽  
Rachael Kilding ◽  
Ruth Smith ◽  
Sameena Khalid ◽  
Robert Sandler ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Newly Diagnosed ◽  
Tertiary Centre ◽  
Function Tests ◽  
Low Sensitivity ◽  
New Diagnosis

Abstract Background Interstitial lung disease (ILD) is a serious extra-articular manifestation of rheumatoid arthritis (RA). Risk factors include smoking, the presence of rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibodies (CCP). Pulmonary function tests (PFT) show reduced carbon monoxide diffusion capacity (DLCO) early and reduced forced vital capacity (FVC) later in disease. HRCT is the gold standard diagnostic test while chest X-ray (CXR) has low sensitivity. PFT are routinely performed in the majority of RA patients at baseline at our tertiary centre. The aim of this study was to evaluate the frequency of abnormal PFT, specificity for ILD and influence on subsequent decision-making in patients newly diagnosed with RA. Methods A retrospective analysis was undertaken of patients with a new diagnosis of RA between January 2016 and December 2017. Patients meeting the ACR (2010) criteria for RA, with baseline PFT data available were included. Clinic letters and the hospital electronic records were used to obtain the data. Results 139 patients were included in the data analysis (Table 1). 23 patients had DLCO <70% predicted, while 7 patients had an FVC <80% predicted. Patients with abnormal PFT were more likely to be older, female, seropositive and to have smoked. Of the patients with DLCO <70%, CXR was abnormal in 6 patients with changes suggesting ILD in 2 patients. 13 patients had HRCT and 7/13 patients had evidence of ILD and 6/13 patients had significant emphysema on CXR or HRCT. 1 patient with DLCO of 82% had changes of ILD on a CT scan organised for another reason. Methotrexate was commenced in 19/23 patients with DLCO<70% and discontinued in 2 patients for respiratory reasons. Conclusion This evaluation suggests baseline PFT are more sensitive than baseline CXR in detecting ILD but that a DLCO <70% is not specific for this diagnosis. The abnormal PFT lead to HRCT being requested in 13/24 patients, of whom 7 had ILD which had not been identified by CXR in 5 patients. Baseline PFT are also useful as a reference point in patients who go on to develop respiratory symptoms at a later point in their illness. Disclosures A. Haque None. R. Kilding None. R. Smith None. S. Khalid None. R. Sandler None. M. Cox None. T. Hendry None. A. Flores-martin None. K. Lindop None. J. Maxwell None.

PULMONARY FUNCTION TESTS IN INTERSTITIAL LUNG DISEASE

2001 ◽  
Vol 22 (4) ◽  
pp. 715-750 ◽  
Author(s):  
Esam H. Alhamad ◽  
Joseph P. Lynch ◽  
Fernando J. Martinez
Keyword(s):  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Function Tests
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