Brief Report: Pulmonary Function Tests: High Rate of False‐Negative Results in the Early Detection and Screening of Scleroderma‐Related Interstitial Lung Disease

Suliman, Yossra A; Dobrotă, Rucsandra; Huscher, Dörte; Nguyen-Kim, Thi Dan Linh; Maurer, Britta; Jordan, Suzana; Speich, Rudolf; Frauenfelder, Thomas; Distler, Oliver

doi:10.1002/art.39405

Cited by 186 publications

(173 citation statements)

References 14 publications

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“…A patient with purely restrictive physiology will also demonstrate a normal ratio of forced expiratory volume in 1 second (FEV1) to forced vital capacity (FVC), representing the maximum proportion of vital capacity a patient can exhale in the first second of expiration. PFT should not be used in isolation to diagnose ILD as they lack specificity(15). …”

Section: Initial Approach To the Patient With Ctd-ildmentioning

confidence: 99%

Management of connective tissue diseases associated interstitial lung disease

Wallace

Vummidi

Khanna

2016

Current Opinion in Rheumatology

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Purpose of review Interstitial lung disease (ILD), though a common and often a severe manifestation of many connective tissue diseases (CTD), is challenging to manage due to its variable presentation and the relative lack of guidelines to assist the clinician. In this review, we discuss the approach to diagnosis, treatment, and monitoring patients with CTD-associated ILD, with a focus on systemic sclerosis (SSc), rheumatoid arthritis (RA), and idiopathic inflammatory myopathy (IIM). Recent findings High-resolution CT scan and pulmonary function testing can be reliably used to diagnose ILD and monitor progression, and often to determine its likely histologic subtype and severity. In SSc-ILD, randomized controlled trials show ILD stabilization with cyclophosphamide treatment; preliminary data from another randomized controlled trial demonstrates similar findings with mycophenolate. There are no robust clinical trials supporting specific treatments for RA-ILD or IIM-ILD, but rituximab in RA-ILD, and cyclophosphamide, mycophenolate and calcineurin inhibitors in IIM-ILD show promise. Summary Though ILD contributes substantially to morbidity and mortality in patients with CTD, there is minimal data to guide its management except in SSc-ILD.

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Section: Initial Approach To the Patient With Ctd-ildmentioning

confidence: 99%

Management of connective tissue diseases associated interstitial lung disease

Wallace

Vummidi

Khanna

2016

Current Opinion in Rheumatology

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“…However, currently no data are available to distinguish between progressive and stable patients when mild ILD is diagnosed. These patients might be easily overlooked and undertreated due to better pulmonary function tests (PFTs) often within normal values 24. This defines an urgent clinical need for the identification of baseline predictors of lung progression in patients with mild SSc-ILD.…”

Section: Introductionmentioning

confidence: 99%

Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model

et al. 2018

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“…Pulmonary function tests are considered the gold standard for the screening of parenchymal lung involvement. However, because lung function tests as well as chest X-ray tend to be insensitive to detect early lung involvement, high-resolution computed tomography (HRCT) plays an important role in the detection and characterization of interstitial lung disease (ILD) in patients with SSc (3)(4)(5)(6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

The impact of slice-reduced computed tomography on histogram-based densitometry assessment of lung fibrosis in patients with systemic sclerosis

Nguyen-Kim¹,

Maurer²,

Suliman³

et al. 2018

J. Thorac. Dis.

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Background: To evaluate usability of slice-reduced sequential computed tomography (CT) compared to standard high-resolution CT (HRCT) in patients with systemic sclerosis (SSc) for qualitative and quantitative assessment of interstitial lung disease (ILD) with respect to (I) detection of lung parenchymal abnormalities, (II) qualitative and semiquantitative visual assessment, (III) quantification of ILD by histograms and (IV) accuracy for the 20%-cut off discrimination. Methods: From standard chest HRCT of 60 SSc patients sequential 9-slice-computed tomography (reduced HRCT) was retrospectively reconstructed. ILD was assessed by visual scoring and quantitative histogram parameters. Results from standard and reduced HRCT were compared using non-parametric tests and analysed by univariate linear regression analyses. Results: With respect to the detection of parenchymal abnormalities, only the detection of intrapulmonary bronchiectasis was significantly lower in reduced HRCT compared to standard HRCT (P=0.039). No differences were found comparing visual scores for fibrosis severity and extension from standard and reduced HRCT (P=0.051-0.073). All scores correlated significantly (P<0.001) to histogram parameters derived from both, standard and reduced HRCT. Significant higher values of kurtosis and skewness for reduced HRCT were found (both P<0.001). In contrast to standard HRCT histogram parameters from reduced HRCT showed significant discrimination at cut-off 20% fibrosis (sensitivity 88% kurtosis and skewness; specificity 81% kurtosis and 86% skewness; cut-off kurtosis ≤26, cut-off skewness ≤4; both P<0.001). Conclusions: Reduced HRCT is a robust method to assess lung fibrosis in SSc with minimal radiation dose with no difference in scoring assessment of lung fibrosis severity and extension in comparison to standard HRCT.In contrast to standard HRCT histogram parameters derived from the approach of reduced HRCT could discriminate at a threshold of 20% lung fibrosis with high sensitivity and specificity. Hence it might be used to detect early disease progression of lung fibrosis in context of monitoring and treatment of SSc patients.Keywords: Interstitial lung disease (ILD); reduced high-resolution computed tomography of the lung (reduced HRCT of the lung); histogram parameters from reduced high-resolution computed tomography (histogram parameters from reduced HRCT); systemic sclerosis (SSc)

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Brief Report: Pulmonary Function Tests: High Rate of False‐Negative Results in the Early Detection and Screening of Scleroderma‐Related Interstitial Lung Disease

Cited by 186 publications

References 14 publications

Management of connective tissue diseases associated interstitial lung disease

Management of connective tissue diseases associated interstitial lung disease

Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model

The impact of slice-reduced computed tomography on histogram-based densitometry assessment of lung fibrosis in patients with systemic sclerosis

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