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Pulmonary function tests: high rate of false negatives in the early detection and screening of scleroderma interstitial lung disease


Suliman, Yossra A; Dobrota, Rucsandra; Huscher, Dörte; Nguyen-Kim, Thi D L; Maurer, Britta; Jordan, Suzana; Speich, Rudolf; Frauenfelder, Thomas; Distler, Oliver (2015). Pulmonary function tests: high rate of false negatives in the early detection and screening of scleroderma interstitial lung disease. Arthritis and Rheumatology, 67(12):3256-3261.

Abstract

Objective Validated methods for screening and early diagnosis of scleroderma-associated interstitial lung disease (SSc-ILD) are needed. In this study, we evaluated the performance of pulmonary function tests (PFTs) compared to high resolution computer tomography of the chest (HRCT) for the detection of SSc-ILD in clinical practice and aimed to identify predictors of functionally-occult, but HRCT-significant lung involvement. Methods Prospectively enrolled SSc patients were assessed following the EUSTAR standards, including PFTs and HRCT. The images were blindly evaluated by two experienced radiologists. The performance parameters of PFTs for the diagnosis of SSc-ILD were calculated. Predictors of significant ILD in HRCT in patients with normal FVC were identified through logistic regression. Results 64/102 (63.0%) patients showed significant ILD on HRCT, while only 27/102 (26.0%) had an FVC<80% and 54/102 (53.0%) had a decrease in at least one PFT. 40/64 (62.5%) patients with significant ILD on HRCT had a normal FVC, translating into a high false negative rate. Notably, 5/40 (6.0%) of patients with normal FVC had severe, functionally-occult lung fibrosis, 2 of these having all PFTs in normal limits. The false negative cases had more frequently anti-Scl-70 antibodies and diffuse SSc and less frequently anti-centromere antibodies (ACA) compared to ILD-free patients. Conclusion The derived evidence-based data reveal a high risk of missing the detection of significant SSc-ILD when relying solely on PFTs. More comprehensive screening algorithms for the early detection are warranted. Particularly, additional imaging investigations should be considered in ACA negative patients with normal FVC for the early detection of SSc-ILD. This article is protected by copyright. All rights reserved.

Abstract

Objective Validated methods for screening and early diagnosis of scleroderma-associated interstitial lung disease (SSc-ILD) are needed. In this study, we evaluated the performance of pulmonary function tests (PFTs) compared to high resolution computer tomography of the chest (HRCT) for the detection of SSc-ILD in clinical practice and aimed to identify predictors of functionally-occult, but HRCT-significant lung involvement. Methods Prospectively enrolled SSc patients were assessed following the EUSTAR standards, including PFTs and HRCT. The images were blindly evaluated by two experienced radiologists. The performance parameters of PFTs for the diagnosis of SSc-ILD were calculated. Predictors of significant ILD in HRCT in patients with normal FVC were identified through logistic regression. Results 64/102 (63.0%) patients showed significant ILD on HRCT, while only 27/102 (26.0%) had an FVC<80% and 54/102 (53.0%) had a decrease in at least one PFT. 40/64 (62.5%) patients with significant ILD on HRCT had a normal FVC, translating into a high false negative rate. Notably, 5/40 (6.0%) of patients with normal FVC had severe, functionally-occult lung fibrosis, 2 of these having all PFTs in normal limits. The false negative cases had more frequently anti-Scl-70 antibodies and diffuse SSc and less frequently anti-centromere antibodies (ACA) compared to ILD-free patients. Conclusion The derived evidence-based data reveal a high risk of missing the detection of significant SSc-ILD when relying solely on PFTs. More comprehensive screening algorithms for the early detection are warranted. Particularly, additional imaging investigations should be considered in ACA negative patients with normal FVC for the early detection of SSc-ILD. This article is protected by copyright. All rights reserved.

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10 citations in Web of Science®
9 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic and Policlinic for Internal Medicine
04 Faculty of Medicine > University Hospital Zurich > Clinic for Diagnostic and Interventional Radiology
04 Faculty of Medicine > University Hospital Zurich > Rheumatology Clinic and Institute of Physical Medicine
Dewey Decimal Classification:610 Medicine & health
Date:28 August 2015
Deposited On:09 Sep 2015 15:24
Last Modified:28 May 2016 08:04
Publisher:Wiley-Blackwell Publishing, Inc.
ISSN:2326-5205
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1002/art.39405
PubMed ID:26316389

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