Radiographic fibrosis score predicts survival in systemic sclerosis‐associated interstitial lung disease

Takei, Reoto; Arita, Machiko; Kumagai, Shogo; Ito, Yuhei; Tokioka, Fumiaki; Koyama, Takashi; Saito, Rintaro; Nishimura, Keisuke; Tokumasu, Hironobu; Ishida, Tadashi

doi:10.1111/resp.13175

Cited by 34 publications

(42 citation statements)

References 25 publications

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“…Quantifying the extent of fibrosis has yielded important information including prediction of pulmonary function decline 21 and risk for mortality 5,22,23,24 . Advances in quantitative measurements of fibrosis 25 has allowed HRCT to become a valuable tool for detecting response to treatment.…”

Section: Hrctmentioning

confidence: 99%

Management of systemic sclerosis-associated interstitial lung disease

Roofeh

Jaafar

Vummidi

et al. 2019

Current Opinion in Rheumatology

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Purpose of review-To review the recently published data and provide a practical overview for management of systemic sclerosis-interstitial lung disease (SSc-ILD). Recent Findings-Published evidence shows considerable practitioner variability in screening patients for ILD. Recent published data supports use of cyclophosphamide or mycophenolate mofetil as first line treatment of SSc-ILD. For patients not responding to first line therapies, consideration is given to rituximab as rescue therapy. Recent trials of hematopoietic autologous stem cell transplantation have demonstrated benefit in patients with progressive SSc-ILD. Antifibrotic agents are approved in idiopathic pulmonary fibrosis; studies with anti-fibrotics are underway for SSc-ILD. Summary-The specter of rapidly progressive lung disease requires clinicians to risk stratify patients according to known predictors for progression and rigorously monitor for symptoms and advancing disease. The above-mentioned therapies promise improved efficacy and favorable side effect profiles compared to cyclophosphamide.

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Section: Hrctmentioning

confidence: 99%

Management of systemic sclerosis-associated interstitial lung disease

Roofeh

Jaafar

Vummidi

et al. 2019

Current Opinion in Rheumatology

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“…Takei et al could demonstrate in confirmation of known evidence (11,12) that a high fibrosis extension score is associated with an increased risk of death, and further independent from HRCT pattern (13). In this context reduced HRCT might qualify for the visual scoring of lung fibrosis extension as no difference to standard HRCT was found and could be used as a follow-up examination in monitoring and therapy management of patients with SSc-ILD.…”

Section: Discussionmentioning

confidence: 61%

“…Different studies have shown that the extent and severity of ILD can be adequately assessed using visual scales (3,6,8). Further the extension of fibrosis is evidently associated with an increased risk of death (11)(12)(13).…”

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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“…Mortality risk has been reported to be higher in patients with a disease extent of at least 20% on HRCT (10-year survival was 43% versus 67%, respectively, in patients with disease extent above versus below the 20% threshold) 60 . Similarly, a high fibrosis score on HRCT (based on the extent of reticulation and honeycombing) has been associated with increased mortality 61 . Large esophageal diameters are associated with increased ILD severity and decreased DLCO 48 .…”

Section: Prognosismentioning

confidence: 99%

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Chung

Walker

Hobbs

2020

JoVE

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Early diagnosis of systemic sclerosis-related interstitial lung disease (SSc-ILD) is important to enable treatment to be administered with minimal delay. However, diagnosing SSc-ILD is challenging because key symptoms are non-specific. Highresolution computed tomography (HRCT) of the chest is recognized as a sensitive imaging method for diagnosing and assessing SSc-ILD. Exposure of patients to ionizing radiation may be considered as a limitation, although methodological steps may be taken to moderate this. We present practical recommendations for performing HRCT scans and interpreting the results. Key features of SSc-ILD on HRCT include a non-specific interstitial pneumonia (NSIP) pattern with peripheral ground-glass opacities and extensive traction bronchiectasis. Despite similarities between SSc-ILD and idiopathic pulmonary fibrosis (IPF), HRCT can be used to differentiate between these conditions: in SSc-ILD compared with IPF, there is a greater proportion of ground-glass opacity and fibrosis is less coarse. A dilated, air-filled esophagus with diameter >10 mm, suggestive of esophageal dysmotility is commonly seen in SSc-ILD. Pulmonary artery size greater than the adjacent ascending aorta suggests coexistent pulmonary hypertension. Nodules must be monitored due to the increased risk of lung cancer. A large extent of disease on HRCT (≥20%) or a high fibrosis score suggests an increased risk of mortality. HRCT is central to diagnosing SSc-ILD, and serial assessments can be helpful in monitoring disease progression or treatment response.

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Radiographic fibrosis score predicts survival in systemic sclerosis‐associated interstitial lung disease

Abstract: A high radiographic fibrosis score was a poor prognostic factor in SSc-ILD. More widespread fibrosis was associated with an increased risk of death, independent of HRCT pattern.

Cited by 34 publications

References 25 publications

Management of systemic sclerosis-associated interstitial lung disease

Management of systemic sclerosis-associated interstitial lung disease

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

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

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