Standardization of interstitial lung disease assessment by ultrasound: results from a Delphi process and web-reliability exercise by the OMERACT ultrasound working group.

OBJECTIVES: Over the last years ultrasound has shown to be an important tool for evaluating lung involvement, including interstitial lung disease (ILD) a potentially severe systemic involvement in many rheumatic and musculoskeletal diseases (RMD). Despite the potential sensitivity of the technique the actual use is hampered by the lack of consensual definitions of elementary lesions to be assessed and of the scanning protocol to apply. Within the Outcome Measures in Rheumatology (OMERACT) Ultrasound Working Group we aimed at developing consensus-based definitions for ultrasound detected ILD findings in RMDs and assessing their reliability in dynamic images. METHODS: Based on the results from a systematic literature review, several findings were identified for defining the presence of ILD by ultrasound (i.e., Am-lines, B-lines, pleural cysts and pleural line irregularity). Therefore, a Delphi survey was conducted among 23 experts in sonography to agree on which findings should be included and on their definitions. Subsequently, a web-reliability exercise was performed to test the reliability of the agreed definitions on video-clips, by using kappa statistics. RESULTS: After three rounds of Delphi an agreement >75 % was obtained to include and define B-lines and pleural line irregularity as elementary lesions to assess. The reliability in the web-based exercise, consisting of 80 video-clips (30 for pleural line irregularity, 50 for B-lines), showed moderate inter-reader reliability for both B-lines (kappa = 0.51) and pleural line irregularity (kappa = 0.58), while intra-reader reliability was good for both B-lines (kappa = 0.72) and pleural line irregularity (kappa = 0.75). CONCLUSION: Consensus-based ultrasound definitions for B-lines and pleural line irregularity were obtained, with moderate to good reliability to detect these lesions using video-clips. The next step will be testing the reliability in patients with ILD linked to RMDs and to propose a consensual and standardized protocol to scan such patients.

Computed tomography assessment of evolution of interstitial lung disease in systemic sclerosis: Comparison of two scoring systems.

BACKGROUND: The aim of this study was to evaluate and compare the internal and external responsiveness of a computed-aided method (CaM) with a conventional visual reader-based score (CoVR) to measure interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) on high resolution computed tomography (HRCT). METHODS: Forty-five patients were evaluated in this retrospective cohort. HRCTs were collected at baseline and after 1 year. HRCT abnormalities were evaluated according to a CoVR (Warrick's method) and a quantitative CaM. Internal 1-year responsiveness was tested with a standardized mean response (SRM). Analyses of the receiver operating characteristic curves (ROCs) evaluated the sensitivity and specificity of the two methods to discriminate between clinically relevant progression and no relevant progression, using expert judgment as the gold standard (external responsiveness). RESULTS: In one year, lung involvement was stable/improved in 17 of the 45 patients (37.8%) and worsened in 28 patients (62.2%). HRCT scores changed moderately over the follow-up period. Using SFM, CaM was significantly more responsive in detecting changes due to treatment than the CoVR method. Likewise, in the analysis of the ROC curve, CaM scores showed the highest performance (AUC ROC CaM vs. CoVR, 0.951 vs. 0.807; p = 0.0065). CONCLUSION: Quantitative analysis of CaM was more responsive than the CoVR method to accurately evaluate and monitor SSc-ILD progression or response to therapy.

Ultrasound as a potential tool for the assessment of interstitial lung disease in rheumatic patients. Where are we now?

Lung ultrasound (LUS) achieved an intriguing role in the management of pulmonary involvement in patients affected by connective tissues diseases (CTDs). Few studies have been performed to support its usefulness in the evaluation of the presence and the severity of interstitial lung disease (ILD), relating it to the information obtained with chest high-resolution computed tomography (HRCT). These results open up new fields of research in order to demonstrate the utility of LUS as screening tool to evaluate ILD in CTD. The aim of this review is to provide the "state of the art" of the role of LUS in the management of ILD associated with CTD.

The Expanded Risk Score in Rheumatoid Arthritis (ERS-RA): performance of a disease-specific calculator in comparison with the traditional prediction scores in the assessment of the 10-year risk of cardiovascular disease in patients with rheumatoid arthrit.

AIMS OF THE STUDY: To assess the performance of the Expanded Risk Score in Rheumatoid Arthritis (ERS-RA), a disease-specific cardiovascular disease (CVD) prediction score, in evaluating the 10-year risk, in comparison with other traditional algorithms in patients with rheumatoid arthritis (RA). METHODS: Consecutive RA patients, aged 40-75 years, without established CVD, were included. We calculated the disease-specific ERS-RA and four traditional CVD prediction scores: the modified Systematic Coronary Risk Evaluation (mSCORE), the Framingham Risk Score using body mass index (FRS BMI), the calculator developed by the American College of Cardiology / American Heart Association in 2013 (ACC/AHA 2013) and the QRISK3. Subjects also underwent ultrasound assessment of the carotid arteries. The presence of a carotid intima-media thickness (CIMT) >0.90 mm or of carotid plaques identified the high-risk patients. RESULTS: Of the 84 patients evaluated, 33 (39.3%), 16 (19.0%), 24 (28.6%), 25 (29.8%) and 33 (39.3%) subjects were defined as having high CVD risk according to ACC/AHA 2013, mSCORE, FRS BMI, QRISK3 and ERS-RA, respectively. Compared with the ultrasound results, all the areas under the receiver operating characteristic curves (AUC-ROC) showed good discrimination properties (0.848 - FRS BMI, 0.816 - mSCORE, 0.828 - ACC/AHA 2013, 0.844 - QRISK3, 0.869 - ESR-RA). Comparison of the AUC-ROCs did not show that discriminative ability for detecting subclinical atherosclerotic damage was improved with ESR-RA. CONCLUSIONS: Using a surrogate marker of subclinical atherosclerotic organ damage as indicator of CVD burden, the newly ERS-RA risk score that incorporates specific aspects of RA performs as well as ACC/AHA 2013, mSCORE, FRS BMI and QRISK3 estimators.

Ultrasound in the assessment of pulmonary fibrosis in connective tissue disorders: correlation with high-resolution computed tomography.

OBJECTIVE: To investigate the correlation between ultrasound (US) B-lines and high-resolution computed tomography (HRCT) findings in the assessment of pulmonary fibrosis (PF) in patients with connective tissue disorders (CTD). METHODS: Thirty-four patients with a diagnosis of CTD were included. Each patient underwent clinical examination, pulmonary function test (PFT), chest HRCT, and lung US by an experienced radiologist or rheumatologist. A second rheumatologist carried out US examinations to assess interobserver agreement. In each patient, US B-line lung assessment including 50 intercostal spaces (IS) was performed. For the anterior and lateral chest, the IS were the second to the fifth along the parasternal, mid-clavicular, anterior axillary, and medial axillary lines (the left fifth IS of the anterior and lateral chest was not performed because of the presence of the heart, which limits lung visualization). For the posterior chest, the IS assessed were the seventh to the eighth along the posterior-axillary and subscapular lines. The second to eighth IS were assessed in the paravertebral line. In each IS, the number of US B-lines under the transducer was recorded, summed, and graded according to the following semiquantitative scoring: grade 0 = normal (< 10 B-lines); grade 1 = mild (11 to 20 B-lines); grade 2 = moderate (21 to 50 B-lines); and grade 3 = marked (> 50 B-lines). RESULTS: A total of 1700 IS in 34 patients were assessed. A significant linear correlation was found between the US score and the HRCT score (p < 0.001; correlation coefficient ρ = 0.875). A positive correlation was found between US B-line assessments and values of DLCO (p = 0.014). Both κ values and overall percentages of interobserver agreement showed excellent agreement. CONCLUSION: Our study demonstrates that US B-line assessment may be a useful and reliable additional imaging method in the evaluation of PF in patients with CTD.

Interreader reliability in assessment of nailfold capillary abnormalities by beginners: pilot study of an intensive videocapillaroscopy training program.

OBJECTIVE: To test the learning curve of rheumatologists with different experience in videocapillaroscopy (VCP) attending an intensive training program focused on interpretation of the main capillary nailfold abnormalities, the scleroderma (systemic sclerosis, SSc) pattern, and the normal pattern, and to determine their interreader agreement with an experienced investigator. METHODS: Five investigators (1 senior, 1 junior, and 3 beginners) participated in the exercise. The study was composed of 2 steps. First, an independent investigator selected representative VCP images of normal patterns and capillary abnormalities. The second step included the training program, which ran 4 hours per day for 7 days. The senior rheumatologist taught investigators to recognize and interpret the normal pattern, the capillary abnormalities, and the different types of SSc pattern. These abnormalities were considered: homogeneously enlarged capillaries, giant capillaries, irregularly enlarged capillaries, microhemorrhages, neoangiogenesis, avascular areas, and capillary density. RESULTS: A total of 300 VCP images were read from all the investigators. Both κ values and overall agreement percentages of qualitative and quantitative assessments showed progressive improvement from poor to excellent from the beginning to the end of the exercise. The sensitivity and specificity of the participants in the assessment of SSc pattern at the last lecture session were high. CONCLUSION: Our pilot study suggests that after an intensive 1-week training program, novice investigators with little or no experience in VCP are able to interpret the main capillary abnormalities and SSc pattern and to achieve good interreader agreement rates.

Utility of a simplified ultrasound assessment to assess interstitial pulmonary fibrosis in connective tissue disorders--preliminary results.

INTRODUCTION: Interstitial pulmonary fibrosis (IPF) is a frequent manifestation in patients with connective tissue disorders (CTD). Recently the ultrasound (US) criterion validity for its assessment has been proposed; however, the US scoring systems adopted include the study of several lung intercostal spaces (LIS), which could be time-consuming in daily clinical practice. The aim of this study was to investigate the utility of a simplified US B-lines scoring system compared with both the US comprehensive assessment and the high-resolution computed tomography (HRCT) findings of IPF in CTD patients. METHODS: Thirty-six patients with a diagnosis of CTD were enrolled. Each patient underwent chest HRCT and lung US by an experienced radiologist and rheumatologist, respectively. Both comprehensive and simplified US B-lines assessments were scanned. The comprehensive US assessment was performed at 50 LIS level, whereas the simplified US assessment included bilaterally 14 LIS; for the anterior chest: the second LIS along the para-sternal lines, the fourth LIS along the mid-clavear, anterior axillary and mid-axillary lines; for the posterior chest: the eighth LIS along the paravertebral, sub-scapular and posterior axillary lines. For criterion validity, HRCT was considered the gold standard. Feasibility, inter and intra-observer reliability was also investigated. RESULTS: A highly significant correlation between comprehensive and simplified US assessment was found (P = 0.0001). A significant correlation was also found between the simplified US assessment and HRCT findings (P = 0.0006). Kappa values for the inter-observer simplified US assessment were in a range from 0.769 to 0.885, whereas the concordance correlation coefficient values for the intra-observer were from 0.856 to 0.955. There was a relevant difference in time spent on comprehensive (mean 23.3 ± SD 4.5 minutes) with respect to the simplified US assessment (mean 8.6 ± SD 1.4) (P < 0.00001). CONCLUSIONS: Our results provide a new working hypothesis in favor of the utility of a simplified US B-lines assessment as an adjunct method to assess IPF in patients with CTD.

Interobserver reliability of ultrasonography in the assessment of cartilage damage in rheumatoid arthritis.

OBJECTIVES: To evaluate the interobserver reliability of ultrasonography (US) in the assessment of cartilage damage at metacarpophalangeal (MCP) joint level in patients with rheumatoid arthritis (RA). METHODS: US examinations were performed on 80 MCP joints of 20 patients with RA using a MyLab70 XVG (Esaote Biomedica, Genoa, Italy), equipped with a broadband linear probe (6-18 MHz). For each patient, second and third MCP joints of both hands were examined independently on the same day by two rheumatologists (an experienced musculoskeletal sonographer and an investigator with limited US training). A multiplanar scanning technique on dorsal, lateral and volar aspects of the MCP joints was adopted. All US pathological findings were documented on at least two perpendicular scanning planes. Each joint was assessed by quadrant for the presence or absence of cartilage damage. Cartilage damage was also scored per quadrant on a five-grade semiquantitative scoring system on which investigators reached a consensus prior to the study. RESULTS: Exact agreement between investigators was found in 173 out of 200 quadrants (86.5%) with regard to presence or absence of cartilage damage. Percentages of exact agreement for cartilage damage semiquantitative assessment at dorsal, lateral and volar quadrants were 72.5%, 52.5% and 85%, respectively, while unweighted κ values were 0.561, 0.366 and 0.766, respectively. CONCLUSIONS: The present study demonstrated moderate to good interobserver reproducibility of a semiquantitative scoring system based on qualitative morphological changes for cartilage damage at MCP joint level in patients with RA.