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 ( 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

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

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

The Expanded Risk Score in Rheumatoid Arthritis: 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 arthritis

The increased risk of cardiovascular disease has emerged as a major issue in patients with rheumatoid arthritis – it has been estimated that the cardiovascular disease burden in rheumatoid arthritis is comparable to that of diabetes mellitus

COVID-19 in rheumatic diseases in Italy: first results from the Italian registry of the Italian Society for Rheumatology (CONTROL-19)

OBJECTIVES: Italy was one of the first countries significantly affected by the coronavirus disease 2019 (COVID-19) epidemic. The Italian Society for Rheumatology promptly launched a retrospective and anonymised data collection to monitor COVID-19 in patients with rheumatic and musculoskeletal diseases (RMDs), the CONTROL-19 surveillance database, which is part of the COVID-19 Global Rheumatology Alliance. METHODS: CONTROL-19 includes patients with RMDs and proven severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) updated until May 3rd 2020. In this analysis, only molecular diagnoses were included. The data collection covered demographic data, medical history (general and RMD-related), treatments and COVID-19 related features, treatments, and outcome. In this paper, we report the first descriptive data from the CONTROL-19 registry. RESULTS: The population of the first 232 patients (36% males) consisted mainly of elderly patients (mean age 62.2 years), who used corticosteroids (51.7%), and suffered from multi-morbidity (median comorbidities 2). Rheumatoid arthritis was the most frequent disease (34.1%), followed by spondyloarthritis (26.3%), connective tissue disease (21.1%) and vasculitis (11.2%). Most cases had an active disease (69.4%). Clinical presentation of COVID-19 was typical, with systemic symptoms (fever and asthenia) and respiratory symptoms. The overall outcome was severe, with high frequencies of hospitalisation (69.8%), respiratory support oxygen (55.7%), non-invasive ventilation (20.9%) or mechanical ventilation (7.5%), and 19% of deaths. Male patients typically manifested a worse prognosis. Immunomodulatory treatments were not significantly associated with an increased risk of intensive care unit admission/mechanical ventilation/death. CONCLUSIONS: Although the report mainly includes the most severe cases, its temporal and spatial trend supports the validity of the national surveillance system. More complete data are being acquired in order to both test the hypothesis that RMD patients may have a different outcome from that of the general population and determine the safety of immunomodulatory treatments

Lung ultrasound in patients with rheumatoid arthritis: definition of significant interstitial lung disease

none5noObjectives: The aim of this study was to determine the cut-off number of lung ultrasound (LUS) B-lines that identifies a significant rheumatoid arthritis-interstitial lung disease (RA-ILD). Methods: RA patients with suspected RA-ILD were consecutively enrolled. Patients underwent LUS (carried out in 14 defined intercostal spaces), chest HRCT, pulmonary function tests, and clinical evaluation. The diagnosis of RA-ILD was based on a semi-quantitative evaluation of chest HRCT using a computer-aided method (CaM). The discriminative validity of the LUS versus HRCT has been studied by using the receiver operating characteristic (ROC) curve analysis. Results: 72 consecutive RA patients (21 male, 51 female) were evaluated, with a mean age of 63.0 (SD 11.5 years). The mean estimate of pulmonary fibrosis using the CaM was 11.20% (SD 7.48) at chest HRCT, while at LUS the mean number of B-lines was 10.65 (SD 15.11). A significant RA-ILD, as measured by the CaM at HRCT, was detected in 25 patients (34.7%). The presence of 9 B-lines was found to be the optimal cut-off at ROC curve analysis. This LUS cut-off defines the presence of significant RA-ILD with a sensitivity of 70.0%, a specificity of 97.62%, and a positive likelihood ratio of 29.4. Conclusions: The present study provided data to determine the number of B-lines to identify a significant RA-ILD. LUS may represent a useful technique to select RA patients to be assessed by chest HRCT.noneDi Carlo, Marco; Tardella, Marika; Filippucci, Emilio; Carotti, Marina; Salaffi, FaustoDi Carlo, Marco; Tardella, Marika; Filippucci, Emilio; Carotti, Marina; Salaffi, Faust

Abatacept in rheumatoid arthritis-associated interstitial lung disease: short-term outcomes and predictors of progression

Introduction: Interstitial lung disease in rheumatoid arthritis (RA-ILD) is an extra-articular involvement that impairs the prognosis and for which there is still no well-coded treatment. The aim of this study was to evaluate abatacept (ABA) effectiveness and safety in patients with RA-ILD. Methods: RA-ILD patients who started ABA treatment were consecutively enrolled. Chest high-resolution computed tomography (HRCT), clinical, laboratory and respiratory function variables were collected at baseline and after 18 months of ABA treatment. HRCT abnormalities were evaluated using a computer-aided method (CaM). ABA response was established based on the change in the percentage of fibrosis evaluated at HRCT-CaM, dividing patients into "worsened" (progression ≥ 15%), "improved" (reduction ≥ 15%), and "stable" (changes within the 15% range). The multivariate regression model was used to assess the associations between RA characteristics and ABA response. Results: Forty-four patients (81% women, mean age 59.1 ± 8.0, mean disease duration of 7.5 ± 3.1 years) were studied. Five patients (11.4%) showed RA-ILD progression, 32 patients (72.6%) were considered stable, and 7 patients (16.0%) showed an RA-ILD improvement. The proportion of current smokers was significantly different between "worsened" patients, respect to those defined as "improved/stable" (p = 0.01). Current smoking habit (p = 0.005) and concomitant methotrexate treatment (p = 0.0078) were the two variables related to RA-ILD progression in multivariate regression analysis. Conclusion: Treatment with ABA is associated with a RA-ILD stability or improvement in the 88.6% of patients. Current smoking habit and concomitant treatment with methotrexate are the modifiable factors associated with RA-ILD worsening. Key Points • Abatacept plays a favourable role in the control of RA-ILD, with a significant worsening in only 11.4% of patients during a 18-month follow-up period. • The predictive variables related to RA-ILD progression during abatacept therapy are the concomitant treatment with methotrexate and current smoking habit

Multiple diaphyseal sclerosis (Ribbing disease): what about neridronate?

Sclerosing bone disorders are uncommon diseases and represent a diagnostic challenge. Osteocondensation is a bone alteration, involving both acquired and hereditary conditions. Multiple diaphyseal sclerosis (Ribbing disease) is an inherited condition. It is characterized by excessive proliferation of endosteal and periosteal osseous tissue at the diaphyses of long bones, especially of tibias and femurs. The conventional radiology depicts cortical thickening of diaphyses of long bones while bone scintigraphy shows characteristically an abnormal tracer concentration in the involved diaphyses. The magnetic resonance imaging (MRI) examination confirms the presence of sclerosis and reveals bone marrow edema in the diaphyses of the afflicted bones. Due to the lack of knowledge of the pathophysiology, the treatment is empirical with glucocorticoids or bisphosphonates. Concerning bisphosphonates, the literature reports are conflicting. We report the case of a patient that showed lack of response to intravenous neridronate within 1 year of treatment, both in terms of pain and persistence of bone marrow edema at MRI

A retrospective study of the efficacy of JAK inhibitors or abatacept on rheumatoid arthritis-interstitial lung disease

Objectives: To examine the effectiveness of Janus-kinase inhibitors (JAKis) or abatacept (ABA) in patients with rheumatoid arthritis-interstitial lung disease (RA-ILD). Methods: Patients with RA-ILD receiving JAKis or ABA were retrospectively evaluated at baseline and after 18 months of treatment. A computer-aided method (CaM) was used to assess the extent of high-resolution computed tomography (HRCT) fibrosis percentage. According to HRCT fibrosis changes, patients were classified as "worsened" (progression of 15% or more), "stable" (changes within 15%) or "improved" (reduction of 15% or more). Correlations between RA characteristics and JAKis or ABA responses were studied using a multivariate regression model. Results: Seventy-five patients (69.3% women) were evaluated, 31 received a JAKi while 44 received ABA. In the JAKis group, five patients (16.1%) showed RA-ILD progression, 20 patients (64.5%) were considered stable, and six patients (19.4%) demonstrated RA-ILD improvement. In the ABA group, five patients (11.3%) showed RA-ILD progression, 32 patients (72.7%) were stable, and seven patients (16.0%) demonstrated RA-ILD improvement. In both groups, the percentage of current smokers was different between those classified as "worsened" and those classified as "improved/stable" (p = 0.01). In multivariate regression analysis, current smoking habit (p = 0.0051) and concomitant methotrexate treatment (p = 0.0078) were the two variables related to RA-ILD progression in ABA-treated patients, whereas in JAKis-treated patients, the only RA-ILD progression-related variable was disease duration of RA (p < 0.001). Conclusions: Treatment with JAKis or ABA was related to stability or improvement of RA-ILD in 83.9% and 88.6% of patients, respectively. RA duration is the only variable associated with worsening RA-ILD in JAKis-treated patients

Ultrasound B-lines in the evaluation of interstitial lung disease in patients with systemic sclerosis: Cut-off point definition for the presence of significant pulmonary fibrosis

The aim of this study was to establish the cut-off point of ultrasound (US) B-lines number for detecting the presence of significant interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) (SSc-ILD) in relation to high-resolution computed tomography (HRCT) findings.Consecutive SSc-ILD patients underwent chest HRCT, lung US (LUS), pulmonary function test, and clinical assessment. Exclusion criteria were represented by the presence of a coexisting congestive heart failure and a clinical history suggestive of lung or pleural diseases. HRCT images were scored for the presence of ILD by 2 readers, in accordance with the Warrick scoring system. US assessment was performed by a US skilled rheumatologist, blinded to HRCT results and clinical data, and included the bilateral evaluation of 14 lung intercostal spaces (LIS). In each LIS, the number of B-lines was recorded and summed. To test discriminant validity, we used the receiver operating characteristic (ROC) curve analysis applying a Warrick score of 7 as external criterion for the presence of SSc-ILD.Forty patients completed the study. The US B-lines number and the Warrick score confirmed excellent correlation (Spearman rho: 0.958, P = .0001). The ROC curve analysis revealed that the presence of 10 US B-lines is the cut-off point with the greatest positive likelihood ratio (12.52) for the presence of significant SSc-ILD.The detection of 10 B-lines is highly predictive for the HRCT presence of significant SSc-ILD. In SSc patients, the LUS assessment as first imaging tool may represent an effective model to improve the correct timing of chest HRCT