Objective To identify the main computed tomography (CT) features that may help distinguishing a progression of interstitial lung disease (ILD) secondary to Systemic sclerosis (SSc) from COVID-19 pneumonia. Methods This multicentric study included 22 international readers divided in the radiologist group (RAD) and non-radiologist group (nRAD). A total of 99 patients, 52 with COVID-19 and 47 with SSc-ILD, were included in the study. Results Fibrosis inside focal ground glass opacities (GGO) in the upper lobes; fibrosis in the lower lobe GGO; reticulations in lower lobes (especially if bilateral and symmetrical or associated with signs of fibrosis) were the CT features most frequently associated with SSc-ILD. The CT features most frequently associated with COVID- 19 pneumonia were: consolidation (CONS) in the lower lobes, CONS with peripheral (both central/peripheral or patchy distributions), anterior and posterior CONS and rounded-shaped GGOs in the lower lobes. After multivariate analysis, the presence of CONS in the lower lobes (p < 0.0001) and signs of fibrosis in GGO in the lower lobes (p < 0.0001) remained independently associated with COVID-19 pneumonia or SSc-ILD, respectively. A predictive score was created which resulted positively associated with the COVID-19 diagnosis (96.1% sensitivity and 83.3% specificity). Conclusion The CT differential diagnosis between COVID-19 pneumonia and SSc-ILD is possible through the combination the proposed score and the radiologic expertise. The presence of consolidation in the lower lobes may suggest a COVID-19 pneumonia while the presence of fibrosis inside GGO may indicate a SSc-ILD.

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Systemic sclerosis is a rare and chronic connective tissue disease with a multifaceted pathogenesis characterised by heterogeneous multi-organ clinical manifestations. Every year, many studies contribute to enrich the knowledge on the pathogenesis, organ involvement and treatment of this complex and severe disease. We herein provide an overview on the most relevant contributions published in the literature in 2020.

Objectives Lung ultrasound (LUS), through assessment of B-lines and pleural line alterations, is able to evaluate interstitial lung disease (ILD), a frequent complication of systemic sclerosis (SSc). Different scanning schemes and counting methods have been proposed, but no clear cut-off values have been indicated for screening. We aimed to evaluate the accuracy of different LUS methodological approaches to detect ILD, compared with high resolution computed tomography (HRCT) as gold standard. Methods Sixty-nine SSc patients underwent LUS and chest HRCT on the same day. Both exams were scored by expert readers. The accuracy of different scanning schemes and counting methods was assessed, and clinical and functional data were compared with imaging findings. Results B-lines were more numerous in patients with the diffuse skin subset and Scl70 autoantibody positivity. The number of B-lines correlated with the Scleroderma Lung Study (SLS) I HRCT score (R = 0.754, p< 0.0001). A total number >10 B-lines on the whole chest or > 1 B-line on the postero-basal chest showed 97% sensitivity for detecting even very early ILD signs (corresponding to SLS I score =1). Sensitivity increased to 100% when pleural line alterations were included in the analysis. Conclusions LUS has a very high sensitivity in detecting SSc-related ILD. A cut-off value of > 10 B-lines on the whole chest or > 1 B-line on the postero-basal chest can be used for the screening of SSc-ILD. Assessing only the postero-basal chest seems mostly effective to combine high sensitivity with a less time-consuming approach.

COVID-19 is a world health emergency which may inevitably affect the management of a complex autoimmune disease such as systemic sclerosis (SSc). Several SSc patients are frail and, in this pandemic, need a careful protection. The COVID-19 infection might complicate the clinical scenario of interstitial lung disease (ILD) in SSc because it determines a severe pneumonia characterized by radiological features similar to SSc-ILD. The striking CT similarities between the 2 diseases make it difficult to distinguish a worsening of SSc-ILD from COVID-19-ILD superinfection. Moreover, other aspects, like isolation during lock down, may cause a significant psychological stress which will pile up on the already difficult contact with the patients for a routine check-up. Moreover, the drug shortage is a real problem in these times. For these reasons, the rheumatologist in daily clinical practice should carefully differentiate the possible COVID-19 infection in order to optimize the patient management. Therefore, the challenge in everyday life will be to achieve in due time the differential diagnosis as well as the long-term psychological impact. Key Points• SSc patients should be encouraged to continue their chronic therapy; in case of immunosuppressive therapy it must be discontinued for safety in case of COVID-19 infection. • Psychological support must be guaranteed to every SSc patients.• COVID-19 pneuminia is hard to distinguish from an interstitial lung disease due to SSc lung involvment.• Data sharing is fundamental for an optimal managment of SSc patients during COVID-19 pandemia.

Sjögren’s syndrome (SS) is a complex autoimmune rheumatic disease that specifically targets salivary and lachrymal glands. As such, patients typically had ocular and oral dryness and salivary gland swelling. Moreover, skin, nasal and vaginal dryness are frequently present. In addition to dryness, musculoskeletal pain and fatigue are the hallmarks of this disease and constitute the classic symptom triad presented by the vast majority of patients. Up to 30% to 50 % of patients with SS may present systemic disease; moreover, there is an increased risk for the development of non-Hodgkin’s lymphoma that occurs in a minority of patients. The present work was developed in the framework of the European Reference Network (ERN) dedicated to Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET). In line with its goals of aiming to improve early diagnosis, treatment and care of rare connective and musculoskeletal diseases, ERN-ReCONNET set to review the current state of clinical practice guidelines (CPGs) in the rare and complex connective tissue diseases of interest of the network. Therefore, the present work was aimed at providing a state of the art of CPGs for SS.

The aim of the European Journal of Rheumatology is to cover various aspects of rheumatology for its readers, encompassing the spectrum of diseases with arthritis, musculoskeletal conditions, autoinflammatory diseases, connective tissue disorders, osteoporosis, translational research, the latest therapies and treatment programs. European Journal of Rheumatology publishes original articles, invited reviews, case based reviews, letters to the editor and images in rheumatology. The publication language of the journal is English.

In carpal tunnel syndrome (CTS), manual therapy interventions (MTI) reduce tissue adhesion and increase wrist mobility. We evaluated the efficacy of a MTI in relieving CTS signs and symptoms. Twenty-two CTS patients (pts) (41 hands) were treated with a MTI, consisting in 6 treatments (2/week for 3 weeks) of soft tissues of wrist and hands and of carpal bones. Pts were assessed for hand sensitivity, paresthesia, hand strength, hand and forearm pain, night awakening; Phalen test, thenar eminence hypotrophy and Boston Carpal Tunnel Questionnaire (BCTQ) Symptom Severity Scale (SSS) and Functional Status Scale (FSS). Median nerve was studied by sensory nerve conduction velocity (SNCV) and distal motor latency (DML). CTS was scored as minimal, mild, medium, severe and extreme. We considered as control group the same pts assessed before treatment: at baseline (T0a) and after 12 weeks (T0b). Pts were evaluated at the end of treatment (T1) and after 24-week (T2) follow-up. At T0b, versus T0a, forearm pain and Phalen test positivity were increased and hand strength reduced (p < 0.05). BCTQ-SSS and BCTQ-FSS scores improved at T1 versus T0b (p < 0.05) with the amelioration maintained at T2. At T1, the number of pts with paresthesia, night awakening, hypoesthesia, Phalen test, hand strength reduction and hand sensitivity was reduced with the lacking of symptoms maintained at T2 (p < 0.05). No changes in SNCV, DML and CTS scoring were shown. MTI improved CTS signs and symptoms, with benefits maintained at follow-up. Thus, it may be valid as a conservative therapy.