Systemic sclerosis in the time of COVID-19

The COVID-19 pandemic represents one of the biggest challenges of the 21st century. In addition to the general effect on society and health-care systems, patients with systemic sclerosis and their physicians face specific challenges related to the chronic nature of their disease, the involvement of multiple organs, and the use of immunosuppressive treatments. Data from registries and single centre cohorts indicate that the risk of contracting SARS-CoV-2 does not seem to increase substantially in people with systemic sclerosis;conversely, severe COVID-19 outcomes are seen more frequently in these patients than in the general population. Vaccination against SARS-CoV-2 is therefore highly recommended for patients with systemic sclerosis;however, no specific recommendations are available regarding the different vaccine platforms. Both patients and physicians should be aware that the effectiveness of vaccines might be reduced in patients taking immunosuppressive therapy, because antibody responses might be blunted, specifically in patients treated with rituximab and mycophenolate mofetil.

Lung involvement in systemic sclerosis

Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy, immune system activation, and fibrosis of the skin and internal organs [1]. While over the recent years, the prognosis of SSc patients has greatly improved, thanks to a better understanding of the underlying pathogenetic phenomena and the arousal of targeted therapies, the disease still presents the highest morbidity and mortality among all connective tissue diseases [2]. Compared to those years when SSc renal crisis represented the leading cause of death, now in the last decades, lung involvement (both pulmonary hypertension and pulmonary fibrosis) has become the primary cause of SSc-related death [3]. Therefore, clinicians involved in SSc care should be aware of the most recent evolutions in the field of SSc with particular attention to lung involvement. In fact, the early detection and the prompt treatment both of fibrotic and vascular lung disease may have fundamental impact on patients' quality of life and survival. In SSc, lung involvement is characterized by interstitial lung disease (ILD), which is due to the fibrosis of the alveolo-capillary membrane, and pulmonary arterial hypertension (PAH), which reflects the proliferative vasculopathy affecting pulmonary vessels [1,4,5]. Usually, SSc is classified according to 2013 ACR/EULAR criteria [6] and is also subsetted in a limited cutaneous (lcSSc;affecting the face, neck and distal limbs) and a diffuse cutaneous SSc (dcSSc;affecting the proximal limbs, abdomen, and chest). Both subsets are associated with specific clinical features and autoantibody positivity [7]. In this chapter, either SSc-ILD or SSc-PAH will be discussed, and, given the historical period, the clinical and prognostic data on COVID-19 in SSc patients will be also presented. © 2022 Elsevier B.V.

The experience of a rheumatology unit during the COVID19 lockdown: Telemedicine allows a safe follow up of patients with rheumatic diseases

Background: In March this year, most of the routine activities were cancelled during the streaming of the pandemic in Italy. This prompted a pragmatic reorganization of the traditional care model of nursing and medicine, to quickly give an efficient clinical response. During the first phase of the pandemic, outpatient visits dropped by more than 60%, forcefully shifting to telemedicine to assure continuity of care despite the lockdown. Objectives: The aim of the present work was to describe the strategy adopted during and immediately after the lockdown to assure the follow up of patients and the maintenance of their treatment in an outpatient “virtual” telemedicine clinic dedicated to RDs. Methods: the patient flow to a rheumatology division during the lockdown was evaluated retrospectively from March to September 2020 in accordance with local restrictions, and three periods are described. Results: 653/913 (71.5%), 542/542 (100%) and 1.048/1.048 (100%) infusion activities scheduled were performed at the centre for daily infusion and pre-infusion assessment, respectively during the 1st, 2nd and 3rd period. In the outpatient clinic during the 1st period, 96.96% of the cases was shifted to Telemedicine, which decreased to 52.45% in the 2nd period;while in the 3rd period, 97.6% of the performances were carried out at the clinic. Diagnostic procedures, such as ultrasound, capillaroscopy, and joint injection were generally postponed during the 1st period, reduced drastically during the 2nd and performed regularly during 3rd period. Ulcer treatment and the Clinical Trial Unit never stopped their activity. The flow of the activity of the outpatient clinic and the day hospital is represented as monthly trends in graph 1 (See Graph 1). Conclusion: Our data show the feasibility of Telemedicine in a lockdown condition. Shifting stable patients to Telemedicine has the potentiality to minimize the risk of contagion and allow continuity of care. In the future, the use of Telemedicine for specific clinical uses might assure patient assistance also in non-pandemic conditions. (Figure Presented).

The role of chest ct in understanding interstitial lung disease (ILD): Systemic sclerosis (SSC). versus COVID-19

Background: COVID-19 pandemic is a global emergency which may overlap on the clinical and radiological scenario of ILD in SSc. In clinical practice, the striking similarities observed at computed tomography (CT) between the diseases make it difficult to distinguish a COVID-19 superinfection from a progression of SSc-ILD. Objectives: The aim of our study was to identify the main CT features that may help distinguishing SSc-ILD from COVID-19 pneumonia. Methods: 22 international readers were included and divided in the radiologist group (RAD) and non-radiologist group (nRAD). The RAD group included nonchest RAD and chest-RAD. 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 parameters most frequently associated with SSc-ILD. The CT parameters 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. These two variables were combined in a predictive score which resulted positively associated with the COVID-19 diagnosis, with 96.1% sensitivity and 83.3% specificity: 3 different risk class for COVID-19 pneumonia may be identified: high risk for COVID-19 pneumonia (5-9 points);probable overlap COVID-19 pneumonia in SSc-ILD (4 points);low risk for COVID-19 pneumonia (0-3 points). Conclusion: The CT differential diagnosis between COVID-19 Pneumonia and SSc-ILD is possible and may be fostered in practice by the use of a radiological score. In the case where an overlap of both diseases is suspected, 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.