Publications by authors named "Marco Matucci-Cerinic"

509 Publications

Nintedanib in patients with systemic sclerosis-associated interstitial lung disease: subgroup analyses by autoantibody status and skin score.

Arthritis Rheumatol 2021 Sep 12. Epub 2021 Sep 12.

Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.

Objective: We used data from the SENSCIS trial to assess the effects of nintedanib versus placebo in subgroups of patients with SSc-ILD based on characteristics associated with progression of SSc-ILD in previous studies.

Methods: Patients with SSc-ILD were randomized to receive nintedanib or placebo, stratified by anti-topoisomerase I antibody (ATA) status. We assessed the rate of decline in forced vital capacity (FVC) (mL/year) over 52 weeks in subgroups by baseline ATA status, modified Rodnan skin score (mRSS) (<18 versus ≥18), and SSc subtype (limited cutaneous SSc [lcSSc] versus diffuse cutaneous SSc [dcSSc]).

Results: At baseline, of 576 patients treated, 60.8% were ATA-positive, 51.9% had dcSSc, and 77.5% of 574 patients with mRSS data available had mRSS <18. The effect of nintedanib versus placebo on reducing the rate of decline in FVC (mL/year) was numerically more pronounced in patients who were ATA-negative (difference: 57.2 [95% CI -3.5, 118.0]) than ATA-positive (difference: 29.9 [-19.1, 78.8]), in patients who had mRSS ≥18 (difference: 88.7 [7.7, 169.8]) than mRSS <18 at baseline (difference: 26.4 [-16.8, 69.6]), and in patients with dcSSc (difference: 56.6 [3.2, 110.0]) than lcSSc (difference: 25.3 [-28.9, 79.6]), but exploratory interaction P values did not indicate heterogeneity in the effect of nintedanib versus placebo between these subgroups (P > 0.05 for all).

Conclusion: In patients with SSc-ILD, no heterogeneity was detected in the treatment effect of nintedanib in reducing the annual rate of decline in FVC across subgroups based on ATA status, mRSS, and SSc subtype.
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http://dx.doi.org/10.1002/art.41965DOI Listing
September 2021

Covid-19 And Rheumatic Autoimmune Systemic Diseases: Role of Pre-Existing Lung Involvement and Ongoing Treatments.

Curr Pharm Des 2021 Sep 2. Epub 2021 Sep 2.

Humanitas Clinical and Research Center IRCCS, Milano, Italy.

Background: The Covid-19 pandemic may have a deleterious impact on patients with autoimmune systemic diseases (ASD) due to their deep immune-system alterations.

Objective: To investigate the prevalence of symptomatic Covid-19 and its correlations with both organ involvement and ongoing treatments in a large series of Italian ASD patients during the first wave of pandemic.

Method: Our multicenter telephone 6-week survey included 3,029 unselected ASD patients enrolled at 36 tertiary referral centers of northern, central, and southern Italian macro-areas with different diffusion of pandemic. Symptomatic SARS-CoV-2 infection was classified as definite Covid-19 (presence of symptoms plus positive oral/nasopharyngeal swabs) or highly suspected Covid-19 (highly suggestive symptoms, in absence of a swab testing).

Results: A significantly higher prevalence of definite plus highly suspected Covid-19 compared to Italian general population was detected in the whole ASD series (p=.000), as well as in patients from the three macro-areas (p=.000 in all). Statistically higher prevalence of Covid-19 was also found in connective tissue diseases compared to chronic arthritis subgroup (p=.000) and in ASD patients with pre-existing interstitial lung involvement (p=.000). Patients treated with either conventional disease modifying anti-rheumatic drugs (DMARDs) and/or biological DMARDs showed a significantly lower prevalence of Covid-19 (p=.000 in both). Finally, scleroderma patients undergoing low-dose aspirin showed significantly lower rate of Covid-19 compared to those without (p=0.003).

Conclusion: The higher prevalence of Covid-19 in ASD patients along with the significant correlations with important clinical features and therapeutic regimens suggests the need to develop targeted prevention/management strategies during the current pandemic wave.
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http://dx.doi.org/10.2174/1381612827666210903103935DOI Listing
September 2021

Rheumatoid arthritis, systemic lupus erythematosus and primary Sjögren's syndrome shared megakaryocyte expansion in peripheral blood.

Ann Rheum Dis 2021 Aug 30. Epub 2021 Aug 30.

Department of Pathology, Provincial Key Laboratory of Infectious Diseases and Molecular Immunopathology, Shantou University Medical College, Shantou, China

Objectives: Rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS) share many clinical manifestations and serological features. The aim of this study was to identify the common transcriptional profiling and composition of immune cells in peripheral blood in these autoimmune diseases (ADs).

Methods: We analysed bulk RNA-seq data for enrichment of biological processes, transcription factors (TFs) and deconvolution-based immune cell types from peripheral blood mononuclear cells (PBMCs) in 119 treatment-naive patients (41 RA, 38 pSS, 28 SLE and 12 polyautoimmunity) and 20 healthy controls. The single-cell RNA-seq (scRNA-seq) and flow cytometry had been performed to further define the immune cell subsets on PBMCs.

Results: Similar transcriptional profiles and common gene expression signatures associated with nucleosome assembly and haemostasis were identified across RA, SLE, pSS and polyautoimmunity. Distinct TF ensembles and gene regulatory network were mainly enriched in haematopoiesis. The upregulated cell-lineage-specific TFs , , and demonstrated a strong gene expression signature of megakaryocyte (MK) expansion. Gene expression-based cell type enrichment revealed elevated MK composition, specifically, CD41bCD42b and CD41bCD61 MKs were expanded, further confirmed by flow cytometry in these ADs. In scRNA-seq data, MKs were defined by TFs and pre-T-cell antigen receptor gene, . Cellular heterogeneity and a distinct immune subpopulation with functional enrichment of antigen presentation were observed in MKs.

Conclusions: The identification of MK expansion provided new insights into the peripheral immune cell atlas across RA, SLE, pSS and polyautoimmunity. Aberrant regulation of the MK expansion might contribute to the pathogenesis of these ADs.
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http://dx.doi.org/10.1136/annrheumdis-2021-220066DOI Listing
August 2021

The role of lung ultrasound B-lines and serum KL-6 in the screening and follow-up of rheumatoid arthritis patients for an identification of interstitial lung disease: review of the literature, proposal for a preliminary algorithm, and clinical application to cases.

Arthritis Res Ther 2021 08 14;23(1):212. Epub 2021 Aug 14.

Department of Experimental and Clinical Medicine & Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, University of Florence, Florence, Italy.

Screening and follow-up of interstitial lung disease associated with rheumatoid arthritis (RA-ILD) is a challenge in clinical practice. In fact, the majority of RA-ILD patients are asymptomatic and optimal tools for early screening and regular follow-up are lacking. Furthermore, some patients may remain oligosymptomatic despite significant radiological abnormalities. In RA-ILD, usual interstitial pneumonia (UIP) is the most frequent radiological and pathological pattern, associated with a poor prognosis and a high risk to develop acute exacerbations and infections. If RA-ILD can be identified early, there may be an opportunity for an early treatment and close follow-up that might delay ILD progression and improve the long-term outcome.In connective tissue disease-associated interstitial lung disease (CTD-ILD), lung ultrasound (LUS) with the assessment of B-lines and serum Krebs von den Lungen-6 antigen (KL-6) has been recognized as sensitive biomarkers for the early detection of ILD. B-line number and serum KL-6 level were found to correlate with high-resolution computed tomography (HRCT), pulmonary function tests (PFTs), and other clinical parameters in systemic sclerosis-associated ILD (SSc-ILD). Recently, the significant correlation between B-lines and KL-6, two non-ionizing and non-invasive biomarkers, was demonstrated. Hence, the combined use of LUS and KL-6 to screen and follow up ILD in RA patients might be useful in clinical practice in addition to existing tools. Herein, we review relevant literature to support this concept, propose a preliminary screening algorithm, and present 2 cases where the algorithm was used.
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http://dx.doi.org/10.1186/s13075-021-02586-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8364073PMC
August 2021

Pulmonary magnetic resonance imaging in systemic sclerosis: a jump in the future to unravel inflammation in interstitial lung disease.

Clin Rheumatol 2021 Sep 30;40(9):3461-3464. Epub 2021 Jul 30.

Department of Experimental and Clinical Biomedical Sciences, and Radiodiagnostic Unit N. 2, University of Florence-Azienda Ospedaliero-Universitaria Careggi, Largo Brambilla 3, 50134, Florence, Italy.

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http://dx.doi.org/10.1007/s10067-021-05869-3DOI Listing
September 2021

A randomised, double-blind, placebo-controlled phase 3 study of lenabasum in diffuse cutaneous systemic sclerosis: RESOLVE-1 design and rationale.

Clin Exp Rheumatol 2021 Jul-Aug;39 Suppl 131(4):124-133. Epub 2021 Jul 21.

UCL Centre for Rheumatology and Connective Tissue Diseases, Royal Free Campus, University College London, UK.

Objectives: The multi-systemic, heterogenous nature of diffuse cutaneous systemic sclerosis (dcSSc) presents challenges in designing clinical studies that can demonstrate a treatment effect on overall disease burden. We describe the design of the first Phase 3 study in dcSSc patients where the American College of Rheumatology (ACR) Combined Response Index in diffuse cutaneous Systemic Sclerosis (CRISS) score was chosen prospectively as the primary outcome. The CRISS measures key clinical disease parameters and patient-reported outcomes (PROs).

Methods: RESOLVE-1 is a Phase 3, randomised, double-blind, placebo-controlled trial of dcSSc patients evaluating the efficacy and safety of lenabasum. Patients ≥18 years of age with dc-SSc and disease duration ≤6 years were eligible. Patients could continue stable background therapy for dcSSc, including stable immunosuppressive therapies. They were randomised to lenabasum 5 or 20 mg twice daily or placebo. The primary efficacy outcome was the mean change from baseline to 52 weeks in the ACR CRISS score.

Results: The study enrolled 365 patients over 1.5 years at 77 sites in 13 countries in North America, Europe, Israel, and Asia-Pacific, with the last patient first visit on May 1, 2019.

Conclusions: RESOLVE-1 is the first Phase 3 interventional study to date in dcSSc to prospectively use the ACR CRISS as the primary efficacy outcome. Eligibility criteria allowed background therapy as might occur in clinical practice. This approach also facilitated timely patient enrolment. RESOLVE-1 provides a novel study design that may be used for future Phase 3 dcSSc studies to assess the holistic efficacy of therapy.
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August 2021

THE ROLE OF CHEST CT IN DECIPHERING INTERSTITIAL LUNG INVOLVEMENT: SYSTEMIC SCLEROSIS VERSUS COVID-19.

Rheumatology (Oxford) 2021 Jul 28. Epub 2021 Jul 28.

Department of Experimental and Clinical Medicine, University of Florence, and Infectious and TropicalDiseases Unit, AOUC, Florence, Italy.

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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http://dx.doi.org/10.1093/rheumatology/keab615DOI Listing
July 2021

Tofacitinib in the treatment of skin and musculoskeletal involvement in patients with systemic sclerosis, evaluated by ultrasound.

Rheumatol Int 2021 Oct 27;41(10):1743-1753. Epub 2021 Jul 27.

Department of Internal Diseases, Medical University of Plovdiv, Plovdiv, Bulgaria.

Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by fibrosis of the skin and internal organs, autoimmunity-driven damage and vasculopathy. The current approved disease-modifying treatments have limited efficacy, and treatment is guided toward alleviating organ complications. Thus, there is an unmet need for discovering new effective treatment options. There is recent evidence that the JAK/STAT signaling pathway is markedly activated in SSc patients. To assess the efficacy and safety of tofacitinib (TOF) on skin and musculoskeletal involvement as compared to methotrexate (MTX) in systemic sclerosis (SSc). In this 52-week pilot study, 66 patients with SSc were enrolled: 33 patients received 5 mg of oral TOF twice a day; 33 received 10 mg of MTX weekly. The proportion of dcSSc and lcSSc patients was similar (dcSSc: 42% TOF group and 36% MTX group; lcSSc: 58% TOF group and 64% MTX group). The primary outcome was the change in the modified Rodnan skin score (mRSS). Secondary outcomes included ultrasound (US) skin thickness and musculoskeletal involvement (US10SSc score). Digital ulcers (DUs) and adverse events (AEs) were documented through the treatment. Both groups had similar characteristics and medians on the outcome measures at baseline. At week 52, the TOF median mRSS was significantly lower than the MTX (p < 0.001) with a mean reduction of 13 points versus MTX 2.57. The mean percent improvement in the TOF group was 44% higher than in the MTX group. TOF median US skin thickness was significantly lower than MTX (p < 0.001), with a mean reduction of 0.31 mm versus 0.075 mm in the MTX group. The US10SSc median score was significantly lower in the TOF group (p = 0.002); mean reduction of 10.21 versus 5.27 in the MTX group. Healing of DUs with no new occurrences was observed in the TOF group. There was no significant difference between the groups in the number of AEs from baseline to week 52. TOF showed greater efficacy than MTX in reducing mRSS, skin thickness and musculoskeletal involvement in SSc and a satisfactory safety profile.
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http://dx.doi.org/10.1007/s00296-021-04956-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8390399PMC
October 2021

Peripheral neuropathy in systemic lupus erythematosus: what can neuromuscular ultrasonography (NMUS) tell us? A cross-sectional study.

Lupus Sci Med 2021 07;8(1)

Department of Rheumatology, Rehabilitation and Physical Medicine, Assiut University, Assiut, Egypt.

Objective: To evaluate peripheral nerve involvement in patients with SLE with neuromuscular ultrasonography (NMUS) and understand its role in investigating SLE-related peripheral neuropathy.

Methods: This is an observational cross-sectional study on patients with SLE and healthy controls. Five nerves in each patient were examined bilaterally with NMUS, and the cross-sectional area (CSA) of each nerve at certain sites was estimated. The mean CSA at each site, for each nerve, in each group was statistically analysed and compared between groups.

Results: 370 nerves were evaluated in 37 patients. By nerve conduction study (NCS), 36 patients had polyneuropathy (80.6% mixed type, 19.4% sensory). Significant mean CSA enlargement was present among the ulnar nerve at the Guyon's canal and mid-humerus (both p=0.001); tibial nerve at the distal leg and proximal to the tarsal tunnel (p=0.003 and p=0.001, respectively); and peroneal nerve at the popliteal fossa (p=0.042). The mean CSA showed high specificity compared with NCS.

Conclusion: Our study shows that CSA could be a complementary tool to NCS for studying peripheral neuropathy in SLE. Furthermore, NMUS provides data on the different pathophysiological aspects of nerve involvement in SLE. Future studies using more than one sonographic parameter in combination with NCS and nerve histopathology are recommended to further investigate SLE-related neuropathy. NCT04527172.
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http://dx.doi.org/10.1136/lupus-2021-000521DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8314698PMC
July 2021

Similarities between COVID-19 and systemic sclerosis early vasculopathy: A "viral" challenge for future research in scleroderma.

Autoimmun Rev 2021 Jul 15;20(10):102899. Epub 2021 Jul 15.

Division of Rheumatology, Allergy and Immunology, University of Toledo Medical Center, Toledo, OH, USA.

Objective: To review similarities between COVID-19 and systemic sclerosis (SSc) early vasculopathy to provide novel insights into both diseases.

Methods: A narrative review of the literature supplemented with expert opinion.

Results: There is clear evidence that the endothelium is at the centre stage in SSc and COVID-19, with endothelial cell activation/injury and dysfunction creating the crucial evolving step in the pathogenesis of both diseases. The angiotensin system has also been implicated in the early stages of both COVID-19 and SSc. Autoptic studies provide novel insights into the effects of SARS-CoV-2 on the endothelium. Normal endothelium and endothelial dysfunction in COVID-19 and SSc are discussed. It is debated whether SARS-CoV-2 infection triggers autoimmunity with production of autoantibodies which is of mechanistic interest because other viral illnesses are potentially involved in endothelial dysfunction and in SSc pathogenesis.

Conclusion: COVID-19 is due to a direct assault of SARS-CoV-2 on the vascular system as an acute infection, whereas SSc remains a chronic/sub-acute autoimmune disease of largely unknown etiology Further study and exploration of the SARS-CoV-2 pathogenic mechanisms might provide further useful milestones in the understanding of the early SSc pathogenesis.
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http://dx.doi.org/10.1016/j.autrev.2021.102899DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8280663PMC
July 2021

Exploring the Oral Microbiome in Rheumatic Diseases, State of Art and Future Prospective in Personalized Medicine with an AI Approach.

J Pers Med 2021 Jun 30;11(7). Epub 2021 Jun 30.

Department of Clinical and Experimental Medicine, University of Florence, Largo Brambilla 3, 50134 Florence, Italy.

The oral microbiome is receiving growing interest from the scientific community, as the mouth is the gateway for numerous potential etiopathogenetic factors in different diseases. In addition, the progression of niches from the mouth to the gut, defined as "oral-gut microbiome axis", affects several pathologies, as rheumatic diseases. Notably, rheumatic disorders (RDs) are conditions causing chronic, often intermittent pain affecting the joints or connective tissue. In this review, we examine evidence which supports a role for the oral microbiome in the etiology and progression of various RDs, including rheumatoid arthritis (RA), Sjogren's syndrome (SS), and systemic lupus erythematosus (SLE). In addition, we address the most recent studies endorsing the oral microbiome as promising diagnostic biomarkers for RDs. Lastly, we introduce the concepts of artificial intelligence (AI), in particular, machine learning (ML) and their general application for understanding the link between oral microbiota and rheumatic diseases, speculating the application of a possible AI approach-based that can be applied to personalized medicine in the future.
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http://dx.doi.org/10.3390/jpm11070625DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8306274PMC
June 2021

Oral Species in Systemic Sclerosis.

Microorganisms 2021 Jun 15;9(6). Epub 2021 Jun 15.

Department of Experimental and Clinical Medicine, Department of Geriatric Medicine, Division of Rheumatology, University of Firenze, 50124 Firenze, Italy.

In systemic sclerosis (SSc), the gastrointestinal tract (GIT) plays a central role in the patient's quality of life. The microbiome populates the GIT, where a relationship between the and gastrointestinal motility has been suggested. In this study, the analysis of oral species in SSc patients and healthy subjects using culture-independent molecular techniques, together with a review of the literature on microbiota and lactobacilli in SSc, has been carried out. Twenty-nine SSc female patients (mean age 62) and twenty-three female healthy subjects (HS, mean age 57.6) were enrolled and underwent tongue and gum swab sampling. Quantitative PCR was conducted in triplicate using specific primers 1, 1o and 2 for the RNA-polymerase β subunit gene. Our data show significantly ( = 0.0211) lower spp sequences on the tongue of patients with SSc compared to HS. The mean value of the amount of gene on the gumsofSSc patients was minor compared to HS. A significant difference between tongue and gums ( = 0.0421) was found in HS but not in SSc patients. In conclusion, our results show a lower presence of in the oral cavity of SSc patients. This strengthens the hypothesis that may have both a protective and therapeutic role in SSc patients.
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http://dx.doi.org/10.3390/microorganisms9061298DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8232208PMC
June 2021

New Insights into Profibrotic Myofibroblast Formation in Systemic Sclerosis: When the Vascular Wall Becomes the Enemy.

Life (Basel) 2021 Jun 24;11(7). Epub 2021 Jun 24.

Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, 50134 Florence, Italy.

In systemic sclerosis (SSc), abnormalities in microvessel morphology occur early and evolve into a distinctive vasculopathy that relentlessly advances in parallel with the development of tissue fibrosis orchestrated by myofibroblasts in nearly all affected organs. Our knowledge of the cellular and molecular mechanisms underlying such a unique relationship between SSc-related vasculopathy and fibrosis has profoundly changed over the last few years. Indeed, increasing evidence has suggested that endothelial-to-mesenchymal transition (EndoMT), a process in which profibrotic myofibroblasts originate from endothelial cells, may take center stage in SSc pathogenesis. While in arterioles and small arteries EndoMT may lead to the accumulation of myofibroblasts within the vessel wall and development of fibroproliferative vascular lesions, in capillary vessels it may instead result in vascular destruction and formation of myofibroblasts that migrate into the perivascular space with consequent tissue fibrosis and microvessel rarefaction, which are hallmarks of SSc. Besides endothelial cells, other vascular wall-resident cells, such as pericytes and vascular smooth muscle cells, may acquire a myofibroblast-like synthetic phenotype contributing to both SSc-related vascular dysfunction and fibrosis. A deeper understanding of the mechanisms underlying the differentiation of myofibroblasts inside the vessel wall provides the rationale for novel targeted therapeutic strategies for the treatment of SSc.
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http://dx.doi.org/10.3390/life11070610DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8307837PMC
June 2021

Rehabilitation of the face and temporomandibular joint in systemic sclerosis.

Ther Adv Musculoskelet Dis 2021 8;13:1759720X211020171. Epub 2021 Jun 8.

Researcher in Rheumatology, Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Largo Brambilla 3, Florence, 50134, Italy.

Background: Systemic sclerosis (SSc) alterations of the face and of the mouth cause aesthetic modifications and disability, impairing self-esteem and quality of life (QoL). The aim of this study was to verify the effects of two rehabilitation protocols on facial mimic and mouth opening.

Methods: A total of 47 SSc patients (40 females and 7 males, mean age ± SD 59.08 ± 10.31 years), were consecutively selected: 22 were randomly assigned to protocol 1 [home exercises for temporomandibular joint (TMJ), mimic, masticatory and cervical spine muscles] and 25 to protocol 2 (home exercises and combined physiotherapeutic procedures performed by a physiotherapist). Each treatment had a duration of 12 weeks with a follow up of 8 weeks. TMJ dysfunction, orofacial involvement, disability, QoL, and safety were assessed at enrollment (T0), at the end of the treatment (T1), and at follow up (T2).

Results: Both Protocol 1 and Protocol 2 induced significant improvements of some clinical and clinimetric parameters, but better results were obtained with Protocol 2. In the comparison between the effects of Protocol 1 and Protocol 2 at T1 and T2, a significant difference was observed only for Mouth Handicap in SSc [MHISS; Total ( = 0.00178] and for MHISS Mouth opening ( = 0.0098) at T1. No significant difference of indices of short-form 36 was observed.

Conclusion: The present data suggest that TMJ involvement in SSc may be managed by rehabilitation treatments. The action of a physiotherapist prescribing and personalizing exercises may induce better therapeutic effects.
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http://dx.doi.org/10.1177/1759720X211020171DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8191075PMC
June 2021

Digital pitting scars are associated with a severe disease course and death in systemic sclerosis: a study from the EUSTAR cohort.

Rheumatology (Oxford) 2021 Jun 19. Epub 2021 Jun 19.

Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy.

Objective: Digital pitting scars (DPS) are frequent, but little studied in SSc to date.

Methods: An analysis of SSc patients enrolled in the EUSTAR database. Primary objectives were to 1) examine DPS prevalence, 2) whether DPS are associated with digital ulcers (DUs) and active digital ischaemia (DUs or gangrene), and 3) describe other associations with DPS including internal organ complications. Secondary objectives were whether DPS are associated with 1) functional impairment, 2) structural microvascular disease, 3) and mortality. Descriptive statistics and parametric/non-parametric tests were used. Binary logistic regression was used to examine the association between DPS and DUs, active digital ischaemia, and mortality.

Results: 9671 patients were included with reported DPS at any time point (n = 4924) or 'never' DPS (n = 4747). The majority (86.9%) were female and mean age was 55.7 years. DPS were associated with longer disease and Raynaud's duration (both P = <0.001). DPS were associated with interstitial lung disease, pulmonary hypertension, conduction blocks, telangiectases, calcinosis (all P = <0.001) and joint synovitis (P = <0.021). Patients were more likely to have more severe capillaroscopic abnormality and greater hand functional impairment. Multivariable logistic regression analyses showed that DPS were associated (OR) with DUs: 22.03 (19.51 to 24.87), active digital ischaemia: 6.30 (5.34 to 7.42), and death: 1.86 (1.48 to 2.36).

Conclusion: DPS are associated with a severe disease course including death. The impact of DPS on hand function and ischaemia is significant. The presence of DPS should alert the clinician to a poor prognosis and need to optimise the therapeutic strategy.
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http://dx.doi.org/10.1093/rheumatology/keab510DOI Listing
June 2021

A Practical Approach to the Management of Digital Ulcers in Patients With Systemic Sclerosis: A Narrative Review.

JAMA Dermatol 2021 Jul;157(7):851-858

Department of Experimental and Clinical Medicine, University of Florence, Division of Rheumatology AOUC, Florence, Italy.

Importance: Digital ulcers (DUs) occurring on the fingers in patients with systemic sclerosis (SSc) are associated with substantial pain and disability and are often challenging to treat. However, careful clinical assessment and prompt intervention (wound bed management and systemic pharmacologic treatment) may modify the clinical course.

Objectives: To provide a practical approach to the assessment and management of SSc-DUs and highlight unmet needs and research priorities.

Evidence Review: A narrative review of the extant literature was undertaken to provide a broad overview of current knowledge and augmented by expert opinion.

Findings: Half of the patients with SSc have a history of DUs, and there is a point of prevalence of approximately 10%. Digital ulcers are often very painful and affect all aspects of physical, social, and family life as well as occupation. Digital ulcers are associated with a severe disease course. Systemic sclerosis DUs, particularly those occurring on the fingertips, represent a vascular ischemic complication, although other etiopathogenic factors play an important role. To guide management, a structured clinical approach is required, including DU definition, classification, and categorization. Digital ulcers require a multidisciplinary approach with close cooperation between physicians and specialist nursing and other allied health professionals to guarantee the appropriate treatment and provide patient education. Local wound bed management is necessary for all DUs and is combined with systemic (pharmacologic) treatments. When treating a DU, the clinician should actively review the therapeutic strategy to prevent further DUs, including the level of systemic disease control, and monitor closely for the development of DU complications, including infection and progression to gangrene. Despite a wide available therapeutic armory, a number of unmet needs and challenges remain that that require resolution to optimize DU management.

Conclusions And Relevance: A practical approach to DU management, including local wound bed management and systemic treatments, is useful. Digital ulcers are of interest to a broad range of dermatologists, rheumatologists, and other physicians providing care for patients with SSc. Careful clinical assessment and prompt intervention can substantially modify the clinical course of DUs in SSc.
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http://dx.doi.org/10.1001/jamadermatol.2021.1463DOI Listing
July 2021

Baricitinib in the treatment of rheumatoid arthritis: clinical and ultrasound evaluation of a real-life single-centre experience.

Ther Adv Musculoskelet Dis 2021 10;13:1759720X211014019. Epub 2021 May 10.

Department Experimental and Clinical Medicine & Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, Firenze, Italy.

Background: Ultrasound (US) is useful in monitoring RA patients, with the US7 score allowing grey-scale and power-Doppler (PD) semi-quantitative evaluation of synovitis and teno-synovitis. We evaluated real-life efficacy and safety of Baricitinib, an oral selective JAK1-2 inhibitor, in RA patients using clinical, clinimetric, and US assessments.

Methods: Disease activity score in 28 joints calculated with C-reactive protein (DAS28-CRP), disease activity score in 28 joints calculated with erythrocyte sedimentation rate (DAS28-ESR), clinical disease activity index (CDAI), simplified disease activity index (SDAI), visual analogue scale (VAS)-pain, health assessment questionnaire (HAQ), COCHIN scale, adverse events (AE), concomitant medications, laboratory parameters, and US7 were performed/recorded at baseline, 1, 3, and 6 months in RA patients starting Baricitinib. Responder/non-responder status was determined according to the EULAR Response Criteria at 3 months. SDAI clinical remission or low disease activity (LDA) were calculated at 3 and 6 months.

Results: In 43 enrolled patients, a significant improvement in disease activity and US7 components (except tendon PD) and a reduction of steroid dosage were observed. Responders at 3 months showed a significantly higher reduction of CDAI, SDAI, COCHIN scale, VAS-pain, and US7 synovialPD, compared with non-responders. At 3 and 6 months, remission/LDA was achieved by 12.8/53.8% and 21.6/51.3% patients, respectively. The csDMARD co-treatment was independently associated with remission/LDA at 3 months. Safety-related drop-outs were in line with literature data. The steroid dosage was associated with AE development at 6 months.

Conclusion: The real-life data, also obtained with US evaluation, confirmed the Baricitinib efficacy in RA disease control, as well as the utility of assessment during the follow up of disease activity.
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http://dx.doi.org/10.1177/1759720X211014019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8120535PMC
May 2021

The target on B cells in Systemic Sclerosis: a "midsummer dream" to extinguish inflammation and prevent early disease progression to fibrosis.

Clin Rheumatol 2021 Jul 22;40(7):2529-2533. Epub 2021 May 22.

Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132, Milan, Italy.

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http://dx.doi.org/10.1007/s10067-021-05733-4DOI Listing
July 2021

Systematic Analysis of the Literature in Search of Defining Systemic Sclerosis Subsets.

J Rheumatol 2021 May 15. Epub 2021 May 15.

This work was supported by a grant from the Scleroderma Foundation and the World Scleroderma Foundation. SRJ is supported by a Canadian Institutes of Health Research New Investigator Award and the Gurmej Kaur Dhanda Scleroderma Research Award. DK was funded by the National Institutes of Health/ National Institute of Arthritis and Musculoskeletal and Skin Diseases grant 5K24AR063120-07. T. Nevskaya, MD, PhD, J.E. Pope, MD, MPH, M.A. Turk, MSc, J. Shu, MD, HBSc, Schulich School of Medicine & Dentistry, Western University, London, Ontario, Canada; A. Marquardt, DO, D. Khanna, MD, MS, University of Michigan, Ann Arbor, Michigan, USA; F. van den Hoogen, MD, PhD, St. Maartenskliniek and Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands; J. Fransen, MSc, PhD, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands; M. Matucci-Cerinic, MD, PhD, Department of Experimental and Clinical Medicine & Division of Rheumatology AOUC, Florence Italy University of Florence, Florence, Italy; M. Baron, MD, McGill University, Division Head Rheumatology, Jewish General Hospital, Montreal, Quebec, Canada; C.P. Denton, FRCP, PhD, University College London, Division of Medicine, London, UK; S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Toronto Western and Mount Sinai Hospitals, Department of Medicine, and Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. The authors declare no conflicts of interest relevant to this article. Address correspondence to Dr. S.R. Johnson, Toronto Scleroderma Program, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON M5T 2S8, Canada. Email: Accepted for publication April 26, 2021.

Objective: Systemic sclerosis (SSc) is a multisystem disease with heterogeneity in presentation and prognosis. An international collaboration to develop new SSc subset criteria is underway. Our objectives were to identify systems of SSc subset classification and synthesize novel concepts to inform development of new criteria.

Methods: Medline, Cochrane MEDLINE, the Cumulative Index to Nursing and Allied Health Literature, EMBASE, and Web of Science were searched from their inceptions to December 2019 for studies related to SSc subclassification, limited to humans and without language or sample size restrictions.

Results: Of 5686 citations, 102 studies reported original data on SSc subsets. Subset classification systems relied on extent of skin involvement and/or SSc-specific autoantibodies (n = 61), nailfold capillary patterns (n = 29), and molecular, genomic, and cellular patterns (n = 12). While some systems of subset classification confer prognostic value for clinical phenotype, severity, and mortality, only subsetting by gene expression signatures in tissue samples has been associated with response to therapy.

Conclusion: Subsetting on extent of skin involvement remains important. Novel disease attributes including SSc-specific autoantibodies, nailfold capillary patterns, and tissue gene expression signatures have been proposed as innovative means of SSc subsetting.
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http://dx.doi.org/10.3899/jrheum.201594DOI Listing
May 2021

Comparison of three treatment protocols with intra-articular low or intermediate molecular weight hyaluronic acid in early symptomatic knee osteoarthritis.

Ther Adv Musculoskelet Dis 2021 20;13:1759720X21994024. Epub 2021 Apr 20.

Department of Clinical and Experimental Medicine, University of Florence and Department of Geriatric Medicine, Div. of Rheumatology AOUC, SOD Reumatologia, via delle Oblate 4, Firenze (FI), 50139, Italy.

Introduction: Viscosupplementation with hyaluronic acid (HA) is indicated for non-responders to non-pharmacological therapy, to analgesics or when non-steroidal anti-inflammatory drugs (NSAIDs) are contraindicated. The aim of this study is to compare the efficacy, safety and costs of three different HA treatments (, sinovial one and hyalgan).

Patients And Methods: Ninety patients with grade I/II Kellgren-Lawrence knee osteoarthritis were included in three groups, the first was treated with hyalgan (weekly for 5 weeks), the second with Sinovial® Forte (weekly for 3 weeks) and the third group with a single injection of sinovial one.

Results: All three treatments were effective, with an average reduction in the Western Ontario and McMaster Universities osteoarthritis index (WOMAC) score of 18.9 points for hyalgan, 18.04 points for and 17.92 points for sinovial one. The comparison of the three groups did not show any statistical difference in terms of efficacy. National health system (NHS) and social costs are, respectively, €419.12 and €853.43 for hyalgan, €338.64 and €599.22 for , €221.56 and €308.42 for sinovial one.

Conclusion: All three treatments were equally effective with no statistically significant differences; thus, the treatment with sinovial one may be considered as clinically effective as the other two regimens, but with a very efficient cost profile in early symptomatic knee osteoarthritis.
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http://dx.doi.org/10.1177/1759720X21994024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8064515PMC
April 2021

Systemic sclerosis and COVID-19: what's new in the literature.

Clin Exp Rheumatol 2021 Jul-Aug;39 Suppl 131(4):157-158. Epub 2021 May 3.

Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Firenze, Italy.

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August 2021

New promising drugs for the treatment of systemic sclerosis: pathogenic considerations, enhanced classifications, and personalized medicine.

Expert Opin Investig Drugs 2021 Jun 13;30(6):635-652. Epub 2021 May 13.

Department of internal medicine, Division of rheumatology and Michigan Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA.

: Systemic sclerosis (SSc), also known as scleroderma, is a complex orphan disease characterized by early inflammatory features, vascular hyper-reactivity, and fibrosis of the skin and internal organs. Although substantial progress has been made in the understanding of the pathogenesis of SSc, there is still no disease-modifying drug that could significantly impact the natural history of the disease.: This review discusses the rationale, preclinical evidence, first clinical eevidence,and pending issues concerning new promising therapeutic options that are under investigation in SSc. The search strategy was based on PubMed database and clinical trial.gov, highlighting recent key pathogenic aspects and phase I or II trials of investigational drugs in SSc.: The identification of new molecular entities that potentially impact inflammation and fibrosis may constitute promising options for a disease modifying-agent in SSc. The early combinations of antifibrotic drugs (such as pirfenidone) with immunomodulatory agents (such as mycophenolate mofetil) may also participate to achieve such a goal. A more refined stratification of patients, based on clinical features, molecular signatures, and identification of subpopulations with distinct clinical trajectories, may also improve management strategies in the future.
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http://dx.doi.org/10.1080/13543784.2021.1923693DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8292968PMC
June 2021

Interleukin-1 and Systemic Sclerosis: Getting to the Heart of Cardiac Involvement.

Front Immunol 2021 23;12:653950. Epub 2021 Mar 23.

Department of Experimental and Clinical Medicine, University of Florence, and Division of Rheumatology AOUC, Florence, Italy.

Systemic sclerosis (SSc) is rare, severe connective tissue disease characterized by endothelial and vascular damage, immune activation, and resulting in inflammation and fibrosis of skin and internal organs, including the heart. SSc is associated with high morbidity and mortality. Cardiac involvement is frequent in SSc patients, even though often asymptomatic at early stages, and represents one of the major causes of SSc-related mortality. Heart involvement has a variable clinical presentation, and its pathogenesis is not completely understood. Myocardial fibrosis is traditionally considered the immunopathologic hallmark of heart involvement in SSc. This unique histological feature is paralleled by distinctive clinical and prognostic features. The so-called "vascular hypothesis" represents the most credited hypothesis to explain myocardial fibrosis. More recently, the prominent role of an inflammatory myocardial process has been identified as a cardinal event in the evolution to fibrosis, thus also delineating an "inflammation-driven pathway to fibrosis". The pro-inflammatory cytokine interleukin (IL)-1 has an apical and cardinal role in the myocardial inflammatory cascade and in cardiac dysfunction. The primary aim of this perspective article is: to present the emerging evidence on the role of IL-1 and inflammasome in both SSc and heart inflammation, to review the complex interplay between cellular metabolism and inflammasome activation, and to discuss the rationale for targeted inhibition of IL-1 for the treatment of SSc-heart involvement, providing preliminary experimental and clinical data to support this hypothesis.
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http://dx.doi.org/10.3389/fimmu.2021.653950DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8021854PMC
March 2021

The Role of Pro-fibrotic Myofibroblasts in Systemic Sclerosis: from Origin to Therapeutic Targeting.

Curr Mol Med 2021 Mar 24. Epub 2021 Mar 24.

Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, Florence. Italy.

Systemic sclerosis (SSc, scleroderma) is a complex connective tissue disorder characterized by multisystem clinical manifestations resulting from immune dysregulation/autoimmunity, vasculopathy and, most notably, progressive fibrosis of the skin and internal organs. In recent years, it has emerged that the main drivers of SSc-related tissue fibrosis are myofibroblasts, a type of mesenchymal cells with both the extracellular matrix-synthesizing features of fibroblasts and the cytoskeletal characteristics of contractile smooth muscle cells. The accumulation and persistent activation of pro-fibrotic myofibroblasts during SSc development and progression result into elevated mechanical stress and reduced matrix plasticity within the affected tissues and may be ascribed to a reduced susceptibility of these cells to pro-apoptotic stimuli, as well as their increased formation from tissue-resident fibroblasts or transition from different cell types. Given the crucial role of myofibroblasts in SSc pathogenesis, finding the way to inhibit myofibroblast differentiation and accumulation by targeting their formation, function and survival may represent an effective approach to hamper the fibrotic process or even halt or reverse established fibrosis. In this review, we discuss the role of myofibroblasts in SSc-related fibrosis, with a special focus on their cellular origin and the signaling pathways implicated in their formation and persistent activation. Furthermore, we provide an overview of potential therapeutic strategies targeting myofibroblasts that may be able to counteract fibrosis in this pathological condition.
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http://dx.doi.org/10.2174/0929867328666210325102749DOI Listing
March 2021

Prognostic role of KL-6 in SSc-ILD patients with pleuroparenchymal fibroelastosis.

Eur J Clin Invest 2021 Aug 23;51(8):e13543. Epub 2021 Mar 23.

Respiratory Diseases and Lung Transplantation, Department of Medical and Surgical Sciences & Neurosciences, Siena University Hospital, Siena, Italy.

Background: Krebs von den Lungen-6 (KL-6) is a high-molecular-weight (200kDa) glycoprotein proposed as a diagnostic biomarker for differentiating interstitial lung disease (ILD). Systemic sclerosis (SSc) is a rare immune-mediated disorder, and ILD is the leading cause of morbidity and mortality. Pleuroparenchymal fibroelastosis (PPFE) has been described to have a poor prognosis in SSc-ILD patients. This study undertook to compare serial changes in KL-6 in SSc-ILD patients with and without PPFE, to verify its prognostic value as a disease biomarker.

Materials And Methods: Twenty-five SSc-ILD patients (median IQR, 62 (56-58); 20% males) were retrospectively enrolled. 12 SSc-ILD patients (48%) had also a radiological diagnosis of PPFE. Serum KL-6 concentrations were measured by KL-6 reagent assay (Fujirebio Europe, Ghent, Belgium).

Results: Serum KL-6 measurements were increased in SSc-ILD patients with and without PPFE compared with healthy controls (P < .0001). Comparative analysis of the rate of variation of KL-6 over the 6 years of follow-up was performed by serial two-yearly KL-6 measurements: Δ1(t1-t0), Δ2(t2-t1) and Δ3(t3-t2). In SSc-ILD patients with PPFE pattern, Δ3 was significantly different than those without PPFE pattern (P = .0020). Serum KL-6 levels were significantly different (P = .0455) either at Δ2 and Δ3 in the PPFE group. In SSc-ILD patients with PPFE, at t3 serum KL-6 concentrations were inversely correlated with FEV1 (r = -.76; P = .037) and FVC percentages (r = -.79; P = .028).

Conclusion: These results suggest that serial measurements of KL-6 in the follow-up of these patients may help to monitor disease progression. In real life, in SSc-ILD patients PPFE should be always evaluated at CT and when present should suggest a tight follow-up to monitor its evolution.
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http://dx.doi.org/10.1111/eci.13543DOI Listing
August 2021

Author Correction: Raynaud phenomenon and digital ulcers in systemic sclerosis.

Nat Rev Rheumatol 2021 Apr;17(4):246

Division of Rheumatology, University of Florence, Florence, Italy.

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http://dx.doi.org/10.1038/s41584-021-00591-5DOI Listing
April 2021

Arthritis after SARS-CoV-2 infection.

Lancet Rheumatol 2021 May 16;3(5):e324-e325. Epub 2021 Mar 16.

Department of Experimental and Clinical Medicine, University of Florence, 50143 Florence, Italy.

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http://dx.doi.org/10.1016/S2665-9913(21)00067-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7963449PMC
May 2021

ANCA in systemic sclerosis, when vasculitis overlaps with vasculopathy: a devastating combination of pathologies.

Rheumatology (Oxford) 2021 Mar 21. Epub 2021 Mar 21.

Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy.

In patients with systemic sclerosis (SSc), the coexistence of ANCA-associated vasculitis (SSc-AAV) has been reported to be associated with a severe disease course, including significant pulmonary and renal involvement. The presence of ANCA is not uncommon in patients with SSc and therefore clinicians must maintain a high index of clinical suspicion about SSc-AAV. p-ANCA and anti-MPO antibodies are the most common antibodies observed. Patients typically present with clinical features of microscopic polyangiitis or renal-limited vasculitis There are multiple areas of potential interaction in the pathogenesis of SSc and AAV which can exacerbate/compound vascular disease. In addition, similar patterns of major internal organ involvement (e.g., lung and kidneys) are seen in both conditions. We highlight a diagnostic approach to SSc-AAV and the paucity of data to inform management. As such, SSc-AAV is typically treated as per isolated AAV which can potentially be hazardous in patients with SSc (e.g., the association between high-dose steroid and scleroderma renal crisis). We propose that this rare clinical entity warrants rigorous investigation including definition of a therapeutic strategy to ameliorate the potentially devastating combination of pathologies in SSc-AAV.
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http://dx.doi.org/10.1093/rheumatology/keab278DOI Listing
March 2021

Combined lung and cardiac ultrasound in COVID-related acute respiratory distress syndrome.

Intern Emerg Med 2021 Mar 11. Epub 2021 Mar 11.

Intensive Care Unit and Regional ECMO Referral Centre, Azienda Ospedaliero-Universitaria Careggi, Largo Brambilla 3, 50134, Florence, Italy.

Background: Lung ultrasound (LU) is a useful tool for monitoring lung involvement in novel coronavirus (COVID) disease, while information on echocardiographic findings in COVID disease is to date scarce and heterogeneous. We hypothesized that lung and cardiac ultrasound examinations, serially and simultaneously performed, could monitor disease severity in COVID-related ARDS.

Methods: We enrolled 47 consecutive patients with COVID-related ARDS (1st March-31st May 2020). Lung and cardiac ultrasounds were performed on admission, at discharged and when clinically needed.

Results: Most patients were mechanically ventilated (75%) and veno-venous extracorporeal membrane oxygenation was needed in ten patients (21.2%). The in-ICU mortality rate was 27%%. On admission, not survivors showed a higher LUS score (p = 0.006) and a higher incidence of consolidations (p = 0.003), lower values of LVEF (p = 0.027) and a higher RV/LV ratio (0.008). At discharge, a significant reduction in the incidence of subpleural consolidations (p < 0.001) and, thus, in LUS score (p < 0.001) and an increase in patter A findings (p < 0.001) together with reduced systolic pulmonary arterial pressures were detectable. In not survivors at final examination, an increased in LUS score (p < 0.001), and in RV/LV ratio (p < 0.001) associated with a reduction in TAPSE (p = 0.013) were observed. A significant correlation was observed between LUS and systolic pulmonary arterial pressure (p = 0.04). LUS and RV/LV resulted independent predictors of in-ICU death.

Conclusions: In COVID-related ARDS, the combined lung and cardiac ultrasound proved to be an useful clinical tool in monitoring disease progression and in identifying parameters (LU score and RV/LV ratio) able to risk stratifying these patients.
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http://dx.doi.org/10.1007/s11739-021-02646-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7947148PMC
March 2021

Ultrasonographic imaging of systemic sclerosis digital ulcers: A systematic literature review and validation steps.

Semin Arthritis Rheum 2021 04 19;51(2):425-429. Epub 2021 Feb 19.

Department of Medicine, Rheumatology Division, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, United States; Department of Experimental and Clinical Medicine, University of Florence, and Division of Rheumatology AOUC, Florence, Italy; Department of Rheumatology, University of Washington, Seattle, WA, United States. Electronic address:

Introduction/background: Skin ulcers are a complex array of clinical manifestations of systemic sclerosis (SSc) and are often difficult to treat. However, the definition of SSc-skin ulcers has to date not been promising, demonstrating poor reliability and accuracy. There are emerging data that ultrasound has significant potential to evaluate SSc-skin ulcers.

Objective: To perform a systematic literature review (SLR) to understand the degree to which ultrasound of SSc skin ulcers has been validated according to OMERACT criteria.

Methods: In a SLR, we investigated the Cochrane Library, Web of Science and Pubmed databases for manuscripts from inception to 1st April 2020. Inclusion and exclusion criteria included manuscripts on SSc patients aged over 16 years, performing SSc-related skin ulcer evaluation with ultrasound machines. Papers on animal model, diseases other than SSc, venous ulcers were excluded. Data extraction used a uniform case report form which collected data on patient demographics, disease activity, description of the ultrasound machine and procedures and the degree to which domains of validity were fulfilled. Manuscript evaluation and extraction was performed by two independent assessors, with a third author being consulted in case of disagreement.

Results: amongst 308 manuscripts that were identified, 6 published manuscripts/ posters fulfilled the inclusion/exclusion criteria and were extracted. Face validity was found. Three studies developed an US definition of SSc-ulcers across patients (content validity); one study evaluated the concordance between US image and clinical assessment. Criterion validity was shown by one study and ultrasound detected improvement (sensitivity to change) of SSc-skin ulcer in response to therapy. Feasibility was demonstrated by US use for skin ulcers in multiple settings (the 6 manuuscripts/posters).

Conclusion: This systematic literature review shows that ultrasound for skin ulcers in SSc has been partially validated. It has face, content, criterion validity, responsiveness and reasonable feasibility. Further validation for construct validity, reliability and discrimination is required.
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http://dx.doi.org/10.1016/j.semarthrit.2021.02.008DOI Listing
April 2021
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