Publications by authors named "Silvia Bellando-Randone"

84 Publications

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

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

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

The clinical phenotype of Systemic Sclerosis patients with anti-PM/Scl antibodies: results from the EUSTAR cohort.

Rheumatology (Oxford) 2021 Feb 12. Epub 2021 Feb 12.

Division of Rheumatology, Hospital IRCCS Policlinico S. Matteo Foundation of Pavia, University of Pavia, Pavia, Italy.

Objective: To evaluate clinical associations of anti-PM/Scl antibodies in patients with Systemic Sclerosis (SSc) in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (SRC), malignancies, and functional outcome of interstitial lung disease (ILD).

Methods: (1) Analysis of SSc patients from the EUSTAR database: 144 anti-PM/Scl+ without SSc-specific autoantibodies were compared to 7,202 anti-PM/Scl-, and then to 155 anti-Pm/Scl+ with SSc-specific antibodies. (2) Case-control study: additional data were collected for 165 anti-PM/Scl+ SSc (85 from the EUSTAR registry), and compared to 257 anti-PM/Scl- SSc controls, matched for sex, cutaneous subset, disease duration, and age at SSc onset.

Results: Patients with isolated anti-PM/Scl positivity, as compared with anti-Pm/Scl-, had higher frequency of muscle involvement, ILD, calcinosis and cutaneous signs of dermatomyositis, but similar frequency of SRC and malignancies (either synchronous with SSc onset or not). The presence of muscle involvement was associated with a more severe disease phenotype. Although very frequent, ILD had a better functional outcome in cases than in controls.In patients with both anti-PM/Scl and SSc-specific antibodies, a higher frequency of typical SSc features than in those with isolated anti-PM/Scl was observed.

Conclusion: The analysis of the largest series of anti-PM/Scl+ SSc patients so far reported helps to delineate a specific clinical subset with muscle involvement, cutaneous dermatomyositis, calcinosis, and ILD characterized by a good functional outcome. SRC and malignancies do not seem to be part of this syndrome.
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http://dx.doi.org/10.1093/rheumatology/keab152DOI Listing
February 2021

Digital Ulcers in Systemic Sclerosis.

Presse Med 2021 Feb 3;50(1):104064. Epub 2021 Feb 3.

Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Firenze, & Department of Geriatric Medicine, Division of Rheumatology AOUC, Firenze, Italy. Electronic address:

Digital ulcers (DU) are one of the most common complication of Systemic Sclerosis (SSc)-related vasculopathy and represent an important burden for the patients as well as for the society. Still today there is no agreement on the definition, classification and cathegorization of DU even if they are of pivotal importance in clinical practice, for treatment choice and prognostic outcomes, as well as for clinical trials. DU management requires a dedicated multidisciplinary team, that must remain ever vigilant for the development of infective complications and gangrene throughout their disease course, as well as patient education that is crucial to obtain the best compliance to assure the success of the treatment. Currently several drugs are available for DU treatment but in the future, more investigations will be needed to ameliorate the approach and the systemic and local therapies.
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http://dx.doi.org/10.1016/j.lpm.2021.104064DOI Listing
February 2021

Glycolysis-derived acidic microenvironment as a driver of endothelial dysfunction in systemic sclerosis.

Rheumatology (Oxford) 2021 Jan 20. Epub 2021 Jan 20.

Department of Experimental and Clinical Biomedical Sciences "Mario Serio", Section of Experimental Pathology and Oncology, University of Florence, Florence, Italy.

Objectives: Systemic sclerosis (SSc) is an autoimmune disease characterized by peripheral vasculopathy and skin and internal organ fibrosis. Accumulating evidence underlines a close association between a metabolic reprogramming of activated fibroblasts and fibrosis. This prompted us to determine the metabolism of SSc dermal fibroblasts and the effect on the vasculopathy characterizing the disease.

Methods: Seahorse XF96 Extracellular Flux Analyzer was exploited to evaluate SSc fibroblast metabolism. In vitro invasion and capillary morphogenesis assays were used to determine the angiogenic ability of endothelial cells (EC). Immunofluorescence, flow cytometer and real time PCR techniques provided evidence of the molecular mechanism behind the impaired vascularization that characterizes SSc patients.

Results: SSc fibroblasts, compared with control, showed a boosted glycolytic metabolism with increased lactic acid release and subsequent extracellular acidification, that in turn was found to impair EC invasion and organization in capillary-like networks without altering cell viability. A molecular link between extracellular acidosis and endothelial dysfunction was identified as acidic EC up-regulated MMP-12 which cleaves and inactivates uPAR, impairing angiogenesis in SSc. Moreover, the acidic environment was found to induce the loss of endothelial markers and the acquisition of mesenchymal-like features in EC, thus promoting the endothelial-to-mesenchymal transition (EndoMT) process that contributes to both capillary rarefaction and tissue fibrosis in SSc.

Conclusion: This study disclosed a liaison among the metabolic reprogramming of SSc dermal fibroblasts, extracellular acidosis and endothelial dysfunction that may contribute to the impairment and loss of peripheral capillary networks in SSc disease.
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http://dx.doi.org/10.1093/rheumatology/keab022DOI Listing
January 2021

Pleuroparenchymal fibroelastosis in rheumatic autoimmune diseases: a systematic literature review.

Rheumatology (Oxford) 2020 Dec;59(12):3645-3656

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

Objectives: Pleuroparenchymal fibroelastosis (PPFE) is characterized by predominantly upper lobe pleural and subjacent parenchymal fibrosis; PPFE features were described in patients with rheumatic autoimmune diseases (RAID). A systematic literature review was performed to investigate the prevalence, prognosis and potential association of PPFE with previous immunosuppression in RAID.

Methods: EMBASE, Web of Science and PubMed databases were questioned from inception to 1 September 2019. Articles published in English and addressing PPFE in patients with RAID were selected.

Results: Twenty out of 794 papers were selected with a total of 76 cases of RAID-PPFE patients (20 SSc, 9 RA, 6 IIM6 primary SS, 5 overlap syndromes, 3 ANCA-associated vasculitides, 2 granulomatosis with polyangiitis, 1 microscopic polyangiitis, 1 UCTD, 1 SLE, 1 GCA and 21 patients with non-specified RAID). Dyspnoea was the most frequently reported symptom (37/48 patients, 77%). Patients frequently presented with a restrictive pattern and decline in diffusing lung capacity for carbon monoxide. During the follow-up, 7/12 patients had progression at imaging, 22/39 presented a generic clinical worsening, 19/38 had a functional deterioration and 15/43 remained stable.

Conclusion: The present systematic literature review confirms that PPFE features are present in RAID. Rheumatologists should be aware of this new radiological pattern that holds a bad prognosis.
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http://dx.doi.org/10.1093/rheumatology/keaa451DOI Listing
December 2020

Mycosis fungoides: from early stages to fatal central nervous system involvement.

G Ital Dermatol Venereol 2020 Nov 23. Epub 2020 Nov 23.

Department of Experimental and Clinical Medicine, AOU Careggi Hospital, University of Florence Medical School, Florence, Italy.

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http://dx.doi.org/10.23736/S0392-0488.20.06537-2DOI Listing
November 2020

Lung magnetic resonance imaging in systemic sclerosis: a new promising approach to evaluate pulmonary involvement and progression.

Clin Rheumatol 2021 May 7;40(5):1903-1912. Epub 2020 Nov 7.

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

Introduction/objectives: Interstitial lung disease (ILD) is frequent and highly disabling in systemic sclerosis (SSc). Magnetic resonance imaging (MRI) is not routinely used to evaluate the lung, due to poorer spatial resolution compared to high-resolution computed tomography (HRCT). We aimed to compare lung MRI signal with HRCT and evaluate the role of MRI in predicting ILD progression.

Methods: Thirty SSc patients underwent lung MRI and HRCT. STIR and T1 mapping sequences were acquired before and after gadolinium injection. Patients were classified as normal (group 1 with normal HRCT and MRI), discordant (group 2 without ILD signs on HRCT but areas of hyperintensity on MRI), and abnormal (group 3 with ILD signs on HRCT and areas of hyperintensity on MRI). Patients were followed up for ILD progression.

Results: Mean STIR and T1 values were different between the three groups (p < 0.0001). STIR values correlated with HRCT score (R = 0.79, p < 0.0001), lung ultrasound B-lines (R = 0.73, p < 0.0001), and %DLco (R = - 0.63, p = 0.0001). Nine events were recorded during a follow-up of 25 ± 20 months. Continuous STIR values were independently associated with events (HR 1.018; CI 1.005-1.031, p = 0.005). A STIR value >90 ms discriminated patients at a higher risk of worsening pulmonary involvement (HR 8.80; CI 1.81-42.74; p < 0.007).

Conclusions: Lung MRI can detect SSc-related ILD, with good correlations with other ILD markers. STIR values, independently of HRCT appearance, may predict worsening lung involvement. Lung MRI, although very preliminary, is a promising tool that in a near future could help selecting patients for an early treatment of SSc-related ILD and a more appropriate use of HRCT. Key points • Lung MRI has the potential to differentiate inflammation-predominant versus fibrosis-predominant lesions, but it is not currently used in routine clinical practice to assess SSc-related ILD. • Lung MRI STIR and T1 values are significantly different between patients with and without SSc-related ILD. STIR values, independently of HRCT appearance, are also able to predict worsening lung involvement over time. • These preliminary data suggest that, in a near future, MRI could support the choice for an early treatment of SSc-related ILD, as well as a more appropriate use of HRCT.
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http://dx.doi.org/10.1007/s10067-020-05491-9DOI Listing
May 2021

COVID-19 and rheumatic autoimmune systemic diseases: report of a large Italian patients series.

Clin Rheumatol 2020 Nov 27;39(11):3195-3204. Epub 2020 Aug 27.

Department of Clinical and Experimental Medicine, Immuno-Endocrine Section of Internal Medicine, Laboratory of Primary Human Cells, School of Medicine, University of Pisa, Via Savi, 10, I-56126, Pisa, Italy.

Introduction: Covid-19 infection poses a serious challenge for immune-compromised patients with inflammatory autoimmune systemic diseases. We investigated the clinical-epidemiological findings of 1641 autoimmune systemic disease Italian patients during the Covid-19 pandemic.

Method: This observational multicenter study included 1641 unselected patients with autoimmune systemic diseases from three Italian geographical areas with different prevalence of Covid-19 [high in north (Emilia Romagna), medium in central (Tuscany), and low in south (Calabria)] by means of telephone 6-week survey. Covid-19 was classified as (1) definite diagnosis of Covid-19 disease: presence of symptomatic Covid-19 infection, confirmed by positive oral/nasopharyngeal swabs; (2) highly suspected Covid-19 disease: presence of highly suggestive symptoms, in absence of a swab test.

Results: A significantly higher prevalence of patients with definite diagnosis of Covid-19 disease, or with highly suspected Covid-19 disease, or both the conditions together, was observed in the whole autoimmune systemic disease series, compared to "Italian general population" (p = .030, p = .001, p = .000, respectively); and for definite + highly suspected diagnosis of Covid-19 disease, in patients with autoimmune systemic diseases of the three regions (p = .000, for all comparisons with the respective regional general population). Moreover, significantly higher prevalence of definite + highly suspected diagnosis of Covid-19 disease was found either in patients with various "connective tissue diseases" compared to "inflammatory arthritis group" (p < .000), or in patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs treatments (p = .011).

Conclusions: The finding of a higher prevalence of Covid-19 in patients with autoimmune systemic diseases is particularly important, suggesting the need to develop valuable prevention/management strategies, and stimulates in-depth investigations to verify the possible interactions between Covid-19 infection and impaired immune-system of autoimmune systemic diseases. Key Points • Significantly higher prevalence of Covid-19 is observed in a large series of patients with autoimmune systemic diseases compared to the Italian general population, mainly due to patients' increased susceptibility to infections and favored by the high exposure to the virus at medical facilities before the restriction measures on individual movement. • The actual prevalence of Covid-19 in autoimmune systemic diseases may be underestimated, possibly due to the wide clinical overlapping between the two conditions, the generally mild Covid-19 disease manifestations, and the limited availability of virological testing. • Patients with "connective tissue diseases" show a significantly higher prevalence of Covid-19, possibly due to deeper immune-system impairment, with respect to "inflammatory arthritis group". • Covid-19 is more frequent in the subgroup of autoimmune systemic diseases patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs, mainly hydroxyl-chloroquine and methotrexate, which might play some protective role against the most harmful manifestations of Covid-19.
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http://dx.doi.org/10.1007/s10067-020-05334-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7450255PMC
November 2020

The Role of Endogenous Eicosapentaenoic Acid and Docosahexaenoic Acid-Derived Resolvins in Systemic Sclerosis.

Front Immunol 2020 19;11:1249. Epub 2020 Jun 19.

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

Resolvins, the member of specialized pro-resolving mediators, are produced from omega-3 polyunsaturated fatty acids as a response to an acute inflammatory process in that termination and resolution of inflammation. In the acute inflammation, these lipid mediators limit polymorphonuclear cells infiltration, proinflammatory cytokine production; promote efferocytosis, and regulate several cell types being important roles in innate and adaptive immunity. Any dysregulation or defect of the resolution phase result in prolonged, persistent inflammation and eventually fibrosis. Resolvins are implicated in the development of various chronic autoimmune diseases. Systemic sclerosis (SSc) is a very complicated, chronic autoimmune disorder proceeding with vasculopathy, inflammation, and fibrosis. Dysregulation of innate and adaptive immunity is another important contributing factor in the pathogenesis of SSc. In this review, we will focus on the different roles of this new family of lipid mediators, characterized by the ability to prevent the spread of inflammation and its chronicity in various ways and how they can control the development of fibrotic diseases like SSc.
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http://dx.doi.org/10.3389/fimmu.2020.01249DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7318896PMC
April 2021

Quantitative analysis of pulmonary vasculature in systemic sclerosis at spirometry-gated chest CT.

Ann Rheum Dis 2020 09 30;79(9):1210-1217. Epub 2020 Jun 30.

Dept Experimental and Clinical Medicine, University of Florence, Florence, Italy.

Objective: To prospectively investigate whether differences in pulmonary vasculature exist in systemic sclerosis (SSc) and how they are distributed in patients with different pulmonary function.

Methods: Seventy-four patients with SSc undergoing chest CT scan for interstitial lung disease (ILD) screening or follow-up were prospectively enrolled. A thorough clinical, laboratory and functional evaluation was performed the same day. Chest CT was spirometry gated at total lung capacity and images were analysed by two automated software programs to quantify emphysema, ILD patterns (ground-glass, reticular, honeycombing), and pulmonary vascular volume (PVV). Patients were divided in restricted (FVC% <80, DLco%<80), isolated DLco% reduction (iDLco- FVC%≥80, DLco%<80) and normals (FVC%≥80, DLco%≥80). Spearman ρ, Mann-Whitney tests and logistic regressions were used to assess for correlations, differences among groups and relationships between continuous variables.

Results: Absolute and lung volume normalised PVV (PVV/LV) correlated inversely with functional parameters and positively with all ILD patterns (ρ=0.75 with ground glass, ρ=0.68 with reticular). PVV/LV was the only predictor of DLco at multivariate analysis (p=0.007). Meanwhile, the reticular pattern prevailed in peripheral regions and lower lung thirds, PVV/LV prevailed in central regions and middle lung thirds. iDLco group had a significantly higher PVV/LV (2.2%) than normal (1.6%), but lower than restricted ones (3.8%).

Conclusions: Chest CT in SSc detects a progressive increase in PVV/LV as DLco decreases. Redistribution of perfusion to less affected lung regions rather than angiogenesis nearby fibrotic lung may explain the results. Further studies to ascertain whether the increase in PVV/LV reflects a real increase in blood volume are needed.
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http://dx.doi.org/10.1136/annrheumdis-2020-217359DOI Listing
September 2020

The systemic sclerosis patient in the COVID-19 era: the challenging crossroad between immunosuppression, differential diagnosis and long-term psychological distress.

Clin Rheumatol 2020 Jul 8;39(7):2043-2047. Epub 2020 Jun 8.

Department of Experimental and Clinical Medicine, Department of Geriatric Medicine, Division of Rheumatology AOUC & Scleroderma Unit, University of Florence, Florence, Italy.

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.
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http://dx.doi.org/10.1007/s10067-020-05193-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7276334PMC
July 2020

Effect of Dysmetabolisms and Comorbidities on the Efficacy and Safety of Biological Therapy in Chronic Inflammatory Joint Diseases.

J Clin Med 2020 May 2;9(5). Epub 2020 May 2.

Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Via delle Oblate 4, 50141 Florence, Italy.

In the present study we evaluated how systemic arterial hypertension (SAH), dyslipidemia and diabetes mellitus influence the efficacy, safety and retention rate of biological disease-modifying anti-rheumatic drug (bDMARD) treatment in rheumatic musculoskeletal disorders (RMDs). The charts of RMD patients treated with the first-line bDMARD were reviewed, collecting data on safety, efficacy and comorbidities at prescription (baseline, BL), after 6 months (6M) and at last observation on bDMARD (last observation time, LoT). In 383 RMD patients, a higher rate of adverse events at 6M ( = 0.0402) and at LoT ( = 0.0462) was present in dyslipidemic patients. Patients who developed dyslipidemia or SAH during bDMARD treatment had similar results (dyslipidemia = 0.0007; SAH = 0.0319) with a longer bDMARD retention as well (dyslipidemia < 0.0001; SAH < 0.0001). SAH patients on angiotensin converting enzyme inhibitors (ACEis) or angiotensin-II receptor blockers (ARBs) continued bDMARDs for longer than non-exposed patients ( = 0.001), with higher frequency of drug interruption for long-standing remission rather than inefficacy or adverse reactions ( = 0.0258). Similarly, dyslipidemic patients on statins had a better bDMARD retention than not-exposed patients ( = 0.0420). In conclusion, SAH and dyslipidemia may be associated with higher frequency of adverse events but a better drug retention of first-line bDMARD in RMDs, suggesting an additional effect of ACEis/ARBs or statins on the inflammatory process and supporting their use in RMD bDMARD patients with SAH/dyslipidemia.
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http://dx.doi.org/10.3390/jcm9051310DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290363PMC
May 2020

Prognostic Value of Lung Ultrasound B-Lines in Systemic Sclerosis.

Chest 2020 10 29;158(4):1515-1525. Epub 2020 Apr 29.

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

Background: A high percentage of systemic sclerosis (SSc) patients experience interstitial lung disease (ILD) during the disease course. Recent data have shown that lung ultrasound (LUS) can assess ILD by the evaluation of B-lines, the sonographic sign of pulmonary interstitial involvement.

Research Question: To establish the prognostic value of B-lines in a large number of patients with SSc.

Study Design And Methods: A total of 396 consecutive patients with SSc, who were enrolled at three Rheumatology Departments, underwent a comprehensive LUS examination on the anterolateral and posterior chest for a total of 58 scanning sites. All available clinical, imaging, and functional data were recorded. Patients were followed after enrolment to establish the prognostic role of LUS.

Results: The median number of B-lines was higher in patients with the diffuse cutaneous subset (44 vs 17 B-lines; P < .0001), topoisomerase I autoantibodies (39 vs 16 B-lines; P < .0001), and the presence of ILD at chest high-resolution CT (45 vs 9 B-lines; P < .0001). At multivariable analysis, the number of posterior B-lines ≥5 was associated with new development or worsening ILD (hazard ratio, 3.378; 95% CI, 1.137-9.994; P = .028), with additional value over topoisomerase I positivity. The prognostic value was further confirmed in the subgroup of patients with known ILD at baseline (hazard ratio, 1.010; 95% CI, 1.003-1.018; P = .008).

Interpretation: Lung ultrasound B-lines are associated with worsening or development of pulmonary deterioration. In the near future, LUS might become part of the diagnostic and prognostic armamentarium in patients with SSc, which would allow a more sustainable and user-friendly approach to this very fragile population.
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http://dx.doi.org/10.1016/j.chest.2020.03.075DOI Listing
October 2020

Systemic sclerosis Progression INvestiGation (SPRING) Italian registry: demographic and clinico-serological features of the scleroderma spectrum.

Clin Exp Rheumatol 2020 May-Jun;38 Suppl 125(3):40-47. Epub 2020 Apr 14.

Istituto Clinico Humanitas, Rozzano, Milano, Italy.

Objectives: Systemic sclerosis (SSc) is a severe multiple-organ disease characterised by unpredictable clinical course, inadequate response to treatment, and poor prognosis. National SSc registries may provide large and representative patients cohorts required for descriptive and prognostic studies. Therefore, the Italian Society for Rheumatology promoted the registry SPRING (Systemic sclerosis Progression INvestiGation).

Methods: The SPRING is a multi-centre rheumatological cohort study encompassing the wide scleroderma spectrum, namely the primary Raynaud's phenomenon (pRP), suspected secondary RP, Very Early Diagnosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteristics of a population of 2,028 Italian patients at the initial phase of enrolment, mainly focusing on the cohort of 1,538 patients with definite SSc.

Results: Definite SSc showed a significantly higher prevalence of digital ulcers, capillaroscopic 'late' pattern, oesophageal and cardio-pulmonary involvement compared to VEDOSS, as expected on the basis of the followed classification criteria. The in-depth analysis of definite SSc revealed that male gender, diffuse cutaneous subset, and anti-Scl70 seropositivity were significantly associated with increased prevalence of the most harmful disease manifestations. Similarly, patients with very short RP duration (≤1 year) at SSc diagnosis showed a statistically increased prevalence of unfavourable clinico-serological features.

Conclusions: Nationwide registries with suitable subsetting of patients and follow-up studies since the prodromal phase of the disease may give us valuable insights into the SSc natural history and main prognostic factors.
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September 2020

The Renal Resistive Index: A New Biomarker for the Follow-up of Vascular Modifications in Systemic Sclerosis.

J Rheumatol 2021 02 1;48(2):241-246. Epub 2020 Apr 1.

C. Bruni, MD, G. Lepri, MD, G. Tesei, MD, S. Guiducci, MD, D. Melchiorre, MD, S. Bellando-Randone, MD, M. Matucci-Cerinic, MD, Department of Experimental and Clinical Medicine, University of Florence, and Division of Rheumatology AOUC & Scleroderma Unit, Florence.

Objective: The aim of the present retrospective observational study was to evaluate the change of Renal Resistive Index (RRI) over time (ΔRRI) and under treatment in patients with systemic sclerosis (SSc) as well as to correlate these changes with disease complications.

Methods: Two hundred thirty patients [29 male, median age 57 (IQR 48-67) yrs] were enrolled. At baseline and follow-up (3.43, IQR 2.81-4.45 yrs), we collected the following data: disease variables, nailfold videocapillaroscopy (NVC) pattern, forced vital capacity (FVC), diffusing lung capacity for carbon monoxide (DLCO), systolic pulmonary arterial pressure (sPAP), presence of interstitial lung disease, RRI, evaluation of glomerular filtration rate, and new onset of pulmonary arterial hypertension (PAH).

Results: RRI value is high in SSc patients with digital ulcers and anticentromere antibodies, active and late NVC patterns, and limited cutaneous SSc. A significant correlation was observed between ΔRRI and ΔsPAP (R = 0.17, = 0.02), with statistically higher ΔRRI (0.08 ± 0.02 vs 0.03 ± 0.05, = 0.04) in patients complicated by PAH onset. No other new-onset complication was associated with ΔRRI. The receiver-operating characteristic curve analysis confirmed the predictive role of ΔRRI in development of new PAH (area under the curve 0.84, 95% CI 0.75-0.93, = 0.02). In patients with SSc never exposed to sildenafil, ΔRRI was higher (0.04 ± 0.05) compared to both patients exposed to sildenafil during the study period (0.01 ± 0.05, = 0.03) or in those exposed at the time of baseline evaluation (0.00 ± 0.05, = 0.01).

Conclusion: RRI and its variation in time are a reliable marker of SSc-related vasculopathy, both in renal and extrarenal compartments.
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http://dx.doi.org/10.3899/jrheum.191101DOI Listing
February 2021

Alveolar haemorrhage in ANCA-associated vasculitis: Long-term outcome and mortality predictors.

J Autoimmun 2020 03 9;108:102397. Epub 2020 Jan 9.

Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy.

Introduction: Alveolar haemorrhage (AH) is considered an important cause of morbidity and early mortality in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV).

Objectives: The aim of this study was to identify predictors of outcome in patients with AH-AAV and to evaluate outcome and causes of death in this subset.

Materials And Methods: A multicenter retrospective study was conducted in 29 Italian Centers. Clinicians were asked to recruit all patients diagnosed with AAV-associated AH during the last 10 years, from 2007 to 2016. Univariate and multivariable analysis were performed.

Results: One-hundred and six patients were included (median age at onset of 55 years [IQR 42-67]). The majority were ANCA-positive (PR3 57.1%, MPO 33.7%) and 72.6% had also renal involvement. At presentation, anaemia was shown in 97 (92.4%) patients, hemoptysis in 54 (51.9%), respiratory failure in 68 (66.7%), of whom 48 (70.6%), requiring respiratory support. At the end of the 37 months [IQR 13-77] follow-up, 19/106 (17.9%) patients were dead. The main causes of death were active disease and infections. By stepwise regression analysis, age >65 years (HR 3.66 [95% CI 1.4-9.51], p = 0.008) and the need for respiratory support (HR 4.58 [95% CI 1.51-13.87], p = 0.007) at AH onset were confirmed to be predictive of mortality.

Conclusions: Predictors of outcome in AAV-AH were determined. Factors related to the patient's performance status and the severity of the lung involvement strongly influenced the outcome. Balancing harms and benefits for the individual patient in induction and maintenance treatment strategies is crucial.
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http://dx.doi.org/10.1016/j.jaut.2019.102397DOI Listing
March 2020

Systemic Sclerosis Serum Significantly Impairs the Multi-Step Lymphangiogenic Process: in Vitro Evidence.

Int J Mol Sci 2019 Dec 7;20(24). Epub 2019 Dec 7.

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

In systemic sclerosis (SSc), the possible involvement of lymphatic microcirculation and lymphangiogenesis has traditionally been overshadowed by the greater emphasis placed on dysfunctional blood vascular system and angiogenesis. In the present in vitro study, we explore for the first time whether the SSc microenvironment may interfere with lymphangiogenesis, a complex, multi-step process in which lymphatic microvascular endothelial cells (LMVECs) sprout, migrate, and proliferate to generate new lymphatic capillaries. Normal human adult dermal LMVECs from three donors were treated with serum from SSc patients ( = 8), serum from healthy individuals ( = 8), or recombinant human vascular endothelial growth factor (VEGF)-C as a positive control for lymphangiogenesis. Cell proliferation, Boyden chamber Matrigel chemoinvasion, wound healing capacity, and lymphatic capillary morphogenesis on Geltrex were assayed. VEGF-C serum levels were measured by enzyme-linked immunosorbent assay. Gene and protein expression levels of the lymphangiogenic orchestrators VEGF receptor-3 (VEGFR-3)/Flt-4 and neuropilin-2 (NRP-2) were determined by real-time PCR and Western blotting, respectively. Conditioning with SSc serum significantly inhibited LMVEC proliferation, Matrigel invasion, and wound healing capacity with respect to healthy serum. The ability of LMVECs to form lymphatic tubes on Geltrex was also severely compromised in the presence of SSc serum. VEGF-C levels were comparable in SSc and healthy sera. Treatment with SSc serum resulted in a significant downregulation of both VEGFR-3/Flt-4 and NRP-2 mRNA and protein levels. In SSc, the pathologic environment severely hampers every lymphangiogenesis step, likely through the reduction of pro-lymphangiogenic VEGFR-3/NRP-2 co-receptor signaling. The impairment of the lymphangiogenic process opens a new scenario underlying SSc vascular pathophysiology, which is worth investigating further.
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http://dx.doi.org/10.3390/ijms20246189DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6940874PMC
December 2019

Very early systemic sclerosis.

Best Pract Res Clin Rheumatol 2019 08 3;33(4):101428. Epub 2019 Sep 3.

Dept. of Experimental and Clinical Medicine, University of Florence, Italy. Electronic address:

The early diagnosis of systemic sclerosis (SSc) can be very difficult, when most of the typical signs and symptoms are absent. For this reason, the approach to SSc has changed during the last decades because the importance of an early diagnosis and treatment has been widely understood. "Very early SSc" is identified as a condition characterized by Raynaud's phenomenon, puffy fingers, disease-specific autoantibodies, and microvascular alterations at capillaroscopy. However, reliable biomarkers able to predict the disease evolution are missing, and decision whether to treat or not to treat in the earliest phase of the disease remains a dilemma. Presently, the only feasible clinical strategy in very early SSc remains a tight follow-up program to detect in "real time" the onset of internal organ involvement, which may thus allow an aggressive therapeutic agenda.
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http://dx.doi.org/10.1016/j.berh.2019.101428DOI Listing
August 2019

Cardiac magnetic resonance predicts ventricular arrhythmias in scleroderma: the Scleroderma Arrhythmia Clinical Utility Study (SAnCtUS).

Rheumatology (Oxford) 2020 08;59(8):1938-1948

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

Objectives: Cardiac rhythm disturbances constitute the most frequent cardiovascular cause of death in SSc. However, electrocardiographic findings are not a part of risk stratification in SSc. We aimed to translate 24 h Holter findings into a tangible risk prediction score using cardiovascular magnetic resonance.

Methods: The Scleroderma Arrhythmia Clinical Utility Study (SAnCtUS) was a prospective multicentre study including 150 consecutive SSc patients from eight European centres, assessed with 24 h Holter and cardiovascular magnetic resonance, including ventricular function, oedema (T2 ratio) and late gadolinium enhancement (%LGE). Laboratory/clinical parameters were included in multivariable corrections. A combined endpoint of sustained ventricular tachycardia requiring hospitalization and sudden cardiac death at a median (interquartile range) follow-up of 1 (1.0-1.4) year was generated.

Results: Only T2 ratio and %LGE were significant predictors of ventricular rhythm disturbances, but not of supraventricular rhythm disturbances, after multivariable correction and adjustment for multiple comparisons. Using decision-tree analysis, we created the SAnCtUS score, a four-category scoring system based on T2 ratio and %LGE, for identifying SSc patients at high risk of experiencing ventricular rhythm disturbance at baseline. Increasing SAnCtUS scores were associated with a greater disease and arrhythmic burden. All cases of non-sustained ventricular tachycardia (n = 7) occurred in patients with the highest SAnCtUS score (=4). Having a score of 4 conveyed a higher risk of reaching the combined endpoint in multivariable Cox regression compared with scores 1/2/3 [hazard ratio (95% CI): 3.86 (1.14, 13.04), P = 0.029] independently of left ventricular ejection fraction and baseline ventricular tachycardia occurrence.

Conclusion: T2 ratio and %LGE had the greatest utility as independent predictors of rhythm disturbances in SSc patients.
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http://dx.doi.org/10.1093/rheumatology/kez494DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7382593PMC
August 2020

Recent advances steer the future of systemic sclerosis toward precision medicine.

Clin Rheumatol 2020 Jan 23;39(1):1-4. Epub 2019 Nov 23.

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

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http://dx.doi.org/10.1007/s10067-019-04834-5DOI Listing
January 2020

Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course.

J Clin Med 2019 Nov 18;8(11). Epub 2019 Nov 18.

Department of Rheumatology, S. Maria Hospital-IRCCS, 42123 Reggio Emilia, Italy.

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.
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http://dx.doi.org/10.3390/jcm8112013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6912490PMC
November 2019

The Renal Resistive Index in systemic sclerosis: Determinants, prognostic implication and proposal for specific age-adjusted cut-offs.

Eur J Intern Med 2019 Dec 17;70:43-49. Epub 2019 Sep 17.

Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Via Delle Oblate 4, 50134 Florence, Italy; Department of Geriatric Medicine, Division of Rheumatology, Azienda Ospedaliera Universitaria Careggi, Florence, Italy.

Background: Renal Resistive Index (RRI), reflects changes in both renal vascular and tubular-interstitial compartments and in systemic vascular compliance related to age and comorbidities.

Objectives: a) To investigate determinants of RRI in SSc population, b) its association with SSc-related features and c) to test its prognostic impact on organ specific worsening or death.

Methods: 380 SSc patients ≥18 years were enrolled after giving informed consent. Baseline data on RRI, laboratory, instrumental and therapeutic features were retrospectively collected. Age-SSc adjusted cut-offs were created by dividing the population in age quartiles and considering RRI values >75th percentile as pathologic. Clinical follow-up was performed until last available visit or the development/worsening of specific internal organ involvement or death.

Results: RRI was independently predicted by age and systolic pulmonary arterial pressure on Echo. Therefore, we created Age-SSc adjusted pathologic RRI cut-offs, which were significantly associated with various disease related skin and lung fibrotic manifestations, as well as vasculopathic complications. After a mean follow-up of 3.6 ± 2.6 years, RRI was one of the independent predictors (together with modified Rodnan skin score, interstitial lung disease, presence of dyspnoea and late nailfold-videocapillaroscopy pattern) for mortality, with 0.68 as best cut-off (sensitivity 88.5%, specificity 50.9%).

Conclusion: If corroborated, Renal Resistive Index cut-offs might be used to evaluate renal and extrarenal involvement in SSc and could serve as predictors of mortality.
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http://dx.doi.org/10.1016/j.ejim.2019.09.001DOI Listing
December 2019

Pregnancy in Systemic Sclerosis: Results of a Systematic Review and Metaanalysis.

J Rheumatol 2020 06 1;47(6):881-887. Epub 2019 Sep 1.

From the Department of Experimental and Clinical Medicine, University of Florence, and the Department of Geriatric Medicine, Division of Rheumatology and Scleroderma Unit, Azienda Ospedaliero Universitaria Careggi (AOUC), Florence, Italy; Department of Family Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia; Faculty of Medicine, Alexandria University, Alexandria, Egypt; Department of Emergency Medicine, Division of Medicine IV AOUC; Department of Maternal-Neonatal Caref, Careggi University Hospital, Florence, Italy; University of California at Los Angeles, Los Angeles, California; University of Washington, Seattle, Washington, USA; University of Florence, Florence, Italy.

Objective: Through a systematic literature search (SLR) and metaanalysis, to determine maternal and fetal outcomes in pregnancies involving systemic sclerosis (SSc), to analyze the effect of pregnancy on disease activity, and to examine predictors of fetal and maternal outcomes.

Methods: An SLR was performed for articles on SSc and pregnancy published between 1950 and February 1, 2018. Reviewers double-extracted articles to obtain agreement on > 95% of predefined critical outcomes.

Results: Out of 461 publications identified, 16 were included in the metaanalysis. The metaanalysis showed that pregnancies involving SSc were at higher risk of miscarriage (OR 1.6, 95% CI 1.22-2.22), fetuses with intrauterine growth retardation (IUGR; OR 3.2, 95% CI 2.21-4.53), preterm births (OR 2.4, 95% CI 1.14-4.86), and newborns with low birth weight (OR 3.8, 95% CI 2.16-6.56). Patients with SSc had a 2.8 times higher chance of developing gestational hypertension (HTN; OR 2.8, 95% CI 2.28-3.39) and a 2.3 times higher chance of cesarean delivery compared to controls (OR 2.3, 95% CI 1.37-3.8). The definitions of disease worsening/new visceral organ involvement were too inexact to have any confidence in the results, although worsening or new disease manifestations during pregnancy in 44/307 cases (14.3%) and 6 months postpartum in 32/306 cases (10.5%) were reported. The data did not permit definition of predictors of disease progression and of maternal and fetal outcomes.

Conclusion: Pregnancies involving SSc have increased frequency of miscarriages, IUGR, preterm deliveries, and newborns with low birth weight compared to healthy controls. Women with SSc were more prone to develop gestational HTN and to undergo cesarean delivery. Disease manifestations seem to remain stable or improve in most patients.
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http://dx.doi.org/10.3899/jrheum.181460DOI Listing
June 2020

Enthesopathy and involvement of synovio-entheseal complex in systemic sclerosis: an ultrasound pilot study.

Rheumatology (Oxford) 2020 03;59(3):580-585

Department of Experimental and Clinical Medicine, Section of Internal Medicine, University of Florence, and Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), Florence, Italy.

Objectives: SSc is a chronic autoimmune disease characterized by inflammation of the skin and multiple internal organs. Articular involvement is one of the main features of SSc, and typical hallmarks of SpA have been found in SSc patients. The aim of the present study was to estimate the prevalence of entheseal and synovio-entheseal complex (SEC) alterations in a cohort of SSc patients.

Methods: One hundred SSc patients and 25 healthy subjects were included in this cross-sectional study. The enthesis sites of lateral epicondylar common extensor tendons (CET) and the enthesis of the Glasgow Ultrasound Enthesis Scoring System were evaluated. SEC involvement was evaluated only at CET enthesis.

Results: In SSc, the Glasgow Ultrasound Enthesis Scoring System score was significantly higher (median 4.0, interquartile range 2.0-7.0) than in controls (median 1.0, interquartile range 0.0-3.0) (P < 0.0001). CET enthesis of SSc patients showed more frequent US B-mode alterations than that of controls (χ2 = 11.47, P = 0.0007 for size; χ2 = 13.79, P = 0.0002 for cortical irregularity, χ2 = 5.24, P = 0.022 for calcification/enthesophytes). Power Doppler US signal at CET enthesis was significantly more frequent in SSc patients than in healthy controls (χ2 = 9.11, P = 0.0025), as was the concomitant SEC involvement (χ2 = 8.52, P = 0.0035).

Conclusion: These data show that SSc patients frequently present US features of enthesopathy. Moreover, CET enthesopathy was correlated with SEC inflammation, suggesting that entheseal inflammation in SSc may share the same micro-anatomical targets as found in SpA.
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http://dx.doi.org/10.1093/rheumatology/kez322DOI Listing
March 2020

Pleuroparenchymal fibroelastosis in patients affected by systemic sclerosis: What should the rheumatologist do?

Medicine (Baltimore) 2019 Jun;98(26):e16086

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

Pleuroparenchymal fibroelastosis (PPFE) is a rare new interstitial lung disease (ILD) characterized by the fibrotic thickening of the visceral pleura and subadjacent parenchymal areas of the upper lobes This study reveals that patients with ILD-SSc associated with chest HRCT evidence of PPFE require close and recurrent follow-up with periodic evaluation of lung function parameters, DLCO and chest HRCT. Rheumatologists should be aware of this new radiological finding which is accompanied by a negative prognosis, especially when associated with a progressive course. Patients with this radiological pattern need to be monitored with particular attention.
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http://dx.doi.org/10.1097/MD.0000000000016086DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6617070PMC
June 2019
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