roberta priori
@policlinicoumberto1.it
UOC Reumatologia
Azienda Ospedaliera Universitaria Policlinico Umberto I
RESEARCH INTERESTS
rheumatology, Autoimmunity, autoinflammation, sjogren's syndrome
Scopus Publications
Scopus Publications
Emanuel Della-Torre, Rosaria Talarico, Jose Ballarin, Emanuele Bozzalla-Cassione, Chiara Cardamone, Cosimo Cigolini, Francesco Ferro, Tomas Fonseca, George E Fragoulis, Ilaria Galetti,et al.
Elsevier BV
Serena Colafrancesco and Roberta Priori
Elsevier BV
Chiara Mandosi, Cecilia Galli, Viviana Matys, Camilla Di Dio, Martina Briante, Valeria Riccieri, Roberta Priori, and Maria Grazia Piccioni
Clinical and Experimental Rheumatology
OBJECTIVES
The aim of this work is to review the existing literature regarding sexual and reproductive function of women affected by systemic sclerosis and to establish the impact of the disease on the gynaecological-obstetrical field.
METHODS
A systematic search has been conducted by means of PubMed, Cochrane, Google Scholar, until January 2024 by the keywords ''systemic sclerosis'', ''fertility'', "sexual dysfunction" and "pregnancy".
RESULTS
Sexual dysfunction has been described in most of the studies. This could be related to dryness and dyspareunia, but also to the psychosocial impact of SSc on body and facial appearance, which impacts on social and sexual relationships. There is conflicting evidence regarding the influence of SSc and fertility. Before the 1980s pregnancies in these patients were rare. This could be linked to the satisfied reproductive desire before the onset of SSc, or to the fact that pregnancy was labelled as high-risk, leading to counsel against it in most patients. Recently, the evidence supporting infertility is conflicting. There is no certain theory on how the disease may interfere with reproductive function, but a possible linkage can be detected in a pro-inflammatory milieu which can impair the ovarian reserve.
CONCLUSIONS
Women affected by SSc should be followed-up by a multidisciplinary team to prevent sexual dysfunction. Although there is no consensus on the impact of SSc on fertility, these patients should be provided with adequate pre-conceptional counselling and a strict follow-up in high-risk pregnancy units.
Piero Ruscitti, Yannick Allanore, Chiara Baldini, Giuseppe Barilaro, Elena Bartoloni Bocci, Pietro Bearzi, Elisa Bellis, Onorina Berardicurti, Alice Biaggi, Michele Bombardieri,et al.
Elsevier BV
E. Molteni, C. Pirone, F. Ceccarelli, C. Castellani, C. Alessandri, M. Di Franco, V. Riccieri, F.R. Spinelli, R. Priori, R. Scrivo,et al.
PAGEPress Publications
Objective. Data from trials demonstrated that abatacept (ABA) has a good safety and efficacy profile in treating rheumatoid arthritis. We have studied the retention rate of ABA in a real-life cohort of patients with rheumatoid arthritis. Methods. This is a monocentric, retrospective study including patients with rheumatoid arthritis classified by the American College of Rheumatology/European League Against Rheumatism 2010 criteria who started treatment with ABA. The Kaplan-Meier method was applied to evaluate the ABA retention rate. Results. This analysis was conducted on 161 patients [male/female 21/140, median age 65 years, interquartile range (IQR) 18.7, median disease duration 169 months, IQR 144.0]. 111 patients (68.9%) received ABA subcutaneously. ABA was associated with methotrexate in 61.9% of patients and was the first biological disease-modifying antirheumatic drug in 41%. We observed a median ABA survival of 66 months [95% confidence interval (CI) 57.3-74.7], with a retention rate of 88% at 6 months and 50.9% at 5 years. Drug survival was significantly higher in patients treated with ABA subcutaneously and in male patients (p=0.039 and p=0.018, respectively). Adjusted for main confounders, female gender was the main predictor of withdrawal (hazard ratio 5.1, 95% CI 1.2-21.3). Conclusions. Our study shows that better survival is associated with subcutaneous administration and male gender, confirming ABA effectiveness.
Chiara Gioia, Giulio Dolcini, Cristina Iannuccelli, Martina Favretti, Daniele Franculli, Piercarlo Sarzi-Puttini, Fabrizio Conti, Roberta Priori, and Manuela Di Franco
Clinical and Experimental Rheumatology
OBJECTIVES
Fibromyalgia (FM) may have consequences on sexual life. The objective was to validate the Qualisex questionnaire in the assessment of sexual dysfunction in women affected by FM.
METHODS
We consecutively enrolled FM women (American College of Rheumatology-ACR 2016) referring to our Fibromyalgia Clinic, from 2020 to 2022. Demographic, clinical data and evaluation of FM symptoms severity (Revised Fibromyalgia Impact Questionnaire (R-FIQ), Symptoms Severity Scale-SSS, Widespread Pain Index-WPI) were assessed. Hospital Anxiety and Depression Scale (HADS) and Qualisex questionnaire were anonymously administered. Qualisex includes 10 questions on different items of sexual life with higher scores suggestive of greater negative impact of the disease on sexuality.
RESULTS
The cohort was composed by 373 FM women. Cronbach's alpha test was used to validate Qualisex questionnaire (0.878). Moreover, we observed higher values of Qualisex in married women (p<0.001), in women with lower grade of education (p=0.002) and with lower sexual feeling with partner (p<0.001). Higher values of Qualisex Total score showed a positive correlation with HADS-A/D (p<0.001 r=0.312; p<0.001 r=0.542 respectively), VAS pain, VAS fatigue, VAS dryness (p<0.001 r=0,438; p<0.001 r=0.375; p<0.001 r=0.370 respectively) and relationship duration (p<0.001 r=0.202). Multivariate analysis revealed a significant influence of relationship duration, VAS pain, fatigue, dryness, HADS-A/D, R-FIQ and all Qualisex items, on Qualisex Total score corrected for patients' age (p<0.001).
CONCLUSIONS
This study validated Qualisex questionnaire as a good test for the sexual disorders' evaluation in FM women. Its use allows the assessment of different factors associated with sexual dysfunction, showing an impact of FM on sexuality. Moreover, due to demotivation feelings, sexual dysfunction contributes to worsen patients' quality of life.
Federica Maria Ucci, Rossana Scrivo, Cristiano Alessandri, Fabrizio Conti, and Roberta Priori
Frontiers Media SA
Aseptic abscesses syndrome is a rare but increasingly recognized disease that falls within the spectrum of autoinflammatory disorders. Here, we describe the case of a patient who presented with abdominal pain and fever, along with multiple abdominal and extra-abdominal abscesses, in the absence of underlying hematologic, autoimmune, infectious, or neoplastic conditions. Initially, the patient responded to glucocorticoids, but experienced several flares upon discontinuation, leading to the initiation of treatment with a TNFα inhibitor. After 5 years, an attempt to discontinue treatment resulted in a new flare of the disease. Remission was eventually achieved with a biosimilar TNFα inhibitor, albeit requiring shortened infusion intervals.
Chiara Mandosi, Viviana Matys, Marianna Deroma, Valentina Del Negro, Lucia Merlino, Marianna Mariani, Roberta Priori, Enrico Ciminello, Emanuela Anastasi, Maria Grazia Porpora,et al.
Springer Science and Business Media LLC
Abstract Objective This study aimed to assess the potential impact of primary Sjögren’s syndrome (pSS) on fertility and ovarian reserve by evaluating the number of antral ovarian follicles (AFC) through ultrasound and analysing serum levels of anti-müllerian hormone (AMH) and follicle-stimulating hormone (FSH), which are currently the most reliable indicators of fertility potential. Method A total of 52 premenopausal women were recruited from the Maternal, Infantile, and Urological Sciences Department at Umberto I Hospital, Sapienza University of Rome. Among them, 26 had pSS, and 26 served as healthy controls. All participants underwent a gynaecological examination, a transvaginal ultrasound, and serum testing for AMH and FSH levels. Results The study found that serum AMH levels were significantly lower (p = 0.002) in pSS patients compared to the controls, indicating a potential reduction in ovarian reserve in these patients. However, no statistically significant differences were observed in FSH levels between the two groups. Conclusions The findings suggest that pSS may have a negative impact on ovarian reserve, as evidenced by lower AMH levels in comparison to age-matched controls. AFC and FSH levels, however, were similar to those of healthy women. These results provide new insights that could be beneficial for this patient population, though further, larger-scale studies are necessary to more comprehensively understand the relationship between pSS and female fertility. Key Points• The study assesses the possible impact of pSS on fertility and ovarian reserve by evaluation of AMH, FSH, and AFC.• Family planning and fertility are important issues for patients with rheumatic disorders and must be considered and discussed with the patient already at the time of diagnosis, and appropriate counselling must be performed.
Alejandra Flores-Chávez, Pilar Brito-Zerón, Wan-Fai Ng, Antónia Szántó, Astrid Rasmussen, Roberta Priori, Chiara Baldini, Berkan Armagan, Burcugül Özkiziltaş, Sonja Praprotnik,et al.
Clinical and Experimental Rheumatology
OBJECTIVES
To analyse how the key components at the time of diagnosis of the Sjögren's phenotype (epidemiological profile, sicca symptoms, and systemic disease) can be influenced by the potential exposure to climate-related natural hazards.
METHODS
For the present study, the following variables were selected for harmonisation and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Climate-related hazards per country were defined according to the OECD and included seven climate-related hazard types: extreme temperature, extreme precipitation, drought, wildfire, wind threats, river flooding, and coastal flooding. Climatic variables were defined as dichotomous variables according to whether each country is ranked among the ten countries with the most significant exposure.
RESULTS
After applying data-cleaning techniques and excluding people from countries not included in the OECD climate rankings, the database study analysed 16,042 patients from 23 countries. The disease was diagnosed between 1 and 3 years earlier in people living in countries included among the top 10 worst exposed to extreme precipitation, wildfire, wind threats, river flooding, and coastal flooding. A lower frequency of dry eyes was observed in people living in countries exposed to wind threats, river flooding, and coastal flooding, with a level of statistical association being classified as strong (p<0.0001 for the three variables). The frequency of dry mouth was significantly lower in people living in countries exposed to river flooding (p<0.0001) and coastal flooding (p<0.0001). People living in countries included in the worse climate scenarios for extreme temperature (p<0.0001) and river flooding (p<0.0001) showed a higher mean ESSDAI score in comparison with people living in no-risk countries. In contrast, those living in countries exposed to worse climate scenarios for wind threats (p<0.0001) and coastal flooding (p<0.0001) showed a lower mean ESSDAI score in comparison with people living in no-risk countries.
CONCLUSIONS
Local exposure to extreme climate-related hazards plays a role in modulating the presentation of Sjögren across countries concerning the age at which the disease is diagnosed, the frequency of dryness, and the degree of systemic activity.
Serena Colafrancesco, Edoardo Simoncelli, Roberta Priori, and Michele Bombardieri
Clinical and Experimental Rheumatology
The link between immune cell function and cell metabolic reprogramming is currently known under the term "immunometabolism". Similarly to the Warburg's effect described in cancer cells, in activated immune cells an up-regulation of specific metabolic pathways has been described and seems to be pathogenic in different inflammatory conditions.Sjӧgren's syndrome (SS) is a systemic autoimmune disease that affects the exocrine glands and is characterised by a progressive loss of secretory function. Despite the increasing amount of evidence on the ability of metabolism in regulating cell behaviour in inflammatory or tumoral conditions, the field of metabolism in SS is still for the most part unexplored.The aim of this review is to summarise currently available studies evaluating cell metabolism in SS with a particular focus on the possible pathogenic role of metabolic changes in immune and non-immune cells in this condition.
Roberto Giacomelli, Roberto Caporali, Francesco Ciccia, Serena Colafrancesco, Lorenzo Dagna, Marcello Govoni, Florenzo Iannone, Pietro Leccese, Carlomaurizio Montecucco, Giovanni Pappagallo,et al.
Elsevier BV
Sara Monti, Alessandra Milanesi, Catherine Klersy, Alessandro Tomelleri, Lorenzo Dagna, Corrado Campochiaro, Nicola Farina, Francesco Muratore, Elena Galli, Chiara Marvisi,et al.
BMJ
BackgroundImmune and vascular ageing are proposed risk factors for giant cell arteritis (GCA). Data on the impact of age at diagnosis of GCA on the clinical presentation and course of the disease are scarce.MethodsPatients with GCA followed at referral centres within the Italian Society of Rheumatology Vasculitis Study Group were enrolled up to November 2021. Patients were grouped according to age at diagnosis: ≤64, 65–79 and ≥80 years old.ResultsThe study included 1004 patients, mean age 72.1±8.4, female 70.82%. Median follow-up duration was 49 (IQR 23–91) months. Patients in the oldest group (≥80 years) had significantly more cranial symptoms, ischaemic complications and risk for blindness compared with the groups 65–79 and ≤64 years (blindness: 36.98% vs 18.21% vs 6.19%; p<0.0001). Large-vessel-GCA was more frequent in the youngest group (65% of patients). Relapses occurred in 47% of patients. Age did not influence the time to first relapse, nor the number of relapses. Older age was negatively associated with the number of adjunctive immunosuppressants. Patients >65 years old had 2–3 fold increased risk for aortic aneurysm/dissection up to 60 months follow-up. Serious infections, but not other treatment-related complications (hypertension, diabetes, osteoporotic fractures), were significantly associated with older age. Mortality occurred in 5.8% of the population with age >65, cranial and systemic symptoms as independent risk factors.ConclusionsThe highest risk of ischaemic complications, aneurysm development, serious infections and the possible undertreatment make of GCA a very challenging disease in the oldest patients.
Pilar Brito-Zerón, Alejandra Flores-Chávez, Ildiko Fanny Horváth, Astrid Rasmussen, Xiaomei Li, Peter Olsson, Arjan Vissink, Roberta Priori, Berkan Armagan, Gabriela Hernandez-Molina,et al.
Elsevier BV
Pamela Rosso, Elena Fico, Serena Colafrancesco, Mario Giuseppe Bellizzi, Roberta Priori, Bruna Cerbelli, Martina Leopizzi, Carla Giordano, Antonio Greco, Paola Tirassa,et al.
MDPI AG
Primary Sjögren’s Syndrome (pSS) is a systemic autoimmune disease that primarily attacks the lacrimal and salivary glands, resulting in impaired secretory function characterized by xerostomia and xerophthalmia. Patients with pSS have been shown to have impaired salivary gland innervation and altered circulating levels of neuropeptides thought to be a cause of decreased salivation, including substance P (SP). Using Western blot analysis and immunofluorescence studies, we examined the expression levels of SP and its preferred G protein-coupled TK Receptor 1 (NK1R) and apoptosis markers in biopsies of the minor salivary gland (MSG) from pSS patients compared with patients with idiopathic sicca syndrome. We confirmed a quantitative decrease in the amount of SP in the MSG of pSS patients and demonstrated a significant increase in NK1R levels compared with sicca subjects, indicating the involvement of SP fibers and NK1R in the impaired salivary secretion observed in pSS patients. Moreover, the increase in apoptosis (PARP-1 cleavage) in pSS patients was shown to be related to JNK phosphorylation. Since there is no satisfactory therapy for the treatment of secretory hypofunction in pSS patients, the SP pathway may be a new potential diagnostic tool or therapeutic target.
Andrea Latini, Giada De Benedittis, Serena Colafrancesco, Carlo Perricone, Giuseppe Novelli, Lucia Novelli, Roberta Priori, Cinzia Ciccacci, and Paola Borgiani
MDPI AG
Background: The PCSK3 gene encodes for the protease enzyme Furin, which promotes proteolytic maturation of important regulators of the immune response, and also enhances the secretion of interferon-γ (IFN). Several studies have suggested its possible involvement in the pathogenesis of chronic inflammatory diseases. Methods: We investigated the PCSK3 gene expression level in peripheral blood mononuclear cells isolated from Sjögren’s Syndrome (SS) patients and healthy controls and we evaluated a possible correlation with IFN-γ gene expression. Moreover, we also explored the variability of two PCSK3 genetic polymorphisms (rs4932178 and rs4702) to evaluate a possible association between these polymorphisms and the expression levels of this gene. Results: We observed, by RT-qPCR, that the PCSK3 expression level was significantly higher in SS patients compared to the controls (p = 0.028), and we confirmed a positive correlation between PCSK3 and IFN-γ expression levels (p < 0.001). Moreover, we reported that the variant homozygous genotype of rs4932178 SNP is associated with a higher expression of the PCSK3 gene (p = 0.038) and with the SS susceptibility (p = 0.016). Conclusions: Our data suggest that Furin could play a role in SS development, also promoting IFN-γ secretion.
Edoardo Simoncelli, Edoardo Conticini, Serena Colafrancesco, Angelica Gattamelata, Francesca Romana Spinelli, Cristina Garufi, Simona Truglia, Silvia Grazzini, Federico Giardina, Raffaella Izzo,et al.
Clinical and Experimental Rheumatology
OBJECTIVES
Data on the safety of anti-SARS-CoV-2 vaccines in patients with rare rheumatic diseases, such as systemic vasculitis (SV), are limited. The aim of this study was to evaluate the occurrence of a disease flare and the appearance of adverse events (AEs) following administration of anti-SARS-CoV-2 vaccine in a multicentre cohort of patients with SV.
METHODS
Patients with SV and healthy controls (HC) from two different Italian rheumatology centres were asked to complete a questionnaire assessing disease flares occurrence, defined as new onset of clinical manifestations related to vasculitis needing an implementation of therapy, and local/systemic AEs appearance following anti SARS-CoV-2 vaccination.
RESULTS
107 patients with SV (57 ANCA-associated) and 107 HC were enrolled. A disease flare occurred in only one patient (0.93%) with microscopic polyangiitis after the first dose of an mRNA vaccine. After both the first and the second vaccine dose administration, no significant differences in AEs between patients with SV and HC were observed; no serious AEs were reported as well.
CONCLUSIONS
These data suggest a good risk profile for anti-SARS-CoV-2 vaccine in patients with systemic vasculitis.
Rossana Scrivo, Chiara Castellani, Silvia Mancuso, Giorgio Sciarra, Federico Giardina, Giulia Bevignani, Fulvia Ceccarelli, Francesca Romana Spinelli, Cristiano Alessandri, Manuela Di Franco,et al.
Clinical and Experimental Rheumatology
OBJECTIVES
The use of biosimilars is constantly growing, prompting healthcare payers to encourage the switch to these drugs which are less expensive than the reference bio-originator. While switching from a bio-originator to a biosimilar is supported by increasing evidence, data on the switch between different biosimilars of the same reference product are scant. Our study aimed to evaluate the effectiveness of the non-medical switch both between adalimumab (ADA) bio-originator and SB5 biosimilar and between two different ADA biosimilars in patients with inflammatory chronic arthritis.
METHODS
We observed adult patients with a diagnosis of rheumatoid arthritis (RA), psoriatic arthritis (PsA), and axial spondyloarthritis (axSpA) treated with ADA bio-originator or ABP501 ADA biosimilar (Amgevita) who switched to SB5 ADA biosimilar (Imraldi) for administrative/economic reasons. Patients were followed up for 4 months.
RESULTS
One hundred and ten patients [33 RA, 40 PsA, 37 axSpA; F:M= 49:61; median age 56 years (25th-75th percentile 48-66)] switched from ADA bio-originator to SB5. After 4 months (T4), we observed a significant reduction of patients in remission/low disease activity (baseline 92.7% vs. T4 80.9%; p=0.009), with a risk of moderate-high disease activity significantly higher after the switch [RR 2.6 (95% IC 1.2 to 5.7), p=0.01]. However, no differences were found in DAS28-CRP, DAPSA, ASDAS-CRP, and BASDAI, while patients with RA and PsA experienced a worsening in the patient global assessment-VAS (p=0.04 and p=0.02, respectively), and in patients with PsA a worsening in HAQ was also observed (p=0.03). Forty patients switched from ABP501 biosimilar to SB5 [12 with RA, 25 with PsA, and 3 with axSpA; F:M=24:16; median age 56 years (25th-75th percentile 44-66)]. After 4 months, no differences in DAS28-CRP and DAPSA nor in the percentage of patients in remission/low disease activity were found compared to baseline. Likewise, no differences were found in patient-reported outcomes (PROs).
CONCLUSIONS
Our results provide a reassuring profile of effectiveness when switching from ADA originator to one of its biosimilars and between two different biosimilars. However, the worse outcome in PROs in patients initially treated with the bio-originator addresses the attention to a possible nocebo response, which should encourage comprehensive communication with patients.
Gabriela Hernández-Molina, Belchin Kostov, Pilar Brito-Zerón, Arjan Vissink, Thomas Mandl, Anneline C Hinrichs, Luca Quartuccio, Chiara Baldini, Raphaele Seror, Antonia Szántó,et al.
Oxford University Press (OUP)
Abstract Objective To characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes. Methods By January 2021, the Big Data Sjögren Project Consortium database included 11 966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified. Results There were 414 patients (355 women, mean age 57 years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n = 197), followed by diffuse large B-cell lymphoma (DLBCL) (n = 67), nodal MZL lymphoma (n = 29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n = 19) and follicular lymphoma (FL) (n = 17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8 years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL). Conclusion In the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%.
Giorgia Carnicelli, Alvise Sernicola, Vito Gomes, Giulia Cundari, Stefania Trasarti, Roberta Priori, and Teresa Grieco
MDPI AG
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare immune-mediated vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Having systemic and possibly severe involvement, a prompt recognition of its clinical features is crucial to achieve favorable patient outcomes. Although cutaneous manifestations represent key elements, these still remain poorly characterized. We report a case of ANCA-positive EGPA presenting with palpable purpura, livedo reticularis, and pemphigoid-like lesions that was successfully treated with glucocorticoid therapy and rituximab. This report portrays the evolution of cutaneous lesions in ANCA-positive EGPA and demonstrates how dermatologic signs may represent indicators of active disease, allowing for timely diagnosis and for the monitoring of disease activity during treatment.
Nevsun Inanc, Belchin Kostov, Roberta Priori, Alejandra Flores-Chavez, Francesco Carubbi, Antónia Szántó, Valeria Valim, Hendrika Bootsma, Sonja Praprotnik, Virginia Fernandes Moça Trevisani,et al.
Clinical and Experimental Rheumatology
Roberta Priori, Federico Giardina, Chiara Gioia, Cristina Iannuccelli, Martina Villa, Angelica Gattamelata, Fabrizio Conti, Manuela Di Franco, and Giuseppe Curcio
Clinical and Experimental Rheumatology
Giada De Benedittis, Andrea Latini, Serena Colafrancesco, Roberta Priori, Carlo Perricone, Lucia Novelli, Paola Borgiani, and Cinzia Ciccacci
MDPI AG
Sjögren’s syndrome (SS) is a chronic autoimmune multifactorial disease characterized by inflammation and lymphocytic infiltration of the exocrine glands. Several studies have highlighted the involvement of oxidative stress in this pathology, suggesting that it could induce mitochondrial dysfunctions. Mitochondria could have a role in inflammatory and immune processes. Since the mitochondrial DNA (mtDNA) copy number could change in response to physiological or environmental stimuli, this study aimed to evaluate possible alterations in the mtDNA copy number in SS. We have analyzed the amount of mtDNA in the peripheral blood of 74 SS patients and 61 healthy controls by qPCR. Then, since mitochondrial fusion and fission play a crucial role in maintaining the number of mitochondria, we investigated the expression variability of the genes most commonly involved in mitochondrial dynamics in a subgroup of SS patients and healthy controls. Interestingly, we observed a highly significant decrease in mtDNA copies in the SS patients compared to healthy controls (p = 1.44 × 10−12). Expression levels of mitochondrial fission factor (MFF), mitofusin-1 (MFN1), and mitochondrial transcription factor A (TFAM) genes were analyzed, showing a statistically significant increase in the expression of MFF (p = 0.003) and TFAM (p = 0.022) in the SS patients compared to healthy controls. These results give further insight into the possible involvement of mitochondrial dysfunctions in SS disease.
Edoardo Conticini, Miriana d’Alessandro, Silvia Grazzini, Marco Fornaro, Daniele Sabella, Giuseppe Lopalco, Federico Giardina, Serena Colafrancesco, Chiara Rizzo, Giuliana Guggino,et al.
Springer Science and Business Media LLC
AbstractSevere acute respiratory syndrome coronavirus-2 (SARS-CoV-2) vaccination plays a crucial role as pivotal strategy to curb the coronavirus disease-19 (COVID-19) pandemic. The present study described the clinical status of patients affected by idiopathic inflammatory myopathies (IIM) after COVID-19 vaccination to assess the number of relapses. We included all patients affected by IIM and followed by Myositis Clinic, Rheumatology and Respiratory Diseases Units, Siena University Hospital, Bari University Hospital, Policlinico Umberto I, Sapienza University, Rome, and Policlinico Paolo Giaccone, Palermo. They underwent a telephone survey. A total of 119 IIM patients (median, IQR 58 (47–66) years; 32males; 50 dermatomyositis, 39 polymyositis and 30 anti-synthetase syndrome) were consecutively enrolled. Except four patients who refused the vaccination, 94 (81.7%) received Comirnaty, 16 (13.9%) Spikevax, 5 (4.4%) Vaxzevria. Seven (6.1%) patients had flare after vaccination. One of them had life-threatening systemic involvement and died two months after second dose of COVID-19 vaccination. From logistic regression analysis, Chi2-log ratio = 0.045,the variable that most influences the development of flare was the number of organs involved (p = 0.047). Sixty-eight patients received the third dose of COVID-19 vaccination: 51(75%) Comirnaty and 17 (25%) Moderna. No patients had flares after third dose. Our study represents the largest cohort of IIM patients in which the incidence of recurrence after anti-SARS-CoV-2 vaccine was assessed. In line with real-life data from other diseases, we found a clinical non-statistically significant risk of relapse in our patients, which occurred seldom, usually mild and in patients with a more severe and aggressive course of disease.