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Background. Hearing dysfunction, caused by the involvement of the vestibulocochlear nerve or by direct damage on inner ear structures has been described in patients with Sjogren's Syndrome (SS). Previous studies evaluating the prevalence and incidence of hearing dysfunction in SS showed conflicting results, therefore, to date, the exact prevalence has not been extensively evaluated. Objectives. The aim of this study is to evaluate the prevalence of hearing involvement in patients with primary SS (pSS). Materials and methods. Patients with pSS (AECC criteria) with >=18 years of age attending a dedicated Sjogren's syndrome clinic were consecutively enrolled Auditory function was investigated by pure tone audiometry (PTA), It-Matrix test (Speech Reception Threshold in noise leading to 50% correct sentences-SRT) and the Hearing Handicap Inventory (HHI) during a baseline visit and at a follow up visit. A questionnaire of auto-evaluation of hearing loss impact on life was also administered to the patients. Results. Twenty-five patients with pSS (24 females) were enrolled in the study. The median age was 56.2 years (IQR 49-64) The mean disease duration was 3.7 years, 8 were treated with hydroxychloroquine (HCQ) and 1 with methotrexate. At baseline evaluation PTA revealed hearing loss in 17 patients (68%) with severity ranging from mild to severe. Fifteen patients (60%) presented mild hearing loss, 1(4%) moderate e 1 (4%) severe. The It-Matrix score ranged from -9.9 to 0.9 (median - 3.50). Median HHI score was 12.17 (min 0, max 68, SD 177.9). For Covid restrictions, a follow-up evaluation was available for 10 patients only. In these patients, a worsening of PTA and HHI was observed. Interestingly, the it-Matrix scores of patients with a stable disease showed an improvement. Conclusions. These preliminary findings suggest that hearing involvement is common in patients with SS and that it progresses over time. If confirmed on larger cohorts, these data will be useful for physicians in counseling patients about their disease and, in case of suspicious symptoms, an early evaluation by an otolaryngologist may prevent delay in diagnosis and allow an appropriate diagnostic evaluation and therapeutic intervention.
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Objectives. To investigate the safety and efficacy of SARS-Cov-2 vaccination in a large international cohort of patients with primary Sjogren syndrome due to scarcity of data in this population. Methods. By the first week of May 2021, all Big Data Sjogren Consortium centers had been contacted and asked for Registry patients to be included in the study if they had received at least one dose of any SARS-CoV-2 vaccine. The in-charge physician asked patients about local and systemic reactogenicity, using a pre-defined electronic questionnaire to collect epidemiologic data, COVID 19 vaccination data, and COVID 19 vaccination side effects. Adverse events were defined as those reported by the patient at the site of injection within 7 days from vaccination (reactogenicity) as local adverse events, systemic symptoms as systemic side effects, and postvaccination AEs of special interest related to SS as SS flares. Results. The vaccination data of 1237 patients (1170 women, with a mean age at diagnosis of primary SjS of 50.5 13.2) were received. A total of 835 patients (67 percent) reported any adverse event, including local (53 percent) and systemic (50 percent) AEs. Subjective symptoms (63%) were the most common local AEs, followed by objective signs at the injection site (16%) and general symptoms were the most commonly reported systemic AEs (46 percent), followed by musculoskeletal (25 percent), gastrointestinal (9 percent), cardiopulmonary (3 percent), and neurological (2 percent). People under 60 years old had a higher risk of developing AE after vaccination (OR 2.48, CI 95 1.89-3.27 percent), as did those with low systemic SS activity (OR 1.62, CI 95 1.22-2.15) and those who received mRNA vaccines, according to a multivariate analysis (OR 1.57, CI 95 percent 1.12- 2.18). The risk of developing systemic AEs was also higher in women (OR 2.85, CI 95 percent 1.60-5.2346), White people (OR 1.73, CI 95 1.14-2.65), and those who received a deficient vaccination regimen (OR 1.78, CI 95 1.12-2.88 percent). In addition to 141 (11%) patients who reported a significant worsening/exacerbation of their pre-vaccination sicca symptoms as a result of post-vaccination SS flares, 15 (1.2%) patients (13 women, mean age at vaccination 41.9 years) reported active involvement in the glandular (n=8), articular (n=7), cutaneous (n=6), pulmonary (n=2), and peripheral nervous system (n=1) domains as post-vaccination systemic flare. All side effects and flares subsided within 1-3 weeks, with no lasting effects or deaths. In terms of vaccination efficacy, breakthrough SARS-CoV-2 infection was confirmed after vaccination in three (0.24 percent) patients, all of whom recovered completely, and positive anti-SARS-Cov-2 antibodies were detected in approximately 95 percent of vaccinated SjS patients, according to data available. Conclusions. SARS-CoV-2 vaccination in patients with primary SjS, like other vaccines with adequate response and no safety signals, raised no concerns about the vaccine's efficacy or safety.
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Background: Vaccinations against SARS-CoV-2 represent a fundamental tool in controlling the pandemic. To date, data on the safety of anti-SARS-CoV-2 vaccines in patients with rare rheumatic diseases, such as systemic vasculitis, are limited. Objectives: In this study we aimed at evaluating the safety of anti-SARS-CoV-2 vaccines in a multicentric cohort of patients with systemic vasculitis. Methods: Patients with systemic vasculitis from two Rheumatology centres who had received anti-SARS-CoV-2 vaccine were retrospectively examined. The primary outcome was to evaluate, in this multi-centric cohort, the occurence of a disease fare after the administration of the vaccine, defned as development of clinical manifestations related to vasculitis with a concomitant increase in serum infammatory markers. As a secondary outcome we aimed at evaluating, in a monocentric cohort of patients with vasculitis, the occurrence of adverse events (AEs) following vaccine administration compared to healthy controls (HC). Results: We examined 111 patients with systemic vasculitis (n=69 female, n=42 male), with a mean age of 64.3 (± 13) years. Sixty had ANCA-associated vas-culitis (AAV), fourty-two had Giant-Cell Arterities (GCA), five had Periarteritis Nodosa, four had Takayasu's arteritis. One-hundred and five patients received a mRNA vaccine and six a viral vector one. A disease fare occurred in only 2 patients (1.8%) after the frst dose of a mRNA vaccine: both had AAV (microscopic poliangioitis) and developed a pulmunary disease fare (respiratory failure requiring hospitalization and treatment with high-dose glucocorticoids). Of note, one of these patients had multiple previous comorbidities, including a severe COPD. Multivaried analysis, adjusted for age and sex, performed in a single monocentric cohort of patients with systemic vasculitis [n=60 (39 AAV, 21 GCA), 37 female, 23 male, mean age 71 (± 12.5) years] demonstrated a statistically sig-nifcant higher frequency of AEs in vasculitis patients compared to HC (p=0.015) after the frst dose of vaccination. No signifcant differences in the frequency of AEs in vasculitis patients compared to HC after the second dose were detected. All the AEs were mild in both groups (malaise was the most frequently reported);no serious AEs were reported. Conclusion: Our data show a very low incidence of disease fares after the administration of anti-SARS-CoV-2 vaccines in patients with systemic vasculitis. Patients with systemic vasculitis seem more prone to develop mild AEs after the frst dose of the vaccine. Taken together, this data suggest a good risk profile for anti-SARS-CoV-2 vaccine in patients with systemic vasculitis.
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Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination plays a crucial role as pivotal strategies to curb the coronavirus dis-ease-19 (COVID-19) pandemic. Despite the mass-scale vaccination, literature data about the incidence of disease fares in IIM patients are still not reported as well as the immunological condition. Objectives: The present study aimed to describe the clinical status of patients affected by IIM after vaccination against COVID19 in order to assess the number of relapses or immune-mediated reactions in a cohort of Italian patients with such disease. Methods: 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. Inclusion criteria were a recent (<3 months) clinical and serological assessment before the survey and a defnite diagnosis of dermatomyosi-tis, polymyositis and anti-synthetase syndrome. All patients underwent a telephone survey in order to establish their clinical status and potential relapses after vaccination. Results: A total of 119 IIM patients (median, IQR 58 (47-66) years;32 males) were consecutively enrolled. Fifty had a diagnosis of DM, 39 had PM and 30 had ASS. The median months of disease duration was 79.62±83.98. According to number of organs involvement, forty-two had only one, 45 had two organs involvement, 20 had three, 11 had four and one had five. The majority of them received two doses of COVID-19 vaccine, except four patients who refused the vaccination: 94 (78.9%) Cominarty, 16 (13.4%) Moderna, 5 (0.04%) AZ. Seven (0.06%) patients had fare after vaccination, the majority of them were mild except one major with three organs involved and one life-threatening with systemic involvement. In order to understand or predict the effect of demographic and clinical features on the fare development after vaccination, a logistic regression analysis was performed. The goodness-of-ft statistics showed a Chi2 associated with the Log ratio (L.R.) of 0.045. From the probability associated with the Chi-square tests, the Type II analysis showed the variable that most influences the development of fare was the number of organs involved (p=0.047). Sixty-eight patients received the third dose of COVID-19 vaccination: 51 (75%) Cominarty and 17 (25%) Moderna. Only one (0.01%) patient (the same who had life-threatening fare with systemic involvement after two doses) had fare after third dose and eventually died. Conclusion: Vaccines against SARS-CoV2 have provided, both in registratory studies and in preliminary real-life evidence, an overall good efficacy and safety. Nevertheless, only scanty data are available for rheumatic patients in general and the ones affected by IIM in particular. To the best of our knowledge, ours represent the largest cohort of IIM patients in which immunogenicity of anti-SARS-CoV2 vaccine was assessed. In line with real-life data from other diseases, we found a non-statistically signifcant risk of relapse in our patients, which occurred seldom, usually mild and in patients with a more severe and aggressive course of disease.
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INTRODUCTION SARS-CoV-2 pandemic has having brought about profound changes in the socio-health sector, especially in the provision of the main levels of hospital and territorial assistance, with consequences of public health. To date, the management path of pregnant women, CoV-2 affected, and of their newborns is also well defined: in the absence of defined risk factors and/or in presence of good general clinical conditions of the woman, the completion of the pregnancy with caesarean section is not recommended, such as preterm labor (<37 weeks) is not justified in the absence of ascertained conditions that could endanger the pregnant or the fetus. Vertical transmission of the new coronavirus infection (intrauterine, intrapartum or peripartum) is very rare, and postnatal horizontal infection is more likely. In the event that a newborn were to become infected, most of them are asymptomatic and do not require ventilatory support;the main risk factors for severe disease are being preterm and having severe underlying medical conditions. Since the likelihood of having a SARS-CoV-2 positive rhino-pharyngeal swab is similar between infants separated from their mother at birth, compared to those in rooming-in (where preventive measures are implemented), current guidelines of the Italian Society of Neonatology approve the maintenance of the mother-child dyad, unless the mother is unable to care for her newborn. UOC of Neonatology with NICU of 'Vincenzo Cervello' hospital in Palermo, responsible for the management of COVID-19 positive births in Western Sicily, has conducted a study to identify how many children were born from CoV-2 affected mothers, the number of vertically infected newborns, the number of preterm births, underling how these data relate to the epidemiological curve of infection. METHODS This cross-sectional study was conducted over a period of 12 months, from March 2020 to March 2021, plotting the data on a histogram. The percentages of preterm births and positive newborns in the first 48-72 h of life (likely vertical transmission) were also calculated. RESULTS The percentage of preterm births (about 7.4%) appears to be slightly lower than the average of the national and international data (10%), probably linked to the small number of the sample, the main limitation of this study. The rarity of vertical transmission is evidenced by the only case of a positive birth from a positive mother (0.8% of the total). The greatest number of related COVID-19 births took place on the 19th of November 2020, when there was a peak of 1837 cases of new infected in a single day, with over a third of cases recorded in the provinces of Palermo and Trapani (Figure 1). The second peak has occurred on the 15th of January 2021, with 1945 new cases of infected people without a concomitant trend in pregnant women. CONCLUSIONS From the results obtained it is evident that the births-COVID curve partially follows the local epidemiological one (Figure 2): many factors can influence the epidemiological curve's trend such as the basic seasonality in births. Further studies are necessary to underline if a real correlation is present or not.
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Background: The severe measures of lockdown imposed in Italy to limit the SARS coronavirus 2 disease (COVID-19) spread caused an increase of reported anxiety, depression and suicidal rate among general population. Patients affected by rheumatic disorders feature an increased risk of mood disorders for the chronic course of the disease itself and for the related disability. Objectives: Aim of this study was to investigate the impact of COVID-19 lockdown on emotional well-being of a large cohort of rheumatic patients through a telemedicine approach. Methods: Patients in follow-up in rheumatologic out-patient clinics of our hospital were invited to participate to an online survey. They were asked also to invite their best friend, matched for age and sex, to participate the survey, as control group. The online survey included demographic questions and validated, psychometric scales for stress vulnerability (Stress Vulnerability Scale-SVS), resilience (Resilience Scale-RS), depression (Zung's depression questionnaire-Zung-D) and anxiety (Zung's anxiety questionnaire-Zung-A) evaluation. Results: The cohort was composed by 484 subjects (84,1% F, 15,9% M). The number of subjects and the frequency of various diagnosis are shown in Table 1. According to the psychometric scales, 55,5% and 43,3% of subject showed respectively an increased stress vulnerability and a reduced resiliency. Moreover, 64% and 40,5% of the enrolled subjects reported respectively anxiety and depressive symptoms worthy of psychiatric attention. There was a significant different distribution of scores for SVS (p<0,0001), Zung-A (p<0,0001) and Zung-D (p<0,0001) among the various diagnosis. In comparison with controls, higher scores of SVS were present in connective tissue diseases (CTD) (p=0,007), Sjogren's Syndrome (SSJ) (p=0,0029) and fibromyalgia (FM) (p<0,0001) patients, higher scores of Zung-A were present in SSJ (p=0,006) and FM (p<0,0001) patients and higher scores of Zung-D were present in FM (p<0,0001) patients (Figure 1). Ordinal regression analysis showed that higher classes of anxiety were independently predicted by the Tension (β=0,32;CI=0,13-0,52;p=0,003) and Demoralization (β=0,22;CI=0,04-0,44;p=0,046) components of SVS and by the Zung-D score (β=0,09;CI=0,05-0,1;p<0,001), while higher classes of depression were independently predicted by SVS total (β=0,17;CI=0,03-0,30;p=0,012), by its subcomponent Demoralization (β=0,22;CI=0,01-0,43;p=0,038), by a lower absolute RS score (β=-0,083;CI=-0,1-0,06;p<0,001) and by the Zung-A score (β=0,11;CI=0,06-0,15;p<0,001). In both cases, a specific diagnosis was not associated to a higher risk of advanced anxiety and depression classes. Conclusion: Rheumatic patients developed a high frequency of anxiety and depressive symptoms following COVID-19 lockdown, of which a large part should be referred for specialist attention according to their severity. There was a large variability of the symptoms reported among the various diagnosis. CTD, SSJ and FM patients were the most susceptible to the development of anxiety, depression and stress vulnerability. The application of psycometric scales through a telemedicine approach represents a useful tool to identify patients with higher levels of anxiety and depression.