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Background. Significant gaps are present in the evidence of the spectrum and severity of COVID-19 infection in idiopathic inflammatory myopathies (IIMs). Patients with IIMs typically require immunosuppressive therapy, may have multiple disease sequelae, and frequent comorbidities, and thus may be more susceptible to severe COVID-19 infection and complications. The possibility of attenuated immunogenicity and reduced efficacy of COVID-19 vaccines due to concomitant immunosuppressive medication is a major concern in these patients, and there is little data available on COVID-19 vaccine breakthrough infections (BI) in IIMs Methods. We developed an extensive patient self-reporting electronic-survey (COVAD survey) featuring 36 questions to collect respondent demographics, SAID details, COVID-19 infection history, COVID-19 vaccination details, 7-day post vaccination adverse events and patient reported outcome measures using the PROMIS tool. After pilot testing, validation, translation into 18 languages on the online platform surveymonkey.com, and vetting by international experts, the COVAD survey was circulated in early 2021 by a multicenter study group of >110 collaborators in 94 countries. BI was defined as COVID-19 infection occurring more than 2 weeks after receiving 1st dose of a COVID-19 vaccine. We analysed data from the baseline survey for descriptive and intergroup comparative statistics based on data distribution and variable type. Results. Data from 10,900 respondents [42 (30-55) years, 74% females, 45% Caucasians] were analysed. Most were HCs (47%), followed by SAIDs (42%) and IIMs (11%). All respondents included in the final analysis had received a single dose of the vaccine and 69% had received 2 primary doses. Pfizer (39.8%) was the most common vaccine received, followed by Oxford/AstraZeneca (13.4%), and Covishield (10.9%). IIM patients were older, had a higher Caucasian representation and higher Pfizer uptake than other SAIDs, and HC. A higher proportion of IIM patients received immunosuppressants than other SAIDs. After adjustment for covariates, COVID-19 severity and BIs were comparable among patients with IIMs, SAIDs, and HCs, except for all-cause hospitalisation prior to vaccination (IIMs vs. HCs, OR=2.5, 95%CI 1.1-5.8) and COVID-19 cases prior to vaccination (IIMs vs. SAIDs, OR=0.6, 95%CI 0.4-0.8, and IIMs vs HCs, OR=0.4, 95%CI 0.3-0.5). BIs in IIMs were uncommon, with only 17 (1.4%) patients reporting BIs, of whom 13 were on immunosuppressants, and 3 required hospitalisation. Unvaccinated individuals with IIMs were at 4.6 (95%CI 2.7-8.0) times higher odds for developing COVID-19, and the 2nd vaccine dose had a protective effect on BI occurrence (Figure 1). Conclusion. IIMs patients reported fewer COVID-19 prior to vaccination than SAIDs and HCs, but had higher odds of all-cause hospitalisation than HCs. Vaccination protected patients with IIMs against COVID-19. COVID-19 breakthrough infections were uncommon in IIMs, with a similar symptom profile, disease duration, and severity in comparison to patients with SAIDs and HCs.
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Sjogren syndrome (SS) is a chronic, systemic autoimmune disease that primarily affects the exocrine glands, causing dry eyes and mouth, but also presents with a variety of other symptoms. SS is a common connective tissue disease but it can be difficult to diagnose due to the non-specific symptoms and lack of diagnostic markers in many cases. This report describes a case of an elderly patient on dialysis with newly diagnosed SS. The patient had been unable to eat a normal diet for a year, but treatment had not been initiated, presumably because of his age and the fact that he was on dialysis. The patient's symptoms improved with the administration of glucocorticoids. This is a very educational case for physicians to recognize undiagnosed SS patients presenting with non-specific symptoms.
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INTRODUCTION: We have previously shown that trained-immunity-based vaccines, namely TIbV, significantly reduce the rate of recurrent infections, both of the respiratory tract (RRTI) and urinary tract infections (RUTI) in SAD patients on disease-modifying drugs (DMARDs). OBJECTIVE: We evaluated the frequency of RRTI and RUTI from 2018 to 2021 in those SAD patients that received TIbV until 2018. Secondarily, we evaluated the incidence and clinical course of COVID-19 in this cohort. METHODS: A retrospective observational study was conducted in a cohort of SAD patients under active immunosuppression immunized with TIbV (MV130 for RRTI and MV140 for RUTI, respectively). RESULTS: Forty-one SAD patients on active immunosuppression that were given TIbV up to 2018 were studied for RRTI and RUTI during the 2018-2021 period. Approximately half of the patients had no infections during 2018-2021 (51.2% no RUTI and 43.5% no RRTI at all). When we compared the 3-year period with the 1-year pre-TIbV, RRTI (1.61 ± 2.26 vs. 2.76 ± 2.57; p = 0.002) and RUTI (1.56 ± 2.12 vs. 2.69 ± 3.07; p = 0.010) episodes were still significantly lower. Six SAD patients (four RA; one SLE; one MCTD) with RNA-based vaccines were infected with SARS-CoV-2, with mild disease. CONCLUSIONS: Even though the beneficial protective effects against infections of TIbV progressively decreased, they remained low for up to 3 years, with significantly reduced infections compared to the year prior to vaccination, further supporting a long-term benefit of TIbV in this setting. Moreover, an absence of infections was observed in almost half of patients.
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Background: Patients with autoimmune systemic diseases (ASDs) can be counted among frail populations as regards the predisposition to COVID-19 due to the frequent visceral organ involvement and comorbidities, as well as the ongoing immunomodulating treatments. Objectives: Our long-term multicenter telephone survey prospectively investigated the prevalence, prognostic factors, and outcomes of COVID-19 in Italian ASD patients during the frst 3 pandemic waves. Methods: A large series of 3,918 ASD patients (815 M, 3103 F;mean age 59±12SD years) was consecutively recruited at the 36 referral centers of COVID-19 & ASD Italian Study Group. In particular, ASD series encompassed the following conditions: rheumatoid arthritis (n: 981), psoriatic arthritis (n: 471), ankylosing spondylitis (n: 159), systemic sclerosis (n: 1,738), systemic lupus (172), systemic vasculitis (n: 219), and a miscellany of other ASDs (n: 178). The development of COVID-19 was recorded by means of telephone survey using standardized symptom-assessment questionnaire (1). Results: A signifcantly increased prevalence of COVID-19 (8.37% vs 6.49%;p<0.0001) was observed in our ASD patients, while the cumulative death rate revealed statistically comparable to the Italian general population (3.65% vs 2.95%;p: ns). In particular, among the 328 ASD patients complicated by COVID-19, 57 (17%) needed hospitalization, while mild-moderate manifestations were observed in the large majority of individuals (83%). In addition, 12/57 hospitalized patients died due to severe interstitial pneumonia and/or cardiovascular manifestations. Interestingly, a signifcantly higher COVID-19-related death rate was observed in systemic sclerosis patients compared to the Italian general population (6.29% vs 2.95%;p=0.018). Other adverse prognostic factors to develop COVID-19 were the patients' older age, male gender, pre-existing ASD-related interstitial lung involvement, and chronic steroid treatment. Conversely, patients treated with conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) showed a signifcantly lower prevalence of COVID-19 compared to those without (3.58% vs 46.99%;p=0.000), as well as the chronic administration of low dose aspirin in a subgroup of SSc patients (with 5.57% vs without 27.84%;p=0.000). Conclusion: The cumulative impact of COVID-19 on ASD patients after the frst 3 pandemic waves revealed less severe than that observed during the frst phase of pandemic (1), especially with regards to the death rate that was comparable to the Italian general population in spite of the increased prevalence of complicating COVID-19 in the same ASD series. Ongoing long-term treatments, mainly csDMARDs, might usefully contribute to generally positive outcomes of in this frail patients' population. Of note, a signifcantly increased COVID-19-related mortality was recorded in only SSc patients' subgroup, possibly favored by pre-existing lung fbrosis. Among different ASD, SSc deserves special attention, since it shares the main pathological alterations with COVID-19, namely the interstitial lung involvement and the endothelial injury responsible for diffuse microangiopathy. Besides SSc, the patients' subgroups characterized by older age, chronic steroid treatment, pre-existing interstitial lung disease, and/or impaired COVID-19 vaccine response (1-3), may deserve well-designed prevention and management strategies.
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Background: Patients with systemic autoimmune diseases (SADs) are often treated with drugs that interfere with the immune system and previous data showed a reduced seroconversion rate after anti-SARS-CoV2 vaccine in these subjects compared to healthy controls1. Administration of a booster dose of the vaccine could be particularly important in these patients, but data available to date are still scarce. Objectives: To evaluate the antibody response to the booster dose of mRNA SARS-CoV2 vaccine in patients with SADs and to compare it to the response after completion of the frst vaccination course. Secondly, to fnd possible correlations between a low antibody titre and patients' clinical features, with special regard to ongoing immunosuppressive therapies. Methods: Consecutive patients with an established diagnosis of SADs undergoing SARS-CoV2 vaccine were prospectively enrolled from January 2021;among them, we selected the patients who received the third vaccination dose between September and December 2021. Demographic and clinical data were collected at enrolment (sex, age, diagnosis, disease duration, ongoing therapies, previous SARS-CoV2 infection, presence of hypogammaglobulinemia);the last three elements were reassessed at each follow-up visit. Blood samples were collected 4 weeks both after the second (W4a) and the third (W4b) dose of the vaccine;a minority of patients was also tested 12 weeks after the second dose (W12). IgG antibodies to SARS-CoV2 receptor-binding domain (RBD) and neutralizing antibodies inhibiting the interaction between RBD and angiotensin converting enzyme 2 were evaluated. IgG anti-RBD were detected by solid phase assay on plates coated with recombinant RBD, while neutralising antibodies by using the kit SPIA (Spike Protein Inhibition Assay). Cut-off values were defned as the 97.5th percentile of a pre-vaccine healthy population. Statistical analysis was performed using IBM SPSS Statistics 20 and GraphPad Prism statistical packages. P values <0.05 were considered signifcant. Results: Forty-five patients (95.6% female;mean age ±SD 55.6±14.1 years;mean disease duration 12.9±10.6 years) were enrolled. Diagnosis was in most cases connective tissue disease (31/45, 68.9%), followed by infammatory arthritis (11/45, 24.4%) and systemic vasculitis (3/45, 6.7%). Two patients (4.4%) had a previous SARS-CoV2 infection and three had hypogammaglobulinemia (6.7%). At the time of the second dose, 18/45 patients were treated with glucocorticoids (GCs) [mean daily 6-methylprednisolone (6MP) dose 3.9 mg (min. 2, max. 14)], 17/45 with conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs) and 12/45 with biologic DMARDs (bDMARDs). At the third dose administration, 19/45 patients were treated with GCs [mean daily 6MP dose 4.1 mg (min. 1.5, max. 10)], 18/45 with csDMARDs and 13/45 with bDMARDs. Anti-RBD IgG were positive in 42/45 patients (93.3%) at W4a, in 16/18 (88.9%) at W12 and in 42/45 (93.3%) at W4b. Neutralizing antibodies were present in 38/45 patients (84.4%) at W4a, in 14/18 (77.8%) at W12 and in 42/45 (93.3%) at W4b. Both anti-RBD IgG titers and neutralizing antibody titers signifcantly increased after the third dose if compared to W4a (p<0.0001 both) (Figure 1). Interestingly, of the 7 patients who had not developed an adequate neutralizing antibody response after the frst vaccination course, 5 mounted an adequate titer after the booster. Two non-responder patients were both on combination therapy (one with low dose of GCs plus mycophenolate mofetil, the other with methotrexate and infiximab). Conclusion: Our data suggest that in patients with SADs there is a decline in the antibody titers developed after COVID-19 vaccination, however the booster dose is effective in restoring an adequate antibody titre. These data consolidate the importance of a booster dose of COVID-19 vaccination in patients with SADs to aid in the generation of an immune response.
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Background: Management of the health emergency caused by COVID-19 pandemic majorly disrupted the delivery of healthcare services to patients with chronic conditions like Systemic Autoimmune Diseases (SAD), both because resources were mainly channeled towards the care of infected patients, but also because patients tended to avoid seeking medical care for fear of becoming infected. PER-MAS is a 2-year project aimed at assessing the clinical, psychopathological, and socio-economic impact of COVID-19 in a cohort of patients with SAD. Objectives: To assess the impact of COVID-19 pandemic on drug withdrawal, disease fares and hospitalizations for disease exacerbation in a cohort of patients with SAD through an interim analysis of data from the PER-MAS project. Methods: A sample of 214 consecutive patients was recruited in a reference center for rare and complex autoimmune diseases from April 2021 to January 2022. Inclusion criteria were definite diagnosis of SAD (Connective Tissue Disease (CTD), Inflammatory Arthritis (IA) or Vasculitis), regular follow-up and at least 2 years of disease. Patients were asked to fill out an extensive self-administered questionnaire on disease activity and healthcare resource use during the pandemic (March 2020-moment of assessment). Pre-pandemic (March 2019-February 2020) and early pandemic (March 2020-February 2021) clinical data were recorded through retrospective chart review and patient interview. Results: At enrolment, 119 patients were affected by CTDs (55.6%), 71 by IA (33.18%), 24 by vasculitis (11.21%), with mean age 50.44± 12.97, and mean disease duration 11.17 ± 8.94. 30.37% took steroids, 39.7% hydroxy-chloroquine, 61.68% DMARDs, and 9.3% vasoactive drugs. Overall, disease course was similar in pre-pandemic and early pandemic phase: in the first period, rheumatologic condition was stable in 57.35% of patients, persistently active in 27.3% and 35.61% had ≥ 1 episode of disease exacerbation (mean 0.665±1.15, range 0-6);in the second period, 60.56% of patients was stable, 24.88% persistently active, and 39.44% had ≥1 exacerbation (mean 0.49 ±0.77, range 0-4). Mean number of visits (2.56±2.57 and 2.61±2.79), hos-pitalizations (0.168±0.698 and 0.14±0.473, p=0.6), number of patients with outpatient visits=0 (7.47 vs 7%), and number of patients with ≥ 1 hospital admission (10.28 vs 11.6%) were also similar, while the number of patients with hospital admissions for disease exacerbation was significantly higher in the second period (6.1 vs 11.21%, p=0.001). 170 patients completed the survey: from March 2020 to enrolment, 18.2% suspended ≥1 anti-rheumatic drug (6.25% of them for fear of contracting COVID-19 disease, 15.6% for difficulty in obtaining medications), 20% self-managed ≥ 1 disease exacerbation, and 40% had ≥ 1 telemedicine consult. From March to July 2020, 41.76% had their visit rescheduled (35.23% for hospital access restrictions, 5.3% for travel restrictions, 1.17% for fear). Conversely, only 14.7% of patients had their visit rescheduled (8.23% for hospital access restrictions, 4.7% for other reasons) from July 2020 to enrolment. Conclusion: In the early pandemic phase, overall disease course was similar to the pre-pandemic phase, but we observed an increase in the number of patients with ≥ 1 hospitalization for disease. Moreover, despite our efforts, patients reported a non-negligible rate of drug discontinuation for non-medical indication and difficulty to get access to rheumatologic consultation, highlighting the need of alternative organizational models in case of future pandemics.
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Background: Signifcant gaps are present in the evidence of the spectrum and severity of COVID-19 infection in idiopathic infammatory myopathies (IIM). IIM patients typically require immunosuppressive therapy, may have multiple disease sequelae, and frequent comorbidities, and thus may be more susceptible to severe COVID-19 infection and complications (1). The possibility of attenuated immunogenicity and reduced efficacy of COVID-19 vaccines due to concomitant immunosuppressive medication is a major concern in these patients, and there is little data available on COVID-19 vaccine breakthrough infections (BI) in IIM (2). Objectives: This study aimed to compare disease spectrum and severity and COVID-19 BI in patients with IIM, other systemic autoimmune and infammatory diseases (SAIDs) and healthy controls (HCs). Methods: We developed an extensive self-reporting electronic-survey (COVAD survey) featuring 36 questions to collect respondent demographics, SAID details, COVID-19 infection history, COVID-19 vaccination details, 7-day post vaccination adverse events and patient reported outcome measures using the PROMIS tool. After pilot testing, validation, translation into 18 languages on the online platform surveymonkey.com, and vetting by international experts, the COVAD survey was circulated in early 2021 by a multicenter study group of >110 collaborators in 94 countries. BI was defned as COVID-19 infection occurring more than 2 weeks after receiving 1st or 2nd dose of a COVID-19 vaccine. We analyzed data from the baseline survey for descriptive and intergroup comparative statistics based on data distribution and variable type. Results: 10900 respondents [mean age 42 (30-55) years, 74% females and 45% Caucasians] were analyzed. 1,227 (11.2%) had IIM, 4,640 (42.6%) had other SAIDs, and 5,033 (46.2%) were HC. All respondents included in the fnal analysis had received a single dose of the vaccine and 69% had received 2 primary doses. Pfzer (39.8%) was the most common vaccine received, followed by Oxford/AstraZeneca (13.4%), and Covishield (10.9%). IIM patients were older, had a higher Caucasian representation and higher Pfzer uptake than other SAIDs, and HC. A higher proportion of IIM patients received immu-nosuppressants than other SAIDs. IIMs were at a lower risk of symptomatic pre-vaccination COVID-19 infection compared to SAIDs [multivariate OR 0.6 (0.4-0.8)] and HCs [multivariate OR 0.39 (0.28-0.54)], yet at a higher risk of hospitalization due to COVID-19 compared to SAIDs [univariate OR 2.3 (1.2-3.5)] and HCs [multivariate OR 2.5 (1.1-5.8)]. BIs were very uncommon in IIM patients, with only 17 (1.4%) reporting BI. IIM patients were at a higher risk of contracting COVID-19 prior to vaccination than ≤2 weeks of vaccination [univariate OR 8 (4.1-15)] or BI [univariate OR 4.6 (2.7-8.0)]. BIs were equally severe compared to when they occurred prior to vaccination in IIMs, and were comparable between IIM, SAIDs, and HC (Figure 1), though BI disease duration was shorter in IIMs than SAIDs (7 vs 11 days, p 0.027). 13/17 IIM patients with BI were on immunosuppressants. Conclusion: IIM patients experienced COVID-19 infection less frequently prior to vaccination but were at a higher risk of hospitalization and requirement for oxygen therapy compared with patients with HC. Breakthrough COVID-19 infections were rare (1.4%) in vaccinated IIM patients, and were similar to HC and SAIDs, except for shorter disease duration in IIM.
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Background: Patients with autoimmune systemic diseases have an increased risk to contract infections and develop severe complications;infections in turn can reactivated and worsen the disease itself in a vicious circle. Thus, vaccination is the main weapon to prevent infectious diseases and represents an important and safe instrument of care for rheumatic patients that needs to be further promoted. However, the immunosuppressive drugs used to treat rheumatic diseases may impair response to vaccines, in particular those targeting B or T cells directly (1). Objectives: The aim of this study is to evaluate the B and T-cell mediated immune response to mRNA vaccination in patients with systemic autoimmune diseases, such as vasculitis or systemic connective tissue diseases, early or continuously treated with B-cell targeting therapies, rituximab (RTX) or beli-mumab (BEL), by comparing with controls and each other. Secondary we evaluated the in vitro effective neutralizing capacity in belimumab-exposed patients. Methods: Twenty-eight consecutive patients under treatment with rituximab (RTX, n=11) or belimumab (BEL, n=17) and 13 age/sex matched controls (non-rheumatic healthcare personnel) were enrolled in the study. Nobody presented anti-SARS-CoV2 antibodies related to previous viral contact and all were always negative at the molecular swab monthly control. All patients and controls received mRNA vaccines and were tested three to four weeks after complete vaccination. All RTX patients started vaccination within 5 months from the last infusion, and all but one of them were B-cell depleted. Anti-SARS-CoV-2 RBD total antibodies were analysed by a diagnostic assay (Elecsys, Roche) while T-cell response was evaluated using the IGRA test (Euroimmun). A subgroup of BEL-patients was tested with pseudovirus neutralization assay. Results: Detectable anti-SARS-CoV2 RBD antibodies were documented in 1/11 RTX patients versus 16/17 BEL patients (p<0.0001). The median concentration was signifcantly lower than that observed in controls (39.6 AU/ml vs 1133 AU/ml, p<0.0001). A very low titer of anti-RBD antibodies were documented only in 1 out of 11 patients in the RTX subgroup (0.93 U/ml, positive if >0,79 U/ml) and the patient was the only one who showed an initial B-cell recovery (CD19+ B-cell 5 cells/microL). Anti-RBD antibodies were documented in 16 out 17 of patients in the BEL subgroup. The median anti-RBD antibody titer in patients receiving BEL was 243 [77.55-744.0] U/ml, and it was signifcantly lower compared to the controls (p=0.002). The IGRA test was positive in 8/11 (72.7%) RTX patients vs 16/17 (94.1%) BEL patients (p=0.7), with interferon release comparable to control subjects (p=0.2). Six patients with BEL were also stratifed according to total antibodies (IgG+I-gA+IgM) against-RBD into high responders (>800 AU/mL, n=3) and low responders (≤45 AU/mL, n=3) and tested with pseudovirus neutralization assay. Two thirds of low titer group of patients neutralized the Wuhan-Hu1 strain at medium-low titer (IC50 ≈102) but were almost ineffective in inhibiting the B.1.1.7 entry into target cells (IC50 =10). Regarding high responders, while two patients were able to inhibit both the strains at medium-high titer (approximately IC50 ≈103 for Wuhan-Hu1 and B.1.1.7), one patient neutralized only the WT strain. Conclusion: B-cell targeting therapies do not preclude SARS-CoV-2 vaccination since a cellular immunity can raise even in the absence of circulating B cells. Most importantly, the immunogenicity of COVID-19 vaccination in SLE patients treated with belimumab is supported. However, patients showing the lowest humoral response to vaccine could remain at higher risk of infections, due to low neutralizing capacity.
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Introduction: Antisynthetase syndrome(ASS) is a rare autoimmune disease characterized by myositis, arthritis, cutaneous findings and interstitial lung disease (ILD). In 15-30% cases of ASS, ILD can be the presenting feature making this a very challenging diagnosis to make, especially during the COVID 19 pandemic. We present a unique case of ILD as presenting feature of ASS. Case: A 31 yr old female, never smoker, with asthma and obesity was referred to the pulmonary clinic for dyspnea of 2 months. Dyspnea was on exertion, associated with cough and pleuritic chest pain. Oxygen saturation on exam was 91% at rest and 86% on exertion. PCR and antibodies for COVID was negative. CXR showed bilateral infiltrates. She was treated with several courses of antibiotics and steroids. Her symptoms improved with steroids yet returned when course completed. Chest CT revealed bilateral parenchyma opacities with sparing of the lung apices. This was repeated 3 months after her course of antibiotics and steroids which revealed worsening of ground glass opacities, now diffuse with areas of organizing pneumonia. Bronchoalveolar lavage showed alveolar macrophages with a mixture of acute and chronic inflammatory cells. PFTs revealed severe restrictive lung disease. Infectious work up including bacterial, viral and fungal causes was negative. Complete blood count with differential was normal. B-type natriuretic peptide, creatine kinase, liver function test, basic metabolic panel, HIV and fungal serologies were unrevealing. A rheumatologic work up revealed elevated ESR (48), CRP (6.9), aldolase (48) with positive anti OJ antibodies. She underwent VATs with wedge lung biopsy which revealed cellular non specific interstitial pneumonia (NSIP). She was diagnosed with ASS and started on a gradual taper of high dose steroids and steroid sparing agent Mycophenolate Mofetil. With time her respiratory symptoms improved and she no longer required supplemental oxygen. She was enrolled in pulmonary rehabilitation and encouraged to loose weight as her BMI of 55 could preclude lung transplantation if needed. Discussion: ASS is a rare autoimmune connective tissue disorder characterized by myositis, polyarthritis, cutaneous findings and ILD. It occurs more commonly in women with average age of onset in 50s. ILD has been reported in 69-100% with NSIP being most common followed by cryptogenic organizing pneumonia and UIP. Treatment consists of steroids, with or without a steroid-sparing agent. Timely diagnosis of ASS is imperative for patients presenting with ILD as delay can lead to progression of ILD which serves as a predictor for morbidity and mortality. (Figure Presented).
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Myxoma (mx) is the most frequent adult cardiac tumour, that often poses a difficult diagnostic challenge due to the variety and aspecifity of presenting clinical signs. Alongside the more typical clinical onset caused by intracardiac obstruction and systemic embolization, mainly at cerebral level, a mx may initially manifest itself with nonspecific systemic symptoms such as fever, weight loss, fatigue, skin rash, myalgia and arthralgia. We present the case of a 58-year-old woman diagnosed in December 2018 with idiopathic, serum-negative arthritis of the metacarpophalangeal joint of the first finger of the right hand, treated with methotrexate (Reumaflex 10 mg/week s.c.), hydroxychloroquine sulphate (Paquenil 200 mg/day per os) and corticosteroids cycles;in January 2021, this therapy was suspended by the patient (pt) as ineffective. In April 2021, pt was admitted for bilateral SARS-CoV-2 pneumonia. As dyspnoea and fatigue persisted during moderate physical activity (NYHA class II), the pt underwent a cardiological examination on 13 August, when echocardiographic diagnosis of a left atrial mass with the appearance of a mx was made. Interleukin-6 (IL-6) was assayed for a suspected relationship between the cardiac tumour and rheumatic symptoms and was found to be elevated (490 pg/m)l. On 23 August, cardiac surgery was performed to remove the left atrial mass by right minithoracotomy. Histopathological examination confirmed that the 3.5 x 2.5 x 1.5 cm neoformation was a mx. On 1 September, strong attenuation of joint pain and IL-6 reduction to 107 pg/ml was detected. On 9 October, resolution of rheumatic symptoms and normalisation of IL-6 to 3.7 pg/ml (N.V < 7) occurred. This clinical case is emblematic of the long time that sometimes can elapse between the first clinical manifestation and the diagnosis of mx. Also the widespread use of echocardiography did not significantly reduce the diagnostic delay when cardiac symptoms are absent. In a large number of cases, onset symptoms mimicking autoimmune connective tissue disease are reported for 5% (“Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases” Medicine (Baltimore) 2001 May;80(3):159-729). The monoarticular localisation we describe is however unusual. Finally, our observation confirms that the association between mx and systemic symptoms is most likely due to IL-6 synthesis by tumour cells. (Figure Presented).
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Background/Aims Chilblain-like lesions (perniosis) have been reported frequently during COVID-19 pandemic in children and adolescents with no history of exposure to cold temperatures or underlying autoimmune conditions. Patients with these skin changes reported mild COVID-19 symptoms or previous contact with confirmed COVID-19 cases before they became symptomatic. In the majority of cases, a causal relationship between SARS-CoV-2 infection and chilblain-like lesion has not been proven. Methods Retrospective review of patients with chilblain-like lesions, possibly secondary to SARS-CoV-2 infection, presenting to a tertiary Adolescent Rheumatology service between January and August 2021. Results We identified five, male, adolescent patients (mean age, 16 years old) who presented with new onset of chilblain-like lesions affecting fingers, toes and heels in December 2020, which coincided with the peak of second wave of COVID-19 infection. One month prior to skin changes occurrence, 3 out of 5 patients experienced mild respiratory COVID-19-like symptoms and the rest of the patients were asymptomatic but were in contact with COVID-19 positive cases following outbreaks in schools. 1 of 3 symptomatic patients had a positive COVID-19 PCR test prior to skin manifestations. 2 out of 4 patients with heel lesions had deep, full thickness skin loss heel ulcers and 2 of 5 patients had superficially ulcerated lesions on a finger and toes, respectively, resulting in inability to attend school. None of the patients had any other symptoms or signs to suggest an underlying autoimmune connective tissue disorder. Demographics, clinical features and serological data are summarised in Table 1. One patient underwent a biopsy of heel ulcer which was histologically consistent with perniosis. In two patients (40%) chilblain like lesions resolved spontaneously within 2 months. Three patients (60%), with progressive ulcerated lesions, required various combinations of treatments with aspirin, calcium channel blockers (nifedipine), topical or oral steroids and hydroxychloroquine with complete resolution of symptoms within 6 months. Conclusion Chilblain-like lesions, including heel involvement associated with mildly symptomatic COVID-19 infection, have been reported before. Our mini-case series raises awareness of ulcerating chilblain like lesions possibly secondary to COVID-19 in adolescent patients, which require early recognition and instigation of treatment leading to better patient's outcomes.
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OBJECTIVES: To describe the frequency of COVID-19 and the effect of vaccination in patients with interstitial lung disease and systemic autoimmune disease (ILD-SAD) and to identify factors associated with infection and severity of COVID-19. METHODS: We performed a cross-sectional multicenter study of patients with ILD-SAD followed between June and October 2021. The main variable was COVID-19 infection confirmed by a positive polymerase chain reaction (PCR) result for SARS-CoV-2. The secondary variables included severity of COVID-19, if the patient had to be admitted to hospital or died of the disease, and vaccination status. Other variables included clinical and treatment characteristics, pulmonary function and high-resolution computed tomography. Two logistic regression was performed to explore factors associated with "COVID-19" and "severe COVID-19". RESULTS: We included 176 patients with ILD-SAD: 105 (59.7%) had rheumatoid arthritis, 49 (27.8%) systemic sclerosis, and 22 (12.54%) inflammatory myopathies. We recorded 22/179 (12.5%) SARS-CoV-2 infections, 7/22 (31.8%) of them were severe and 3/22 (13.22%) died. As to the vaccination, 163/176 (92.6%) patients received the complete doses. The factors associated with SARS-CoV-2 infection were FVC (OR (95% CI), 0.971 (0.946-0.989); p = 0.040), vaccination (OR (95% CI), 0.169 (0.030-0.570); p = 0.004), and rituximab (OR (95% CI), 3.490 (1.129-6.100); p = 0.029). The factors associated with severe COVID-19 were the protective effect of the vaccine (OR (95% CI), 0.024 (0.004-0.170); p < 0.001) and diabetes mellitus (OR (95% CI), 4.923 (1.508-19.097); p = 0.018). CONCLUSIONS: Around 13% of patients with ILD-SAD had SARS-CoV-2 infection, which was severe in approximately one-third. Most patients with severe infection were not fully vaccinated.
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The clinical and immunological spectrum of acute and post-active COVID-19 syndrome overlaps with criteria used to characterize autoimmune diseases such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Indeed, following SARS-Cov2 infection, the innate immune response is altered with an initial delayed production of interferon type I (IFN-I), while the NF-kappa B and inflammasome pathways are activated. In lung and digestive tissues, an alternative and extrafollicular immune response against SARS-Cov2 takes place with, consequently, an altered humoral and memory T cell response leading to breakdown of tolerance with the emergence of autoantibodies. However, the risk of developing severe COVID-19 among SLE and RA patients did not exceed the general population except in those having pre-existing neutralizing autoantibodies against IFN-I. Treatment discontinuation rather than COVID-19 infection or vaccination increases the risk of developing flares. Last but not least, a limited number of case reports of individuals having developed SLE or RA following COVID-19 infection/vaccination have been reported. Altogether, the SARS-Cov2 pandemic represents an unique opportunity to investigate the dangerous interplay between the immune response against infectious agents and autoimmunity, and to better understand the triggering role of infection as a risk factor in autoimmune and chronic inflammatory disease development.
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OBJECTIVES: COVID-19 outcomes in population with systemic autoimmune diseases (SAD) remain poorly understood. The aim was to examine demographic and clinical factors associated with COVID-19 infection in people with rheumatic disease. METHODS: Two phases cross-sectional survey of individuals with rheumatic disease in April 2020 and October 2020. COVID infection, severity of disease, age, sex, smoking status, underlying rheumatic disease diagnosis, comorbidities and rheumatic disease medications taken immediately prior to infection were analyzed. RESULTS: A total of 1,529 individuals with autoimmunity disease diagnosis were included. Out of 50 positive patients, 21 required telephone medical assistance, 16 received assessment by primary care physician, 9 were evaluated in Emergency Department and 4 patient required hospitalization. Multivariate analysis was performed without obtaining differences in any of the systemic autoimmune diseases. Regarding the treatments, significant differences were found (p 0.011) in the treatment with anti-TNF-alpha agents with OR 3.422 (1.322-8.858) and a trend to significance (p 0.094) was observed in patients receiving mycophenolate treatment [OR 2.016 (0.996-4-081)]. CONCLUSIONS: Anti-TNF-alpha treatment was associated with more than 3-fold risk of suffering from SARS-CoV-2 infection, although in all cases infection was mild. Cumulative incidence in patients with SAD was up to 5 times higher than general population but with great differences between autoimmune diseases.
ABSTRACT
Guillain-Barré syndrome (GBS) has an annual incidence rate ranging from 0.4 to 1.7 cases per 1,00,000 population. Pharyngeal-cervical-brachial (PCB) variant is an extremely rare variant of GBS (3%), which presents with muscle weakness initially involving the neck, oropharynx, and upper extremities. GBS often has an infectious inciting event leading to an autoimmune response. There has been an increase in the incidence of GBS during the COVID-19 pandemic, and several case studies have shown an association between the development of GBS and COVID-19 infection. High clinical suspicion is needed to reach a diagnosis. As PCB variant of GBS can have fatal outcomes, a good clinical knowledge of its presentation can allow timely life-saving interventions. Here, we report a case of GBS with acute onset of neck and respiratory muscle weakness that progressed to upper limb weakness. The patient developed these symptoms two weeks after the onset of cough, fever, and malaise. PCB variant of GBS should always be considered as an important differential diagnosis in any patient presenting with limb weakness and bulbar palsy.
ABSTRACT
Patients suffering from autoimmune diseases appear to be at greater risk for developing infections with the influenza virus compared to healthy controls due to their immunosuppressive treatment, suggesting the importance of vaccination. Within this literature review, we highlight the importance, efficacy, and safety of influenza vaccination in individuals with autoimmune diseases, including systemic lupus erythematosus (SLE), Sjogren syndrome (SS), rheumatoid arthritis (RA), and inflammatory bowel disease (IBD) in both vaccinated and unvaccinated individuals. Overall, vaccination is generally well tolerated by SLE patients and the literature recommends the inactivated influenza vaccine to SLE patients according to the recommendations and schedules for the general population and annually against seasonal influenza viruses. While the data are still unclear in patients with SS, there does seem to be a general consensus to vaccinate these individuals to prevent harmful risks of influenza disease. In patients with RA and IBD, vaccination efficacy with the inactivated influenza vaccine should be determined on a case-by-case basis, taking patient therapy into account. In light of the current pandemic and global coronavirus disease 2019 (COVID-19) crisis, it is crucial to emphasize the safety and immunogenicity of influenza vaccination in vulnerable individuals suffering from autoimmune diseases. Public health measures are recommended to protect these individuals with vaccinations, keeping in mind the possibility of the multiple COVID-19 vaccines that are currently available.