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BackgroundAnti-synthetase syndrome (ASS) is a rare auto-immune condition that combines autoantibodies and specifics clinical manifestations, including myositis, interstitial lung disease (ILD), polyarthritis, mechanic's hands, Raynaud's phenomenon, and unexplained fever. The hallmark of this syndrome is the presence of anti-aminoacyl-tRNA-synthetase (ARS) antibodies. Several anti-ARS antibodies have been described, anti-Jo1 being the most common, followed by anti-PL7, anti-PL12, anti-OJ, anti-EJ, anti-KS, anti-YRS, and anti-Zo. According to a recent epidemiological survey, the rising number of patients with autoimmune diseases, including idiopathic inflammatory myopathies (IIM) coincides with the COVID-19 pandemic.ObjectivesTo evaluate the clinical characteristics of ASS patients with different anti-ARS antibodies from a tertiary rheumatology center.MethodsWe conducted a retrospective, single-centered study on consecutive patients diagnosed with ASS from 1 January 2015 to 31 December 2022. Clinical and serologic data were obtained by medical records review from hospital database. Myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) were tested using commercial ELISA kits. We included all patients fulfilling Connor's criteria for ASS.ResultsSixty-one patients (44 females) with mean age 54.4 (13.8) years were included. The most frequently reported clinical manifestation was arthralgia (68.8%), followed by Raynaud's phenomenon (67.2%), ILD (65.6%), myositis (46%), mechanic's hands (44.3%), arthritis (39.3%), and fever (18.0%). The typical triad for ASS, including myositis, arthritis and ILD was present in 17 patients. Twenty-eight (45.9%) patients were PL7+, 21 (34.4%) were Jo1+, 3 (4.9%) were PL12+, and 2 (3.2%) were OJ+. Seven patients were positive for more than two anti-ARS antibodies. The most frequently found MAA was anti-Ro52 (n=23, 37.7%). Of the 61 patients included, 41 (67.2%) patients were diagnosed in the last 3 years (COVID-19 pandemic). The most frequently detected MSA in ASS patients diagnosed during COVID-19 pandemic was anti-PL7 (25/28), while anti-Jo1 was the most common MSA in ASS patients diagnosed before 2020 (p<0.05) (Fig 1).The anti-Jo1+ patients were younger, have significantly more frequent muscle involvement and significantly higher levels of CK than anti-PL7+ patients (p<0.05). The co-occurance of anti-Ro52 antibodies was more frequently observed in anti-Jo1+ patients (n=11, 52.4%) than in anti-PL7+ patients (n=6, 21.4%) (p<0.05). We did not find statistically significant differences between ASS groups regarding sex, disease duration, clinical manifestations including dermatologic lesions, Raynaud's phenomenon, arthralgia/arthritis, ILD, fever, and cancers (all p>0.05).ConclusionASS patients have heterogenous manifestations, and different types of anti-ARS antibodies are associated to distinct clinical and immunological features. The COVID-19 pandemic led to increase prevalence of ASS cases and to a remarkable shift in the anti-ARS antibodies profile, with increased frequency of anti-PL7 antibodies. Further studies are needed to investigate the link between SARS-CoV-2 infections and myositis.References[1]Witt LJ, et al. The Diagnosis and Treatment of Antisynthetase Syndrome. Clin Pulm Med. 2016 Sep;23(5):218-226.[2]Gracia-Ramos AE, et al. New Onset of Autoimmune Diseases Following COVID-19 Diagnosis. Cells. 2021 Dec 20;10(12):3592.[3]Connors GR, et al. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest. 2010 Dec;138(6):1464-74.[4]García-Bravo Let al. Association of anti-SARS-COV-2 vaccine with increased incidence of myositis-related anti-RNA-synthetases auto-antibodies. J Transl Autoimmun. 2022 Jun 30;5:100160.Figure 1.ASS patients with positive anti-ARS antibodies per year (from 2015 to 2022). The green line shows the PL7+ patients;and the orange line shows the Jo1+ cases.[Figure omitted. See PDF]AcknowledgementsI have no acknowledgements to declare.Disclosure of Inter stsNone Declared.
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Objectives: BIOBADAGUAY is the Paraguayan/Uruguayan registry of adverse events in patients with inflammatory rheumatic conditions under biologic therapy (BT). Three years have elapsed from the first case of coronavirus and data about South American patients with COVID are still scarce. In this study we analyzed the frequency and clinical outcomes of COVID-19 in a cohort of patients with rheumatic diseases from Paraguay. Method(s): A cross sectional study of Paraguayan patients with rheumatic diseases from BIOBADAGUAY and controls without BT. Clinical, epidemiological, and COVID-19 data were analyzed. Only cases confirmed by SARSCoV-2 positive PCR test were included. Descriptive analysis were performed for this study. Result(s): 832 patients were included (696 under BT and 136 controls). 116 (13.9%) had COVID-19. 22 had a second infection and 9 a third reinfection. Table 1 shows characteristic of COVID-19 patients. The most frequent diagnosis was rheumatoid arthritis (n = 93, 80.2%) followed by ankylosing spondylitis (n = 6, 5.2%), undifferentiated spondylarthritis (n = 5, 4.3%), psoriatic arthritis (n = 4, 3.4%), juvenile onset arthritis (n = 2, 1.7%), vasculitis (n = 2, 1.7%). Only 1 case (0.8%) were registered for Still's disease, enteropathic spondylarthritis, systemic sclerosis and seronegative polyarthritis, respectively. When comorbidities were analyzed, 46 (39.6%) patients had at least one (Table 1). Of the total treatments received: 65 (56.0%) had methotrexate, 53 (45.7%) leflunomide, 3 (2.5%) sulfasalazine, 15 (12.9%) hydroxychloroquine, 25 (21.5%) glucocorticoid, 52 (44.8%) anti-TNF and 20 (17.2%) non-anti-TNF. COVID-19 severity outcomes were: 101(87%) non severe, 31 (26.7%) severe and 1 fatal(0.8%). 189 (90.9%) patients received vaccination and the mean number of doses were 2.5 doses. 55 (26.4%) had COVID prior to vaccination Conclusion(s): In this study we examined the frequency of COVID-19 in Paraguayan patients with rheumatic diseases. In this cohort of rheumatologic patients, COVID 19 severity was similar to the one in the general population.
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Background/Aims In the recent past, there has been growing research interest in COVID- 19 vaccines and their impact on the disease dynamics of rheumatic and musculoskeletal diseases (RMDs). The intersection of COVID-19 and autoimmunity has led to the theoretical possibility of flare-ups of autoimmune diseases with COVID vaccines. This study was conducted to evaluate the occurrence and the nature of flare-ups following the COVID vaccination on patients with RMDs. Methods This cross-sectional analytical study was conducted at Ragama Rheumatology and Rehabilitation Hospital, Sri Lanka involving 248 clinic patients using a structured questionnaire. Results The mean age of the study population was 52.69 and 75.4% were females. 12 patients (4.7%) reported flare symptoms following 1st dose of vaccination. Of them, 67% were females and 41.7% were within the 40-45 years age group. 75% of patients have experienced the onset of the flare symptoms following 1 week of the vaccination and 41.7% of symptoms have lasted more than 8 weeks. 66.7% who had this flare have received Sinopharm, while 25% received Covishield. In this flare 58.3% got polyarthritis,16.7% monoarthritis, 8.3% oligoarthritis and 16.7% generalized rash. By contrast, 42 (16.9%) patients who received the 2nd dose of the vaccine had flare symptoms. 90.5% of this population were females and 38.1% were within the 50-59 years age group. 42% got flare following 1st week of the vaccination and 57% of symptoms have lasted more than 8 weeks. 5 patients who had flare symptoms following the first dose reported having flare after the 2nd dose too. None of the flare symptoms following 1st or 2nd dose of the vaccinations needed hospitalization. Interestingly gender (p=0.012), use of methotrexate (p=0.043), and the presence of flare to the first dose (p=0.02) were found to be significantly correlated with the occurrence of flare symptoms following 2nd dose of vaccination. Conclusion This study reveals a considerable incidence of non-severe RMD flareups following COVID vaccination, mainly with the 2nd dose. Further studying on the effects of repeated and periodic COVID vaccination among patients with RMDs is timely to reassure and to improve vaccine acceptance in this group of patients.
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Background: It is well established that severe forms of SARS-CoV2 infection can induce a massive cytokine storm, which may disrupt the immune system stability and conceivably stimulate the development of reactive manifestations through a molecular mimicry process. Likewise, anti-COVID-19 vaccines, which have so far proved an excellent tolerability and safety profile, are able boost the immune response via different biologic technologies and adjuvant combinations possibly facilitating, in predisposed subjects, the onset of infammatory or even autoimmune manifestations. Objectives: We report a case series of suspected rheumatic adverse events following immunization (AEFI) associated with anti-COVID-19 vaccine. We focused our attention on the prognosis of these patients by analysing their available follow-up data. Methods: We included patients evaluated at frst-aid rheumatologic consultancy and at rheumatologic outpatient and inpatient clinic at Padua University Hospital between May and September 2021 presenting with new-onset rheumatic manifestation or a fare of an underlying rheumatic disease within 30 days after receiving an anti-COVID-19 vaccine dose. Inclusion and exclusion criteria were in accordance with the World Health Organization guidelines for AEFI surveillance. All patients were re-evaluated in January 2022: telemedicine or face-to-face visit. Response to therapy was classifed as complete, good or absent according to the clinician's judgment based on clinical examination, patient's reporting and analysis of laboratory data. Results: We identifed 30 cases of suspected rheumatic AEFI reported in Table 1. Comprehensively the most common manifestations were infammatory arthritis (40.0%), rheumatic polymyalgia (26.7%) and adult-onset Still disease (13.3%). Among patients with an underlying rheumatic disease we recorded an AOSD fare, a rheumatoid arthritis fare with involvement of hands proximal inter-phalangeal joints, one case of wrist arthritis in a patient with psoriatic arthritis, one of aortitis in a patient with large vessels vasculitis, one case of polyarthritis in undifferentiated connective tissue disease and a nephritis fare in a patient with systemic lupus erythematosus. Treatment for the suspected AEFI was based on systemic glucocorticoids (GC) alone (63.3%), systemic GC plus IL-1R antagonists (13.3%), non-steroidal autoinfammatory drugs (13.3%), intra-articular GC (6.6%), colchicine (3.3%) and non-steroidal anti-infammatory drugs (13.3%). At last follow-up contact (7.8±1.5 months) 26 patients (89.6%) were classified as complete responders. Eleven of them (42.3%) withdrew therapy without experiencing recurrence of disease manifestation. One patient with lupus nephritis had a proteinuric flare after the first BNT162b dose;he showed an initial good response to increased glucocorticoid therapy but had a new 24h proteinuria increase at second follow-up visit three months later requiring implementation of immunosuppressive therapy. Another patient with AOSD was in remission at last FU visit in December 2021 but required hospitalization in January 2022 for disease relapse due to a suspected gastrointestinal infection. Finally, one patient hospitalized for a seronegative polyarthritis after the first BNT162b dose achieved complete remission at last available contact (one month after hospital discharge) but was then lost in follow-up. Conclusion: After a mean follow-up of 7.8±1.5 months nearly all of patients showed a complete/good response to standard therapy and about half of them withdrew the treatment without losing the remission status.
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Background: According to the recent medical literature, COVID-19 disease can lead to a constellation of clinical syndromes lasting well beyond the frst 30 days of infection. The most common post COVID sequalae includes pulmonary, nervous system and neurocognitive, mental, metabolic, cardiovascular, gastrointestinal and several other clinical manifestations. Regarding joint involvement and particularly reactive arthritis (ReA), literature data is limited and describes case reports or series of cases of patients diagnosed with this condition following COVID-19 disease. Objectives: To describe the pattern and the management of post-COVID reactive arthritis. Methods: We have conducted a descriptive study of consecutive adult patients who presented to rheumatology outpatient clinic for joint or peri-articular pain/swelling/stiffness and received a diagnosis of post-COVID 19 reactive arthritis, by excluding other types of rheumatological conditions. The assessed clinical variables were: visual analogue scale (VAS) pain, swollen joint count (SJC), tender joint count (TJC), duration of morning stiffness, presence of enthesitis/tendinitis and axial involvement. Biochemistry and serology was performed: rheumatoid factor, ACPA, ANA, HLA B27, antiChlamydia Trachomatis, Ureaplasma Urealyticum and Mycoplasma Hominis Ab, anti HBs and HBc Ab, and anti HCV. COVID-19 disease prior to diagnosis of ReA was confrmed by PCR test. Results: In the study were included 16 patients with confrmed post COVID-19 ReA. The mean age of the study group was 43.5±10.8 (range 21-60), the female: male ratio was 4:1 and the duration of joint symptoms was 10.4±11.8 (range 1-42) weeks. The severity of COVID-19 disease was mild in 68.7% cases, moderate in 18.7% and severe in only 6.2% of the cases. The duration between COVID-19 diagnosis and ReA varied between cases, with a mean value of 4.3±4.2 (range 1-12) weeks. In 43.7% of the cases patients had peripheral joint involvement (synovitis), in 37.5%-periarticular involvement (enthesitis), 6.25%-isolate axial involvement (sacroiliac joints), 6.25% enthesitis and axial involvement (cervical spine) and 6.25% synovitis and enthesitis. In patients with peripheral joint pattern, the distribution of pain was symmetric (71.4%). The pattern of synovitis was determined by a TJC of 6.25±5.2 (range 1-16) joints and SJC 1.6±2.4 (range 0-7) joints. Both TJC and SJC correlated positively with the duration of morning stiffness (r=0.9 and r=0.6), but did not correlate with the VAS pain scale. In most of the cases synovitis affected the hand (wrist, MCP and PIP) 62.5% and the knee, feet and ankles-50%. Two patients presented with monoarthritis, 1 with oligoarthritis and 5 with polyarthritis, in the majority of cases, involvement being symmetric (75%). Periarticular pattern was determined by enthesi-tis, affecting the elbow and shoulder (50%), costo-sternal enthesitis (25%) and trochanteritis (25%). From the entire study group, 31.2% had elevated serum infammatory markers (ESR and/or CRP). Patients responded well to NSAIDs alone in 68.7% cases, local (intra-articular or peri-articular infltrations) or and systemic corticoids (5 mg Prednisolone equivalent) were administered in 5.3% and 12.5% cases respectively, in 12.5% cases (two patients) Methotrexate was administered. Conclusion: Reactive arthritis represents a post COVID-19 sequelae. The time of onset of ReA varied between 1 and 12 weeks after COVID-19 diagnosis. The clinical pattern of the disease was expressed by joint or periarticular involvement, mainly affecting the hand, feet and knee symmetrically. Cases of axial manifestations were less common. Most of the patients responded well to NSAIDs, only in a few particular cases, low doses of corticoids and/or Methotrexate were recommended.
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Background: Primary Sjögren's syndrome (pSS) is a chronic and progressive multisystem autoimmune disease which rarely onset in children and adolescents. Diagnostic delay in large part of patients are common due to the non-specifc and variable symptoms and the slow progression of disease. Objectives: To analyse demographic data, specifc extraglandular, salivary and ocular manifestations, laboratory parameters and therapy of pSS with juvenile onset. Methods: Retrospective study of all patients (pts) with pSS in single center. Results: pSS was verifed in 15 pts (6.7% were boys), which amounted to 23.8% of all pts with SS in our pediatric rheumatologic department. The median age of pSS onset was 8.0 y.o. [IQR 7.0;10.2]. The median of disease duration at the time of pSS verifcation was 2.75 years [2.2;5.6]. All patients had systemic manifestations at onset: constitutional abnormalities-33.3%, nonerosive polyarthritis-64.3%, polyarthralgias-26.7%, lymphadenopathy-73.3%, cutaneous involvement-53.3% (2-xerosis, 2-annular erythema, 1-erythema nodo-sum, 2-Raynaud phenomenon, 2-nonspecifc spotty rashes, 1-hemorrhagic rash). At the time of diagnosis 7 pts (46.7%) had isolated involvement of salivary glands, 8 pts (53.3%)-combined with involvement of lacrimal glands. The decrease in salivary gland function was recorded in 80% of cases, hypolacrimia-in 46.7%, 1 patient had isolated hypolacrimia. Recurrent parotitis was present in 6 pts (40.0%). At time of diagnosis pulmonary involvement had 20.0% of pts, 1 patient had renal tubular acidosis. 8 pts (53.3%) had various hematological disorders: anemia-in 3 pts (20.0%), leukopenia-in 6 (40.0%). ANA Hep-2 were detected in 100% pts (in titer 1/640-4, 1/1280-7, 1/2560-3, 1/20480-1, with mixed patterns in all pts: speckled + homogeneous-9 pts, speckled + homogeneous+cytoplasmic-6 pts), anti-Ro-in 12 pts (80.0%), anti-La-in 8 pts (53.3%), RF+-in 9 pts (60.0%). 6 pts (40.0%) had polyclonal hypergammaglob-ulinemia, max 42%. 2 pts (13.3%) had concomitant autoimmune non-rheumatic disease;1-cutaneous psoriasis, 1-autoimmune thyroiditis. The treatment of each patient was justifed by the main individual manifestations: 93.3% received glucocorticoids, 26.7%-methotrexate, 33.3%-hydroxychloroquine, 6.7%-mycophenolate mofetil. Treatment with biologics (B) was received by 13 (93.3%) pts (7-rituximab (RTM), 6-abatacept (ABA)) with a good response in 10 pts, including improvement in the function of the salivary and lacrimal glands in 7 pts. 1 patient received 2B-RTM and ABA sequentially due to the development of MAS 7 days after 1st RTM infusion. B was discontinued in 3 pts: 1 due to development of hemorrhagic vasculitis 2 days after the 1st RTM infusion, 1-COVID-19 with lung involvement (CT 3-4) 2 weeks after the 1st RTM infusion, 1-inefficiency of ABA during 15 months. Conclusion: In our pediatric rheumatologic department pts with pSS made up less than a quarter of all pts with SS. The diagnosis was verifed delayed in all pts, which can be explained by a wide range of nonspecifc manifestations at the onset. However, the manifestations of SS that were present at the time of diagnosis were brought under control on the background of complex therapy, including the prescription of B, with a good efficacy and safety profile of therapy.
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Background: The EPISER study is the frst Spanish epidemiological study that has confrmed the great burden of rheumatic diseases in the general population: they consume a large quantity of health resources (doctor visits, medical products) and imply a high social impact in terms of work absenteeism. Rheumatic diseases represent almost 30% of Primary Care medical consultations in Spain1,2. Electronic consultation could be an alternative response to the increase of this demand, both to make an early diagnosis and derivation and to improve communication with Primary Care physicians3,4. Objectives: To analyze the demand of Primary Care and its resolution through the electronic consultation system of the Rheumatology Department of a tertiary hospital. Methods: Retrospective descriptive study of the data collected in the request and information system (Sistema de Peticiones Electrónicas, SIPE) that supports electronic consultation between primary care physicians of the health area and the Rheumatology Department of a tertiary hospital, between July 2020 and May 2021.The following variables were collected: age, sex, reason for consultation, response time in days and destination (primary care/outpatient follow-up). Descriptive statistics were used to present the results. Results: The last 500 consecutive electronic consultations registered in the system, referring to 496 patients, were collected. Mean age was 59.5±17.7 years;74.2% women. Mean response time was 2 days, median response time 1 day and range 0-45. The reasons for consultation (see Graph 1) were: osteoporosis assessment 55 (11%), treatment adjustment 50 (10%), appointment request 49 (9.8%), loss to follow-up 43 (8.6%), local-regional pathology assessment 39 (7.8%), infltration request 28 (5, 6%), suspected rheumatoid arthritis 19 (3.8%), fare 18 (3.6%), suspected polymyalgia rheumatica or giant cell arteri-tis 16 (3.2%), COVID vaccine consultation 14 (2.8%), Raynaud's phenomenon 13 (2.6%), monoarthritis assessment 12 (2.4%), assessment of polyarthritis 11 (2.2%), adverse effects of treatment 11 (2.2%), suspected spondyloarthritis 11 (2.2%), suspected psoriatic arthritis 8 (1, 6%), generalized pain 7 (1.4%), suspected Sjögren's syndrome 5 (1%), suspected systemic lupus erythematosus 1 (0.2%), suspected other systemic autoimmune diseases 9 (1.8%), others 81 (16.2%). Fifty-seven and four % (287) of the patients required an appointment at the Rheumatology outpatient clinic and in 42.6% of the patients (213) the electronic consultation was successful, so it was not necessary to refer the patient to the hospital. Conclusion: Forty-two and six percent of the queries were resolved thanks to the electronic consultation system in an average of two days, otherwise that patients would have been referred to specialized care. The main reasons for consultation were osteoporosis assessment and clarifcation of doubts about the treatment of patients who were already being followed up by the Rheumatology Department.
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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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Three new drugs, all based on messenger RNA or small interfering RNA technology, represented a major therapeutic advance in 2021. But the bigger picture is that most of the new authorisations that advanced patient care were adaptations of existing drugs. And that more than half of this year's new authorisations were not advances, and in fact about one-tenth represented a step backwards compared to existing options.
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Background/Aims Vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has undoubtedly changed the course of the pandemic and lessened hospital admissions and death as a result of the aforementioned virus. Reactive arthritis has been reported with other vaccinations, for example influenza, and now it seems patients are presenting with similar symptoms post SARS-CoV-2 vaccination, the first human mRNA vaccine to be used against a virus. There are occasional case reports in the literature of patients developing various rheumatological conditions post COVID-19 vaccination including reactive arthritis and polymyalgia rheumatica. Currently the Summary of Product Characteristics (SmPC) for Pfizer BioNTech, Moderna and AstraZenica vaccinations only list myalgia and arthralgia as side effects in the musculoskeletal and connective tissue disorders section. Our aim was to collect data in a prospective manner on patients who presented to clinic with a new rheumatological condition which was believed to have been triggered by receiving a COVID-19 vaccination and their progress was followed over time. Methods Data have been collected prospectively from patients who have presented to the West Suffolk NHS Foundation Trust rheumatology department with symptoms which are thought to be attributable to COVID-19 vaccination over the last five months. Results Nineteen patients had a new inflammatory condition that appeared to have been triggered by the COVID-19 vaccination. The mean age of onset of symptoms is 65.2 years (range 22-85 years) with the mean number of days between vaccine and symptom onset of 9.6 days. 89% presented within 14 days of vaccination. Eleven patients received the Pfizer BioNTech vaccine and 6 the AstraZeneca vaccine. Seven patients presented after their first vaccine and seven after their second. Three had symptoms after both. The most common presentation was a small joint polyarthritis (8 patients) and polymyalgia rheumatica type symptoms (5). All but one were seronegative. So far, outcomes have varied between self-resolution or conservative management (6 patients), short- and long-term prednisolone requirement (6) and initiation of disease modifying anti-rheumatic drugs (5). Conclusion The emergence of post-COVID-19 vaccine inflammatory arthritis appears to be a new condition which rheumatologists will be required to treat. With the introduction of booster vaccinations and third doses this phenomenon is likely to be seen in clinic for the foreseeable future. Further data are required in order to guide best treatment options and enable better prognostic indicators.
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Introduction: Eosinophilic fasciitis (EF) is an uncommon scleroderma-like disorder resulting from infiltration of eosinophils and other white blood cells into the fascia. The etiology of EF is frequently idiopathic, but triggers have included trauma, medications, infection, and autoimmune conditions. Case Description: We present a case of EF in a 16-year old female with a history of Hashimoto’s thyroiditis, food allergies, and allergic rhinitis. Two weeks following her 1st dose of the Pfizer mRNA SARS-CoV-2 vaccine, she presented with generalized edema, weight gain (for 2-3 months prior), and polyarthritis. She did not have any scleroderma-like skin changes. Laboratory analysis was remarkable for eosinophilia (2130 cells/uL), elevated aldolase (15.6 U/L), and normal creatinine kinase. Lupus testing was unremarkable. MRI of bilateral thighs showed fasciitis and muscle/fascial biopsy demonstrated inflammatory myofasciitis consistent with EF. Given pending parasite studies, she received ivermectin prior to IV methylprednisolone therapy. Our patient has demonstrated significant improvement on prednisone, methotrexate, intravenous immunoglobulin, and hydroxychloroquine. Infectious work-up revealed positive Toxocara IgG levels, which prompted albendazole treatment. Discussion: Our case demonstrates an interesting perspective on the rare diagnosis of eosinophilic fasciitis. The etiology of her EF is unclear with confounding factors including her history of Hashimoto’s thyroiditis, positive Toxocara serology (IgG positive, IgM testing not available), and temporal relationship to Pfizer SARS-CoV-2 vaccine. This vaccine could have triggered an inflammatory process in the patient’s already hyper-reactive immune system. It is important to promptly recognize EF, as prolonged symptoms prior to diagnosis is associated with a poor treatment response.
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Presentation A 63-year-old man developed polyarthritis two months post recovery from COVID-19 infection. Diagnosis We concluded that the diagnosis was rheumatoid arthritis based upon raised inflammatory markers, positive rheumatoid factor and anti-cyclic citrullinated peptide antibodies. Treatment His symptoms improved with naproxen, corticosteroids, and methotrexate. Discussion We describe a patient with late onset rheumatoid arthritis possibly triggered or unmasked by COVID-19.
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Introduction: systemic lupus erythematosus with juvenile onset (jSLE), especially in boys, can occur with unusual manifestations, including Sjogren's syndrome (SS), accompanied by unexpected complications and difficulties in choosing therapy. Objectives: to present a rare case of successfully application of abatacept in an jSLE+SS in a boy who had two episodes of macrophage activation syndrome (MAS). Methods: Case report. Results: 4-year-old boy admitted to our clinic for the first time in 2010 presenting the 6-month history of disease. There were fever up to 39°C, polyarthritis, anemia, trombocytopenia, hyperIgG-emia, increase TA (2N), CRP (20 mg/l), RF 30 mg/l at the disease onset. Initially JIA, polyarthicular sybtype, RF+ was diagnosed in regional hospital. Treatment with NSAIDs, GC iv 125 mg No3, methotrexati 7,5 mg weekly was started. During the next 3 weeks laboratory results were constantly getting worse (HB 80 τ/π, trombocytes 145000, leucocytes 1000, increase TA 10N) and new clinical symptoms occurred (maculopapular rash with itching;splenomegaly;febrile fever). He received GC iv + per os 1 mg/ kg with a short-term effect. On 1st admission in our clinic in September of 2010, he had general fatigue and tiredness, classic malar rash, severe cutaneous vasculitis (palpable purpura and digital capillaritis), Raynaud's syndrome, enanthema, myopathy, lymphadenopathy, hepatosplenomegaly, polyarthritis. Data of laboratory tests: Hb 96g/l, ESR 27 mm/h, ANA titer 1:640 h+sp+cytopl, anti-SS-A(Ro)>200 U/ml, RF 230 ME/ml, C4 0.07 g/l. The revision of bone marrow biopsy showed changes typical for MAS. So jSLE was diagnosed as with SLICC criteria, 2012 with MAS at onset according the preliminary diagnostic criteria for MAS Complicating SLE. The initial SLEDAI was 29. Patient failed to respond to GC iv repeating courses, GC per os max 0.7 mg/kg, IVIG repeating courses, DMARDs consequentially: cyclophosphamide iv, azathioprine, mycophenolate mofetil (MMF) + hydroxychloroquine. Rituximab (RTX) was introduced in November of 2013 due to inefficiency of prior therapy in dose 375mg/m2 weekly N2 on course. Concomitant therapy: GC per os 0.5 mg/kg, MMF 500 mg/day, hydroxychloroquine 200 mg/day. Treatment with RTX allowed to decrease the dose of GC to 0.2 mg/kg, to reduce the activity of the disease (SLEDAI=4), but relapses of the disease required a repeat of RTX therapy every 6 months (5 courses in total). Patient developed a recurrent episode of MAS on 6th years of disease (the 8th day after RTX - 5th course, 1st infusion). Therapy of RTX was discontinued. In June 2016 Sjogren's syndrome has been verified (according to parotid sialography, unstimulated sialometry in combination with clinical signs of dry mouth, anti-SS-A(Ro)+, RF+). Correction of therapy was carried out at the expense of the change a dose of GC, the dose of MMF was increased to 750 mg per day. Patient received repeat courses of IVIG due to recurrent infections of mouth. Since October 2017 flare due to fever, polyarthritis, skin and mucosal lesions. The dose of GC has been increased to 0.5 mg/kg. In November 2017, abatacept therapy with 10 mg/kg was started with good safety. Inactive status of the disease was achieved after 12 months of therapy. Now boy receives abatacept during 42 months, continues GC 5 mg per day, hydroxychloroquine 100 mg per day, MMF 750 mg per day. In November 2020, he underwent COVID-19 with minimal manifestations (runny nose, fatigue, positive PCR test) without reactivation of rheumatic disease. Conclusion: This clinical case demonstrates efficacy and safety of abatacept in the rare combination of jSLE, SS and recurring episodes of MAS in boy with early onset.
ABSTRACT
Introduction: The past decade in rheumatology has seen tremendous innovation in digital health technologies. With a limited number and distribution of pediatric rheumatologists, telemedicine has been proposed as one way to meet this mission, yet the adoption of this modality has been slower than expected. The current global COVID 19 pandemic has triggered a paradigm shift in many centers to use telemedicine more widely Objectives: We report on the implementation of a telemedicine program in Russian Federation for the evaluation and treatment of patients with rheumatic diseases. Provide an overview of the use of telemedicine consultations in rheumatology department at the Federal Center. Methods: The telemedicine consultations department was established on the basis of the Federal Center on 7th of September in 2018. It is equipped with all the necessary equipment for organizing telemedicine consultations in real time, as well as holding deferred consultations on documents and selecting for hospitalization. Results: In 2020 433 requests were received (122 - emergency, 81 - urgent, 230 - planned), rheumatic disease were excluded in 48 patients, 196 patients were received the recommendations of investigations and correction of therapy, 189 patients were admitted (18 of them in the ICU). In 2021 (4 months) - 199 applications (52- emergency, 46 - urgent, 101- planned), rheumatic disease were excluded in 10 patients, 86 patients were admitted in the rheumatology department (5 of them in the ICU). In 2021 (from January to April) the number of applications increased 3 times in comparison with 2020. From April 2020 to April 2021, 44 initial applications and 19 repeated applications were received with a referral diagnosis of COVID 19, multisystem inflammatory syndrome. COVID 19 were confirmed in 10 patients. Other diagnoses were exposed: 6 - sepsis, 2 - PID, 1 - thrombophilia, 1 - relapse of leukemia, 5 - infection and 2 - deaths. 17 patients were admitted: sJIA were diagnosed in 8 patients, SLE -2, large B-cell lymphoma-1, colitis-1, Kawasaki-4, polyarthritis-1. There were patients from the remote regions: 30 were from Sakhalin Republic, 25 - Republic of Bashkortostan, 25- KhMAO, 28 -Orenburg Region, 14- Amur Region. Conclusion: Telemedicine is becoming more widespread, and the number of applications is increasing every year. We report the successful use of this service to assess the solution to the issue of hospitalization of children with rheumatic diseases for the initiation of DMARD in a region with limited access to rheumatological care.
ABSTRACT
Coronavirus disease 2019 (COVID-19) is caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) affecting mostly the respiratory system, but several other organs and systems can be involved. Extrapulmonary manifestations and autoimmune phenomena following SARS-CoV-2 infection are frequent events occurring during the first 2 weeks or in later stages of the disease course. These can be expressed as an isolated discovery of autoantibodies, mostly antinuclear or antiphospholipid antibodies, through to full-blown autoimmune organ-specific and systemic diseases. Joint pain is a frequent complain in most patients, but to our knowledge, frank arthritis has not been reported so far. A 46-year-old woman developed symmetrical polyarthritis 2 months after SARS-CoV-2 infection. Laboratory tests showed high acute phase reactants, while the immunological profile was negative. Hand and wrists X-rays revealed soft tissue swelling as well as bone erosions at the ulnar base of the third and fourth metacarpophalangeal joint of the right hand and carpal bones. The patient responded well to small doses of prednisone and methotrexate and after 4 months she had a sustained clinical and laboratory improvement. This is the first report making an association between SARS-CoV-2 infection and erosive polyarthritis. Physicians dealing with patients infected from SARS-CoV-2 should be aware for the possible development of musculoskeletal disorders, among them symmetrical polyarthritis. Thus, a close follow-up and monitoring is mandatory.
ABSTRACT
Reactive arthritis (ReA) following bacterial infection from the urogenital and gastrointestinal tract is widely described but is not typical post-viral infections. This report presents the second case of ReA after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in the United States. A 45-year-old black male with chronic low back pain was hospitalized for 45 days with coronavirus disease 2019 (COVID-19), complicated due to the development of multiorgan failure managed with intubation, extracorporeal membrane oxygenation, and hemodialysis. He was subsequently discharged to an acute rehabilitation facility where he complained of new-onset pain in his shoulders, left elbow, and left knee three weeks after a negative SARS-CoV-2 test. He was readmitted from his acute rehabilitation facility due to recurrent fever and the development of a swollen, warm left knee. Laboratory studies at readmission showed elevated inflammatory markers, negative extensive infectious disease workup, and aseptic inflammatory left knee synovial fluid without crystals. Testing returned negative for most common antibodies seen in immune-mediated arthritides (e.g., rheumatoid arthritis, systemic lupus erythematosus), as well as for common respiratory and gastrointestinal tract pathogens responsible for viral arthritis. The multidisciplinary inpatient medical team deemed the clinical presentation and laboratory findings most consistent with ReA. The patient received a course of oral corticosteroids, followed by a second course due to the recurrence of symptoms weeks after initial treatment and recovery. The current body of medical literature on SARS-CoV-2 pathophysiology supports plausible mechanisms on how this infection may induce ReA. Such a scenario should be considered in the differential of COVID-19-recovered patients presenting with polyarthritis as prompt steroid treatment may help patient recovery.