A Case of C-ANCA Positive Systematic Lupus Erythematous and ANCA-Associated Vasculitis Overlap Syndrome Superimposed by COVID-19: A Fatal Trio.

Coronavirus disease 2019 (COVID-19) possesses a substantial challenge for rheumatologists and rheumatologic patients. They are concerned about the reciprocal interaction between connective tissue diseases, such as systemic lupus erythematosus (SLE), and the virus. Here, we report a 21-year-old female SLE patient presented to the emergency department with gastrointestinal symptoms and kidney involvement evidence. Based on the pathology and laboratory assessments, she was suspected of C-anti-neutrophil cytoplasmic antibody (ANCA) positive SLE and ANCA-associated vasculitis overlap syndrome (SLE/AAV OS), and plasmapheresis every other day was performed due to this diagnosis alongside the high titer of C-ANCA. We also administered methylprednisolone (1 g/day, IV) for three days, followed by dexamethasone with the maintenance dosage (1mg/kg/day, IV). Although the patient's general condition improved the next days, her condition deteriorated suddenly on the 7th day of hospitalization. She got intubated and went to the intensive care unit. Despite taking possible measures to manage the patient's condition, she eventually passed away due to severe acute respiratory distress syndrome (ARDS), triggered by COVID-19. The distinct role of C-ANCA in SLE/AAV vascular damage and activating neutrophil cytokine release accompanied by the impaired immune system while facing COVID-19 seems to lead to increased morbidity and mortality in such patients. This report was presented to bring into consideration the possible role of C-ANCA in the burden and prognosis of COVID-19 in SLE/AAV OS patients.

ANCA Associated Glomerulonephritis Following SARS-CoV-2 Vaccination: A Case Series and Systematic Review.

Vaccines against SARS-CoV-2 (COVID-19) proved beneficial for COVID-19 disease attenuation and preventing virus spreading. Cumulative reports of the rarity of antineutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitis (AAV) raise concerns about its relationship with COVID-19 vaccination. Several case reports described ANCA-associated pauci-immune glomerulonephritis (ANCA-GN) following COVID-19 vaccination with some uniqueness. We systematically reviewed COVID-19 vaccine-induced ANCA-GN from PubMed, SCOPUS, and Cochrane library databases until 1 January 2023 according to PRISMA guidelines and presented our three cases. Twenty-six cases from 25 articles, including our 3 cases, were analyzed. Most cases were diagnosed following the second dose of the COVID-19 vaccine (59%) with a median (IQR) interval onset of 14 (16) days. The highest prevalence was related to the mRNA-type vaccine. Anti-myeloperoxidase (MPO) ANCA was far more common than the other ANCAs, with various positive autoantibodies. Fourteen cases (out of 29 cases, 48%) had extra-kidney AAV manifestation. Although severe kidney injury was observed in 10/29 (34%), remission was achieved in 89% (25/28) with no death. The mechanisms of the vaccine-inducing ANCA-GN were postulated here. Since ANCA-GN after the COVID-19 vaccine was rare, the benefit of the COVID-19 vaccine could outweigh the risk of ANCA-GN side effects in the pandemic era.

Spontaneous Improvement of Aortitis Associated with Severe COVID-19 Infection-A Case Report.

Aortitis is a rare complication of the coronavirus disease 2019 (COVID-19) and is often treated empirically with steroids. We present a case of spontaneous resolution of aortitis without treatment. A 65-year-old man was admitted to our intensive care unit for severe COVID-19 pneumonia and underwent rehabilitation in the general ward. On day 12, he developed fever, and on day 13, he developed right cervical pain and increased inflammatory markers. On day 16, a cervical echocardiogram showed vasculitis in the right common carotid artery, and on day 17, computed tomography (CT) of the neck showed thickening of the arterial wall of the right common to the internal carotid arteries. A retrospective assessment of the CT scan on day 12 showed wall thickening from the thoracic aorta to the abdominal aorta, and a diagnosis of aortitis was made. Autoantibody analysis, culture, and magnetic resonance imaging (MRI) of the head and neck showed no abnormalities. During the investigation of the cause of aortitis, the fever and inflammatory reaction spontaneously resolved and the right cervical pain gradually improved. Therefore, the patient was diagnosed with transient COVID-19-related aortitis. To our knowledge, this is the first report describing the spontaneous resolution of COVID-19-related aortitis.

IgA Vasculitis Associated With COVID-19.

IgA vasculitis, also known as Henoch-Schonlein Purpura (HSP), is an inflammatory disorder of small blood vessels that can present with palpable purpura, arthralgias, abdominal pain, and kidney disease. It is most commonly found in pediatric patients after an inciting infection but has been seen across all ages and associated with certain drugs and vaccines. COVID-19 has been associated with various cutaneous manifestations, but HSP is a rarely reported one. We present a case of a 21-year-old female presenting with a petechial rash found to be seronegative IgA vasculitis presenting concurrently with dyspnea secondary to COVID-19. She was initially seen by an outside practitioner, tested negative for COVID, and was prescribed a course of oral prednisone. Shortly thereafter, she visited the ED for worsening shortness of breath and tested positive for COVID-19, for which she received Paxlovid. Biopsy after a visit to a dermatologist confirmed intramural IgA deposition on immunofluorescence, and she was tapered off prednisone and started on azathioprine.

Advances in Management of the Stroke Etiology One-Percenters.

PURPOSE OF REVIEW: Uncommon causes of stroke merit specific attention; when clinicians have less common etiologies of stoke in mind, the diagnosis may come more easily. This is key, as optimal management will in many cases differs significantly from "standard" care. RECENT FINDINGS: Randomized controlled trials (RCT) on the best medical therapy in the treatment of cervical artery dissection (CeAD) have demonstrated low rates of ischemia with both antiplatelet and vitamin K antagonism. RCT evidence supports the use of anticoagulation with vitamin K antagonism in "high-risk" patients with antiphospholipid antibody syndrome (APLAS), and there is new evidence supporting the utilization of direct oral anticoagulation in malignancy-associated thrombosis. Migraine with aura has been more conclusively linked not only with increased risk of ischemic and hemorrhagic stroke, but also with cardiovascular mortality. Recent literature has surprisingly not provided support the utilization of L-arginine in the treatment of patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS); however, there is evidence at this time that support use of enzyme replacement in patients with Fabry disease. Additional triggers for reversible cerebral vasoconstriction syndrome (RCVS) have been identified, such as capsaicin. Imaging of cerebral blood vessel walls utilizing contrast-enhanced MRA is an emerging modality that may ultimately prove to be very useful in the evaluation of patients with uncommon causes of stroke. A plethora of associations between cerebrovascular disease and COVID-19 have been described. Where pertinent, authors provide additional tips and guidance. Less commonly encountered conditions with updates in diagnosis, and management along with clinical tips are reviewed.

Rheumatoid Vasculitis Complicated With Acute Inflammatory Demyelinating Polyneuropathy in an Older Female: A Case Report.

Rheumatoid vasculitis (RV) causes various complications in the heart, lungs, kidneys, and nerves that require intensive treatment. Rapid RV-related peripheral nerve involvement progression is critical and requires prompt treatment. We report the case of a 73-year-old female with RV, with a chief complaint of difficulty walking without any infectious symptoms for several months. We diagnosed Guillain-Barré syndrome (GBS) accompanied by RV and treated the patient with intravenous immunoglobulin and cyclophosphamide. Previous impairments of activities of daily living (ADLs) were resolved. Diagnosing the neurological manifestations of RV and GBS in older patients with an active RV is challenging because of the various patterns of the progression. For effective management, considering both diseases and implementing immunosuppressive and modulatory treatments is critical to stop the progression of neurological symptoms and prevent the deterioration of ADLs.

Treating Exudative Pleurisy Accompanied by Felty Syndrome in an Older Patient With Advanced Rheumatoid Arthritis.

Advanced rheumatoid arthritis (RA) is complicated by extra-articular manifestations such as small- and medium-sized vasculitis, pulmonary fibrosis, and pleurisy. The clinical course of the disease is refractory and critical. Treating advanced RA with multiple extra-articular manifestations is challenging. Here, we report a case of advanced RA in a 75-year-old man with exudative pleurisy and Felty syndrome. Treatment should be initiated promptly while paying attention to the possibility of infection as a differential diagnosis of exudative pleurisy because of the drastic change in the patient's condition due to disease progression. In addition, appropriate treatment is required to differentiate between Felty syndrome and malignant diseases. In older patients with RV complicated by pleurisy and Felty syndrome, starting steroids and immunosuppressive agents is crucial when conducting a thorough examination and considering the rapid progression of symptoms.

Neuromuscular disease: 2021 update.

This review highlights ten important advances in the neuromuscular disease field that were first reported in 2020. The overarching topics include (i) advances in understanding of fundamental neuromuscular biology; (ii) new / emerging diseases; (iii) advances in understanding of disease etiology and pathogenesis; (iv) diagnostic advances; and (v) therapeutic advances. Within this broad framework, the individual disease entities that are discussed in more detail include neuromuscular complications of COVID-19, supervillin-deficient myopathy, 19p13.3-linked distal myopathy, vasculitic neuropathy due to eosinophilic granulomatosis with polyangiitis, spinal muscular atrophy, idiopathic inflammatory myopathies, and transthyretin neuropathy/myopathy. In addition, the review highlights several other advances (such as the revised view of the myofibrillar architecture, new insights into molecular and cellular mechanisms of muscle regeneration, and development of new electron microscopy tools) that will likely have a significant impact on the overall neuromuscular disease field going forward.

Clinical characteristics of COVID-19 associated vasculopathic diseases.

Coronavirus disease 19 (COVID-19) has shown to be an infectious disease affecting not only of the respiratory system, but also cardiovascular system leading to different COVID-19-associated vasculopathies. Venous and arterial thromboembolic events have been frequently described among hospitalized patients with COVID-19 and inflammatory vasculopathic changes have also been observed. Several of the reported COVID-19 associated vasculopathies exhibit differences on epidemiology, clinical characteristics and outcome compared to non-COVID-19 types. This review focuses on the epidemiology, clinical, diagnostic and therapeutic characteristics as well as outcome data of COVID-19 associated thromboembolic events and inflammatory vasculopathies, elaborating similarities and differences with non-COVID-19 cohorts.

LONG-TERM AND BOOSTER VACCINATION RESPONSES IN PATIENTS WITH GIANT CELL ARTERITIS AND POLYMYALGIA RHEUMATICA

BackgroundVaccination remains essential in preventing morbidity of SARS-CoV-2 infections. We previously showed that >10mg/day prednisolone and methotrexate use were associated with reduced antibody concentrations four weeks after primary vaccination in patients with giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) [1].ObjectivesHere, we performed a follow-up study to measure the decay of antibody concentrations over time and the immunogenicity of SARS-CoV-2 booster vaccination.MethodsGCA/PMR patients included in the primary vaccination (BNT162b2 or ChAdOx1) study were asked again to donate blood samples six months after primary vaccination (n=24) and one month after booster vaccination (n=46, BNT162b2 or mRNA1273). Data were compared to that of age-, sex-, and vaccine-matched controls (n=58 and n=42, respectively).ResultsAntibody concentrations decreased faster over time in GCA/PMR patients than in controls, but this decrease was not associated with treatment during primary vaccination. Post-booster antibody concentrations were comparable between patients and controls. Antibody concentrations post booster vaccination associated strongly with antibody concentrations post primary vaccination, but not with treatment during booster vaccination. However, the fold-change of post-booster vaccination showed a slight negative correlation with the post-primary vaccine antibodies.ConclusionThese results indicate that patients with impaired vaccine responses after primary vaccination, have slightly stronger increases in humoral immunity after booster vaccination, but this is not enough to reach a similar protection. The decrease in humoral immunity, and subsequent increase after booster vaccination, is likely not impacted by prednisolone or methotrexate treatment. Rather, these treatments put the patients at an immunogenic disadvantage during primary SARS-CoV-2 vaccination, which is not fully repaired by a single booster vaccination. This longitudinal study in GCA/PMR patients stresses the importance of repeat booster vaccination for patients that used >10mg/day prednisolone or methotrexate during primary vaccination.Reference[1]van Sleen Y, van der Geest, Kornelis SM, Reitsema RD, Esen I, Terpstra JH, Raveling-Eelsing E, et al. Humoral and cellular SARS-CoV-2 vaccine responses in patients with giant cell arteritis and polymyalgia rheumatica. RMD open 2022;8(2):e002479.Figure 1.Acknowledgements:NIL.Disclosure of InterestsYannick van Sleen: None declared, Kornelis van der Geest Speakers bureau: Speaker fees from Roche, Grant/research support from: Grant support from Abbvie, Annemarie Buisman: None declared, Maria Sandovici: None declared, Debbie van Baarle: None declared, Elisabeth Brouwer: None declared.

EFFECTS OF DMARD TREATMENT ON SARS-CoV-2 mRNA VACCINATION IN PATIENTS WITH SYSTEMIC AUTOIMMUNE RHEUMATIC DISEASE: A MATCHED, PROSPECTIVE COHORT STUDY

BackgroundPatients suffering from systemic autoimmune rheumatic disease (SARD) display poor antibody development after two doses of mRNA vaccinations leaving these patients with only limited humoral protection against severe SARS-CoV-2 disease courses. Of key interest is the effect of conventional synthetic (csDMARD) and biological/ targeted drugs (b/tsDMARDs) disease modifying antirheumatic drugs on the time of protection.ObjectivesTo compare antibody titer development in patients with vasculitis and connective tissue disease (CTD) with healthy controls 6 months after two mRNA vaccinations and after third immunization. To analyze factors, that affect the velocity of titer decline, well as qualitative humoral response.MethodsPatients with SARD were enrolled and matched for gender and age with healthy control subjects (HC) and the humoral response after 6 months to two doses of mRNA vaccine BNT162b2 in terms of SARS-COV-2 antibody titer was assessed. In addition to binding antibody units (BAU) we also analyzed neutralizing antibodies. Patients receiving B-cell depleting therapy and those with prior SARS-CoV-2 infection (via detection of nucleocapsid antibodies) were excluded. Differences between two groups were calculated with Wilcoxon signed-rank test.ResultsA total of 53 patients with SARD (42 patients suffering from connective tissue disease and 11 with vasculitis respectively) and 73 HC were analysed. Interestingly only patients receiving a combination therapy of different csDMARDs/ b/tsDMARDs demonstrated diminished antibody titers 6 months after two doses of mRNA vaccine (p-value p-value<0,001), whereas patients receiving only csDMARD as monotherapy displayed comparable antibody levels to healthy controls. This effect was equalized after a third booster vaccination (p-value=0,13). Concerning disease entities, patients with vasculitis seemed to have lower BAU than HC (p-value<0,05) and patients suffering from CTD. After third vaccination both patient groups had lower antibody levels than HC (vasculitis: p-value <0,0001;CTD: p-value p-value<0,01). Lower antibody levels before third vaccination correlated with lower antibodies after third immunization.ConclusionPatients with autoimmune rheumatic diseases undergoing combination therapy may be more vulnerable to SARS-CoV-2 infection, due to reduced antibody levels 6 months following two doses of mRNA vaccine. Our data strongly recommends antibody measurements in patients receiving combination therapy and individualized earlier booster vaccination.Figure 1.Anti-SARS-Cov-2 S antibody titers. A: Antibody titers measured 6 months after two doses of mRNA vaccination in patients with connective tissue disease, vasculitis and healthy controls. B, Antibody levels according to disease entity. AB: antibody;BAU: binding antibody unit;CTD: connective tissue disease;HC: healthy control;mono: disease modifying anti-rheumatic drug monotherapy;combination: combination therapy of disease modifying anti-rheumatic drugs;RBD: receptor binding domain;[Figure omitted. See PDF]Table 1.Demographic parameters and therapy of study participants.SARD (n=53)HC (n=73)Age, mean (standard deviation)53.55 (±14.04)51.27 (±14.07)Female45 (84.9%)47 (64.4%)Connective tissue disease42 (79%)Vasculitis11 (21%)csDMARD or b/tsDMARD monotherapy22 (41%)csDMARD and/or b/tsDMARD combination therapy13 (25%)No therapy18 (34%)Methotrexate8 (15%)Mycophenolate mofetil10 (19%)Hydroxychloroquine17 (32%)Azathioprine8 (15%)Belimumab3 (6%)Tocilizumab3 (6%)Glucocorticoid dose 1. vaccination, mean (standard deviation)2.8 (±10.8)Glucocorticoid dose 2. vaccination, mean (standard deviation)2.6 (±10.7)SARD: Systemic autoimmune rheumatic disease, HC: Healthy controls, csDMARD: conventional synthetic disease modifying antirheumatic drugs and b/tsDMARD: biological/ targeted drugs disease modifying antirheumatic drugsREFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsElisabeth Simader Speakers bureau: Lilly, Thomas Deimel: None declared, Felix Kartnig: None declared, Selma Tobudic: None declared, Helmuth Hasla her Grant/research support from: Glock Health, BlueSky Immunotherapies and Neutrolis, Thomas Maria Karonitsch: None declared, Daniel Mrak: None declared, Thomas Nothnagl: None declared, Thomas Perkmann: None declared, Helga Lechner-Radner: None declared, Judith Sautner: None declared, Florian Winkler: None declared, Heinz Burgmann Speakers bureau: speaker fees from Shionogi, Pfizer, MSD, Paid instructor for: advisory boards for Valneva, MSD, Gilead, Consultant of: consulting fees from MSD, Pfizer, Takeda, Gilead, Daniel Aletaha Speakers bureau: other from Abbvie, Amgen, Lilly, Merck, Novartis, Pfizer, Roche, Sandoz, Grant/research support from: grants from Abbvie, Amgen, Lilly, Novartis, Roche, SoBi, Sanofi, Stefan Winkler: None declared, Stephan Blüml Speakers bureau: personal fees from Abbvie, personal fees from Novartis, Peter Mandl Speakers bureau: reports speaker fees from AbbVie, Janssen and Novartis, Grant/research support from: research grants from AbbVie, BMS, Novartis, Janssen, MSD and UCB.

A Case with the Left Common Carotid Artery Involvement as Large Vessel Vasculitis after COVID-19

It is known that there are many other organ involvements in the coronavirus disease of 2019 (COVID-19) apart from respiratory tract involvement, and the disease may occur in different clinical spectrums in different patients. One of the most important structures affected by the virus is the vascular structure. It creates a tendency to thrombosis, especially by causing damage to the vascular endothelium and activating the coagulation system and may lead to important complications. It is known that the virus, which especially affects venous and small-sized arterial vessels, also affects medium-sized vessels. However, large-sized arterial vascular involvement is very rare. In this case report, it was aimed to present the left common carotid artery vasculitis, together with radiological appearence and clinical features, in a patient who presented with the complaint of left anterior neck pain in 4 weeks after COVID-19 infection. © Copyright 2023 by Anatolian Journal of Family Medicine.

CLINICAL SIGNIFICANCE OF SERUM FERRITIN IN PATIENTS WITH SYSTEMIC SCLEROSIS

BackgroundThe main systemic sclerosis (SSc) manifestations are skin thickening, microangiopathy and ischemic changes in tissues, fibrotic damage to the lungs, heart, kidneys, and digestive system, arthritis, and myopathy. Acute phase reactants (APR) like erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) reflect inflammation activity in various inflammatory conditions. Ferritin is a protein bound to iron;low serum ferritin indicates iron deficiency and/or anemia. Instead, high ferritin levels are associated with inflammatory and non-inflammatory conditions such as dermatomyositis, pulmonary fibrosis, lupus, systemic COVID-19, vasculitis, tissue damage, thromboembolic complications, and metastatic cancer. The possible role of ferritin in SSc as APR is unclear.ObjectivesWe aimed to assess whether ferritin levels can reflect the severity of SSc and predict the outcome.Methods241 files of SSc patients with information on serum ferritin level (ferritin over 300 mg/dL is considered elevated) who visited the Rambam Rheumatology Institute in the years 2004-2021were used for retrospective analysis. Patients' demographic, clinical, laboratory, imaging, and respiratory function data were collected from electronic hospital files. Statistics included Student's T-test, Pearson's chi-squared test, and Kaplan-Meier curve;statistical significance was determined as p<0.05.Results36 patients (FerEl-SSc) had elevated ferritin values;the rest (n=205) represented the second group (FerNor-SSc). Significant differences were seen in gender (male 44.4% - 15.6%), disease duration (4.56 - 7.7 years), modified Rodnan skin score (12.3 - 6.9), as well as in incidence of lung (65.7% - 38.7%), heart (51.4% - 21.1%), and renal (28.6% - 5.9%) involvement. Increased ferritin correlated with elevated ESR, CRP, creatinine, creatine kinase, troponin, and reduced hemoglobin, impaired pulmonary function tests and reduced left ventricular ejection fraction on echocardiography. Patients with elevated ferritin had a significant increase in mortality rates (52.8% and 35.1%) and non-significant reduction in survival.ConclusionOur study demonstrated that ferritin has a potential as a sensitive marker for SSc severity in term of skin thickening, vital organ complications, and mortality. The ferritin test is simple and inexpensive, it can add to the complex SSc assessment and contribute to treatment decision-making in complicated SSc.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

Postcovid distant complications in periodontal tissues in individuals after inpatient treatment in the red zone

There are no data in the literature on long-term complications in periodontal tissues due to a coronavirus infection. The aim is to study postcovid complications arising in periodontal tissues in patients with general somatic pathology. Materials and methods. At the first stage, 296 patients were examined who were treated in hospitals for COVID-19 and 128 people — the comparison group — without concomitant pathology, who were divided into 6 age groups. The examination included measuring the microcirculation index (MI) and determining the degree of oxygenation in periodontal tissues according to Laser Doppler Flowmetry (LDF) data. At the second stage, the same indicators were determined in 74 patients with complications after COVID-19 in the form of periodontitis, and an index evaluation of periodontal tissues was also performed: periodontal index (PI). The severity of periodontal pathology and the period of its occurrence were assessed depending on the age of patients, the effect of postcovid vasculitis on the state of the microcirculatory bed and the nature of the course of pathological processes in the periodontal. Dopplerograms revealed predictors of the development of severe periodontal pathology. Results. The integral microcirculation index and the level of oxygenation in the periodontium and on the phalanx of the finger in patients with COVID-19 undergoing inpatient treatment were statistically significantly lower than in patients of control groups. Against the background of improved microcirculation and oxygenation of periodontal tissues by 3 months after coronavirus infection, there is a decrease in PMA and PI indices. By the 6th and even more than the 9th month, the examined patients showed a drop in the indicators of regional blood flow and the level of tissue saturation against the background of an increase in the indices of PMA and PI. Conclusions. The greatest number of pathological processes in periodontal tissues occurred in the period from 6 to 9 months, the most pronounced in the age group of 65—80 years — 43.1%, 55—64 years — 41.3%, 45—54 years — in 31.3% of patients. In patients with severe hemorrhagic vasculitis (1.1%), the most pronounced changes in periodontal tissues were observed. In this category of patients, 3 months after the infection, there was a sharp development of rapidly progressive periodontopathy, and by the 9th month there was a complete loss of teeth. The severe form of hemorrhagic vasculitis was characterized by severe microcirculation disorders in periodontal tissues (PM=16.1±0.9). The indicators of microcirculation (PM) below 17.1 and hemoglobin oxygen saturation (SO2) below 77.9 are predictors of the development of inflammatory changes in periodontal tissues. © 2023 Clinical Dentistry LLC. All rights reserved.

Multisystem Inflammatory Syndrome in Children: Characteristics and Epidemiology

Shortly after the novel coronavirus (now known as SARSCoV- 2) was recognized, data began to accumulate on the virus's effects on children. Initial data showed that children were more likely to be mildly affected, compared to adults, with lower risks of hospitalization and death. However, in April of 2020, reports appeared of a severe disease in children occurring about two-six weeks after infection with SARS-CoV-2. The features were similar to those seen in a rare vasculitis condition called Kawasaki disease. On May 14, 2020, the Centers for Disease Control and Prevention (CDC) issued a national health advisory regarding this new condition, which was called multisystem inflammatory syndrome in children (MIS-C). The current case definition for MIS-C includes six criteria: (1) serious illness leading to hospitalization or resulting in death;(2) age less than 21 years;(3) measured fever over 38 degrees Celsius or report of subjective fever;(4) laboratory evidence of inflammation;(5) new onset involvement in at least two of the following (cardiac involvement, mucocutaneous involvement, shock, gastrointestinal involvement, and hematologic involvement);and (6) laboratory-confirmed SARS-CoV-2 infection or an epidemiologic link to a person with COVID-19. According to CDC, as of January 3, 2023, there have been 9,333 patients in the United States meeting the case definition of MIS-C, with 76 deaths. The median age of patients was nine years, with half of those affected between the ages of five and 13 years. More than half of the reported patients on whom race-ethnicity information was available were in children who are Hispanic/Latino or Black, non-Hispanic. Over 60% of reported patients were male. Most affected children had previously been healthy. A better understanding of the pathogenesis of this serious illness is needed to provide better treatment options for children with MIS-C. Prevention of MIS-C is focused on the prevention of SARS-CoV-2 infection through staying up to date with COVID-19 vaccination, masking, and other prevention strategies.

REFERRAL BEHAVIOUR OF PRIMARY CARE DOCTORS TO RHEUMATOLOGY CLINIC IN MALAYSIA

BackgroundThe workload at rheumatology clinics have been growing relentlessly and an audit on new.referrals helps to identify referral behaviour of primary care doctors and improvement can be done by providing further training.ObjectivesTo audit on new referral cases to rheumatology clinic from 2020-2022 and to identify new cases with misdiagnosis for future training purpose.MethodsThis was a retrospective study. The medical records of all new referral to rheumatology clinic Hospital Sultan Ismail and Hospital Pakar Sultanah Fatimah from 1st January 2020 to 31th November 2022 were reviewed. The referral diagnosis and final diagnosis were identified and analysed.ResultsThere were total of 927 new cases referral throughout the 35 months during Covid-19pandemic. Majority of them were diagnosed to have rheumatoid arthritis (217/927)followed by systemic lupus erythematosus (190/927), psoriatic arthritis (147/927),gout (62/927), osteoarthritis (58/927), systemic sclerosis (25/927), ankylosing spondylitis (25/927), soft tissue rheumatism (24/927), Sjogren syndrome (24/927),mixed connective tissue disease (14/927), vasculitis (11/927), fibromyalgia (10/927),polymyositis (7/927) and miscellaneous (39/927).45 out of the new cases were diagnosed as unlikely rheumatic diseases. There were 29pending cases awaiting final diagnosis.212 of the referrals were identified as misdiagnosis with the highest as nodal osteoarthritis.(55/212) followed by unlikely rheumatic disease (43/212), soft tissue rheumatism (24/212),psoriatic arthritis (20/212), Sjogren syndrome (14/212), gout (8/212), rheumatoid arthritis (7/212), fibromyalgia (6/212), systemic lupus erythematosus (5/212), ankylosing spondylitis (4/212), mixed connective tissue disease (3/212), systemic sclerosis (2/212), polymyositis (2/212) and others (19/212): diffuse idiopathic skeletal hyperostosis, hypermobility syndrome, RS3PE syndrome, idiopathic uveitis, graft versus host disease, juvenile idiopathic arthritis, antiphospholipid syndrome, hypothyroidism, post streptococcal arthritis, prolapsed intervertebral disc, cerebrovascular disease, traumatic sternoclavicular joint subluxation, ledderhose disease, paraspinal muscle spasm and viral myalgia).ConclusionNodal osteoarthritis and soft tissue rheumatism can be great mimicker for inflammatory.arthritis and if wrongly diagnosed will lead to unnecessary anxiety or wrong treatment. More training is needed to improve clinical skills amongst primary care doctors.ReferencesNA.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

CLINICAL COURSE IN COVID 19 DISEASE IN IMMUNOSUPRESSED PATIENTS DIAGNOSED WITH RHEUMATIC DISEASES

BackgroundPatients with pre-existing rheumatic diseases may be exacerbated during SARS-CoV-2 infection, or may develop new autoimmune features. Furthermore, immunosuppressive agents used to treat autoimmunity-inflammation as well as comorbidities can also affect the disease outcome.ObjectivesTo evaluate the outcome of rheumatic diseases after Covid 19 infection in patients diagnosed with rheumatic diseases, under various immunosuppressive treatment, as well as the effects of vaccines against Covid or antiviral treatment in this sensitive population group.MethodsDuring the pandemic, 1493 patients with autoimmune or autoinflammatory disease who were continuously followed up in two tertiaries hospitals in northern and northwestern Greece were included in the current study. The patients were compared with 769 controls after adjustment for age, sex, weight, vaccination status and comorbidities. Of the 1493 patients, 648 had rheumatoid arthritis, 282 psoriatic arthritis, 173 ankylosing spondylitis, 122 systemic lupus erythematosus, 98 Sjogren's syndrome, 43 polymyalgia rheumatica, 34 mixed connective tissue disease or overlapping syndromes, 31 vasculitis, 27 systemic sclerosis, 18 myositis, 10 Behcet syndrome, 5 primary antiphospholipid syndrome and 2 had Familial Mediterranean Fever. The vast majority of patients and controls were fully vaccinated (82%) and 397 patients received antiviral treatment, 94% of them were fully vaccinated.ResultsCovid 19 disease in vaccinated patients with rheumatic diseases was shown to perform the same or about the same as those in the control group after adjustment for risk factors for severe disease. 19 of our patients required admission in the intensive care unit (62% full vaccinated) while a total of 12 died (66% non vaccinated). Major risk factors for severe disease were previous respiratory failure, chronic renal impairment, obesity, and failure to receive antiviral therapy. It was also shown that infection with Covid led to an exacerbation or induction of autoimmune disorders in 25 of the participants.ConclusionIn this large cohort, Covid 19 disease was shown to affect patients with autoimmune rheumatic diseases the same or approximately the same way as the general population if they are fully vaccinated and if they start timely antiviral treatment where indicated. Further research and monitoring of the results after the multiple mutations of the virus is advisable.ReferencesNone.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

NEW INSIGHTS ON EPIDEMIOLOGICAL DATA AND IMPACT OF THE COVID-19 PANDEMIC ON IGA VASCULITIS IN CHILDREN AND ADULTS IN FRANCE: A NATIONWIDE COHORT

BackgroundIgA vasculitis (IgAV) is a rare autoimmune disease affecting small vessels. It is well established that the incidence is higher in children (3 to 26 per 100,000 children/year,) [1] than in adults (0.1 to 1.8 per 100,000 individuals/year) [1]. However others epidemiological data and impact of the COVID-19 on IgAV remain overlooked [2].ObjectivesTo collect and analyze epidemiological data on IgAV in both adults and children in France.MethodsWe conducted an observational study using a national database called "BNDMR” [3] (Banque Nationale de Données Maladies Rares) on IgA vasculitis (code ORPHA761), which gathered patients managed in the French rare disease expert network. The incidence was estimated from the date of diagnosis, and we calculated the median annual incidence over the period 2010-2022. We specifically assessed the north/south gradient (latitude of the residence higher/lower than the median of the latitudes), the seasonality, and the impact of the COVID-19 pandemic compared to other patients reported within the same period and addressed in the same expert centers used as controls.ResultsDuring this 12-year period, 1988 patients with IgAV were reported (1498 children;490 adults). The male to female ratio was 1.57 for adults and 1.05 for children. The median IgAV annual incidence was 15 cases/year [IQR 9-30] and 82 cases/year [IQR 72-86] for adult and children cases respectively. Time to diagnosis was less than 1 month for both. Compared with other patients reported in the same expert centers, IgAV was more frequently reported in the southern part of France than in the north (OR 4.88 [95% confidence intervals: 4.17 - 5.74] in adults and OR 1.51 [1.35 - 1.68] in children). IgAV was also more frequently observed in winter than during the rest of the year in both adults (OR 1.60 [1.39 - 1.82]) and children (OR 1.22 [1.01 - 1.48]). The incidence of IgAV decreased during the COVID-19 pandemic period (from March 2020 to September 2022) in children (OR 0.62 [0.47 - 0.81]) but not in the adult population (OR 0.90 [0.76 - 1.06]).ConclusionOur study confirms the winter seasonality and sex ratio in IgAV [4,5], but suggests that the incidence or the reporting of IgAV decreased in children during the COVID19 pandemia, possibly due to barrier measures [6]. The observed north/south gradient need confirmation. The main limitation of this study is a possible IgAV under-reporting as this study rely only on cases addressed in expert centers.References[1]Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B. IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects. Autoimmun Rev. 2015;14(7):579-585. doi:10.1016/j.autrev.2015.02.003[2]Deshayes S, Moulis G, Pillebout E, Aouba A, Audemard-Verger A. Positive predictive value of hospital discharge diagnosis code to identify immunoglobulin A vasculitis in France: A validation study. Eur J Intern Med. 2017;43:e18-e19. doi:10.1016/j.ejim.2017.05.025[3]Jannot AS, Messiaen C, Khatim A, Pichon T, Sandrin A, BNDMR infrastructure team. The ongoing French BaMaRa-BNDMR cohort: implementation and deployment of a nationwide information system on rare disease. J Am Med Inform Assoc. 2022;29(3):553-558. doi:10.1093/jamia/ocab237[4]Piram M, Maldini C, Biscardi S, et al. Incidence of IgA vasculitis in children estimated by four-source capture-recapture analysis: a population-based study. Rheumatology (Oxford). 2017;56(8):1358-1366. doi:10.1093/rheumatology/kex158[5]Gardner-Medwin JMM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. 2002;360(9341):1197-1202. doi:10.1016/S0140-6736(02)11279-7[6]Kaya Akca U, Atalay E, Cuceoglu MK, et al. Impact of the COVID-19 pandemic on the frequency of the pediatric rheumatic diseases. Rheumatol Int. 2022;42(1):51-57. doi:10.1007/s00296-021-05027-7Figure.Acknowledgements:NIL.Disclosure of InterestsNone Declared.