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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.
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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.
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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.
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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.
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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.
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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.
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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.
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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.
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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.
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BackgroundDuring the COVID-19 pandemic, asynchronous consultations were introduced for patients with vasculitis. To assess disease activity without of face-to-face clinical reviews and blood testing, patients submitted patient reported outcome measures (PROMs) via electronic survey forms, which were subsequently triaged by clinicians.Objectives1. To investigate how patients' vasculitis disease activity was affected by the COVID-19 pandemic through retrospective comparison of clinician-assessed scores recorded pre-pandemic with intra-pandemic self-reported patient reported outcome measures (PROMs) and disease scores submitted by patients remotely.2. To assess patients' clinical outcomes, including allocation of follow-up and further management/treatment escalation during this period.3. To validate self-reported BVAS scores against an existing PROM.MethodsThis is a retrospectively study of patients with a known diagnosis of vasculitis under the care of the Nuffield Orthopaedic Centre, Oxford. For the purposes of this study, we included patients with all vasculitis diagnoses.Clinician-reported scores (Bristol Vasculitis Activity score v.3, BVAS) were recorded during in-person clinics pre-pandemic (defined as 01/01/2019-31/12/2019) [1].Patients' self-reported BVAS (SR-BVAS) and AAV-PRO (ANCA-associated vasculitis patient-reported outcomes) scores were submitted by patients via electronic forms containing the requisite questionnaires sent out during-pandemic (defined as 01/12/2020-31/03/22) [2].SR-BVAS has not been validated but was collected to allow clinical comparison to disease activity scores completed by clinicians. Response were stored and analysed in a secure database. Score comparison was performed using Wilcoxon Sign Rank testing. Clinical outcome data was collected from the local Electronic Patient Record. Data analysis was performed in Microsoft Excel and R (version 4.2.1).ResultsWe noted a significantly higher overall level of patient-reported disease activity during the pandemic than was recorded in clinics prior. In the total cohort of all vasculitis patients for whom we had data, the median BVAS increased from 2 pre-pandemic (N = 335, range 0-21) to 6 intra-pandemic (N = 143, range 0-42) (p <0.001). The overall proportion of patients with severe/active disease (defined as BVAS ≥4) increased from 27% to 36% during the pandemic period.In a smaller cohort of 64 patients for whom we had paired pre- and during-pandemic scores, increased disease activity was reported (p<0.01). Notably, the number with a BVAS consistent with severe disease increased from 7 (11%) to 19 (30%).There was a significant positive correlation between SR-BVAS and AAV-PRO (r=0.61, p< 0.001) submitted by patients during-pandemic;however, at low BVAS (≤3), the AAV-PRO ranged widely (28-87)Follow-up data was available for all 64 patients in this cohort: 8/19 (42%) with a during-pandemic SR-BVAS ≥4 were seen in clinic within 3 months (telemedicine or face-to-face).ConclusionPatients reported worsening of vasculitis disease activity during the COVID-19 pandemic. This may be attributable to impacts on well-being or access to healthcare services. We note that disease activity scores in vasculitis may be limited in their ability to capture the whole picture disease activity in the absence of clinical assessment [3]. 42% of patients with self-reported high disease activity were seen within 3 months. There was a significant positive correlation between AAV-PRO and SR-BVAS, suggesting it has some use as a PROM.References[1]Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, et al.. Ann Rheum Dis. 2009 Dec;68(12):1827–32.[2]Malley T, Jackman J, Manderson S, Saldana Pena L, Evans E, Barrett J, et al. Ann Rheum Dis. 2021 Jun 1;80(Suppl 1):289.[3]Luqmani RA. Nephrology Dialysis Transplantation. 2015 Apr 1;30(suppl_1):i76–82.Acknowledgements:NIL.Disclosure of InterestsNone Declared.
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BackgroundCOVID-19 has shaped the world over the last 3 years. Although the risk for severe COVID-19 progression in children is low it might be aggravated by chronic rheumatic disease or treatment with immunosuppressive drugs.ObjectivesWe analyzed clinical data of COVID-19 cases among paediatric patients with rheumatic diseases reported to BIKER between March 2020 and December 2022.MethodsThe main task of the German BIKER (Biologics in Pediatric Rheumatology) registry is safety monitoring of biologic therapies in JIA. After the onset of the COVID-19 pandemic, the survey was expanded with a standardized form to proactively interview all participating centers about occurrence, presentation and outcome of SARS-CoV-2 infections in children with rheumatic diseases.ResultsA total of 68 centres participated in the survey. Clinical data from 928 COVID infections in 885 patients with rheumatic diseases could be analyzed. JIA was the most common diagnosis with (717 infections), followed by genetic autoinflammation (103 infections), systemic autoimmune diseases (78 infections), idiopathic uveitis (n=25), vasculitis (n=5).In 374 reported COVID infections (40%), patients were receiving conventional DMARDs, in 331 (36%) biologics, mainly TNF inhibitors (TNFi, n=241 (26%)). In 567 reports (61%) patients used either a biologic or a DMARD, in 339 reports patients (37%) did not use any antirheumatic medication including steroid.Over the last 3 years, COVID-19 occurred in Germany in 5 distinguishable waves, calendar weeks (CW) 10-30 in 2020, CW 21/2020 – 8/2021(both predominantly wild-type variant), CW 9-27 in 2021 (Alpha variant in the majority of infections), CW 28-51 in 2021 (Delta variant), since CW 52/2021 (several Omikron variants;Robert-Koch Institute: VOC_VOI_Tabelle.xlsx;live.com))In our cohort, patients with SARS-CoV-2 infection were slightly older during the 1st and 2nd wave (mean age 12.7+/-3.5 and 12.8+/-4.3 years) compared to the 4th and 5th wave with 11.4+/-3.9 and 11.4+/-4.2 years;p=0.01.160 asymptomatic SARS-CoV-2 infections were reported, frequencies of symptoms associated with COVID-19 are shown in table 1.Five patients were hospitalized for 4-7 days. A 3½-year-old female patient succumbed during the first wave with encephalopathy and respiratory failure. The patient had been treated with MTX and steroids for systemic JIA. Genetic testing revealed a congenital immunodeficiency. No other patient needed ventilation or intensive care. One case of uncomplicated PIMS in an MTX treated JIA patient was reported.The duration of SARS-CoV-2 infection-associated symptoms was markably shorter during the 5th wave with 6.7+/-5.1 days, compared with reports from the other 4 waves (Table1).The duration of symptoms was higher in MTX treated patients (10.2+/-8.4 days) compared to patients without treatment (7.7+/-10.8;p=0.004) or patients treated with TNFi (8.2+/-4.8, p=0.002). Although patients treated with steroids also had a longer duration of symptoms (9.7+/-7.0), this was not significant.ConclusionExcept for one patient with congenital immunodeficiency who died, no case of severe COVID-19 was reported in our cohort. At the time of infection, over 60% of patients had been treated with conventional DMARDs and/or biologics. Although MTX treated patients had a slightly longer duration of symptoms, antirheumatic treatment did not appear to have a negative impact on severity or outcome of SARS-CoV-2 infection.Table 1.Characteristics and frequency of symptoms in SARS-CoV-2 infectionsN or mean (SD)1st wave N=202nd wave N=843rd wave N=384th wave N=1245th wave N=662female14532775432age at COVID-19, years12.7 (3.5)12.8 (4.3)11.8 (3.5)11.4 (3.9)11.4 (4.2)asymptomatic126132694duration of symptoms;days,11.9 (14.7)9.2 (7.0)14.1 (11.6)10.3 (7.6)6.7 (5.1)fever1218541306cough1015652245rhinitis5261344289headache4161227171sore throat61139132musculosceletal pain2751348loss of smell/taste71162113fatigue4882680dizziness122116gastrointestinal symptoms151864dyspnea1117pneumonia11bronchitis1REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Inter stsAriane Klein Speakers bureau: Novartis, Toni Hospach Speakers bureau: Speaking fee Novartis and SOBI., Frank Dressler Speakers bureau: Abbvie, Novartis, Pfizer, Advisory Boards Novartis and Mylan, Daniel Windschall Grant/research support from: research funds by Novartis, Roche, Pfizer, Abbvie, Markus Hufnagel: None declared, Wolfgang Emminger: None declared, Sonja Mrusek: None declared, Peggy Ruehmer: None declared, Alexander Kühn: None declared, Philipp Bismarck: None declared, Maria Haller: None declared, Gerd Horneff Speakers bureau: Pfizer, Roche, MSD, Sobi, GSK, Sanofi, AbbVie, Chugai, Bayer, Novartis, Grant/research support from: Pfizer, Roche, MSD, AbbVie, Chugai, Novartis.
ABSTRACT
BackgroundPatients with immune-mediated rheumatic diseases (IRD) have poorer outcomes of SARS-CoV-2 infection compared to the general population.ObjectivesTo assess and compare clinical course, severity and complications of SARS-CoV-2 infection in patients with rheumatic immune-mediated inflammatory diseases (IMIDs) from Mexico and Argentina.MethodsData from both national registries, CMR-COVID (Mexico) and SAR-COVID (Argentina), were combined. Briefly, adult IRD patients with SARS-CoV-2 infection were recruited between 08.2020 and 09.2022 in SAR-COVID and between 04.2020 and 06.2022 in CMR-COVID. Sociodemographic data, comorbidities, and DMARDs were recorded, as well as clinical characteristics, complications, and treatment for SARS-CoV-2 infection. Descriptive analysis. Chi square, Fisher, Student T, Mann Whitney U tests and multiple logistic regression analyses were performed.ResultsA total of 3709 patients were included, 1167 (31.5%) from the CMR-COVID registry and 2542 (68.5%) from the SAR-COVID registry. The majority (82.3%) were women, with a mean age of 50.4 years (SD 14.4). The most frequent IRD were rheumatoid arthritis (47.5%) and systemic lupus erythematosus (18.9%). Mexican patients were significantly older, had a higher female predominance and had higher prevalence of rheumatoid arthritis, antiphospholipid syndrome, and axial spondyloarthritis, while the Argentine patients had more frequently psoriatic arthritis and ANCA-associated vasculitis. In both cohorts, approximately 80% were in remission or low disease activity at the time of infection. Mexicans took glucocorticoids (43% vs 37%, p<0.001) and rituximab (6% vs 3%, p<0.001) more frequently. They also reported more comorbidities (48% vs 43%, p=0.012).More than 90% of patients presented symptoms related to SARS-CoV-2 infection. The frequency of hospitalization was comparable between the groups (23.4%), however, the Mexicans had more severe disease (Figure 1) and a higher mortality rate (9.4% vs 4.0%, p<0.0001). After adjusting for risk factors, Mexicans were more likely to die due to COVID-19 (OR 2.2, 95%CI 1.5-3.1).ConclusionIn this cohort of patients with IRD from Mexico and Argentina with SARS-CoV-2 infection, the majority presented symptoms, a quarter were hospitalized and 6% died due to COVID-19. Mexicans presented more severe disease, and after considering risk factors they were two times more likely to die.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsCarolina Ayelen Isnardi Grant/research support from: SAR-COVID is a multi- sponsor registry, where Pfizer, Abbvie, and Elea Phoenix provided unrestricted grants. None of them participated or infuenced the development of the project, data collection, analysis, interpretation, or writing the report. They do not have access to the information collected in the database, Deshire Alpizar-Rodriguez: None declared, Marco Ulises Martínez-Martínez: None declared, Rosana Quintana: None declared, Ingrid Eleonora Petkovic: None declared, Sofia Ornella: None declared, Vanessa Viviana Castro Coello: None declared, Edson Velozo: None declared, David Zelaya: None declared, María Severina: None declared, Adriana Karina Cogo: None declared, Romina Nieto: None declared, Dora Aida Pereira: None declared, Iris Jazmin Colunga-Pedraza: None declared, Fedra Irazoque-Palazuelos: None declared, GRETA CRISTINA REYES CORDERO: None declared, Tatiana Sofía Rodriguez-Reyne: None declared, JOSE ANTONIO VELOZ ARANDA: None declared, Cassandra Michele Skinner Taylor: None declared, INGRID MARIBEL JUAREZ MORA: None declared, Beatriz Elena Zazueta Montiel: None declared, Atzintli Martínez: None declared, Cesar Francisco Pacheco Tena: None declared, Guillermo Pons-Estel: None declared.
ABSTRACT
BackgroundPatients with autoimmune rheumatic diseases (ARDs) under moderate/severe immunosuppression are considered a high-risk population to develop severe Covid-19 infection.ObjectivesThe aim of our study was to describe the clinical characteristics and the outcome of patients with ARD who contracted a Sars-Cov-2 infection.MethodsAmong patients with ARD being followed in our tertiary outpatient rheumatology clinic, we retrospectively identified those infected with SARS-CoV-2 between the beginning of the pandemic and August 2022. Patients' medical files were reviewed for demographics (age, gender and comorbidities) and disease-related characteristics, as well as coronavirus disease (COVID-19) characteristics, including vaccination status, treatment, and outcomes (covid-19 severity, hospitalization, death).ResultsA total of 209 cases of ARD patients with confirmed Covid-19 infection were recorded. Most of them were women (62.7%), with a mean age of 52.4± 13.8 years. The most prevalent ARDs were seronegative spondyloarthropathies (28.7%), systematic lupus erythematosus (21.5%), rheumatoid arthritis (16.5%), and systemic sclerosis (11.5%). More than half of the patients received corticosteroids (57.8%), while the most frequently used immunosuppressants were hydroxychloroquine (30.9%), TNF inhibitors (26.5%), mycophenolate mofetil (24.0%), methotrexate (19.1%) and rituximab (15.2%). One hundred and fifty-eight (76%) patients were either on remission or had mild disease activity. Most of the patients (131/209) had at least one comorbidity, more commonly arterial hypertension (48.5%) and pulmonary disease (45.2%). Most of the patients were vaccinated against Sars-Cov-2 (73.7%), either with two doses (38.0%), three doses (57.0%) or four doses (5.0%) of mRNA-based vaccines. The big majority of the patients (83.3%) were asymptomatic or had mild Covid-19 disease. About half of the patients (53.1%) reported to have received Covid-19 treatment. Thirty-two of them (15.3%) needed hospitalization, and five death cases were reported overall. Among the demographic characteristics, age (p<0.0001 for hospitalization) and comorbidities were associated with worse covid-19 outcomes. In particular, cardiovascular disease (OR 5.37, p=0.001 for covid-19 severity, OR 6.89, p=0.001 for hospitalization), pulmonary disease (OR 3.02, p=0.006 for hospitalization), and obesity (OR 3.46, p=0.044 for hospitalization) had the stronger associations. Non-vaccination status was also associated with a higher risk for hospitalization (OR 2.68, p=0.015). In relation to ARD-related factors, treatment with rituximab (OR 4.11, p=0.002 for hospitalization), systemic sclerosis diagnosis (OR 3.45, p=0.03 for Covid-19 severity) and myositis diagnosis (OR 4.91, p=0.033 for hospitalization) were associated with worse Covid-19 outcomes. On the other hand, spondyloarthropathies appear to be negatively associated with Covid-19 severity (OR=0.27, p=0.035).ConclusionAccording to our study, most ARD patients recovered uneventfully from Covid-19. However, there are several indications that we should be vigilant for patients who remain unvaccinated, are older, have a systemic sclerosis or myositis diagnosis, and/or receive intense immunosuppressive regiments such as rituximab.References[1]Papagoras C, Fragoulis GE, et al. Better outcomes of COVID-19 in vaccinated compared to unvaccinated patients with systemic rheumatic diseases. Ann Rheum Dis. 2021 Nov 10.[2]Strangfeld A, Schäfer M, et al. Factors associated with COVID-19-related death in people with rheumatic diseases: results from the COVID-19 Global Rheumatology Alliance physician-reported registry. Ann Rheum Dis. 2021 Jul;80(7):930-942.Table 1.N=209ARD Diagnosisn (%)Rheumatoid arthritis34 (16.3)Seronegative spondyloarthropathies60 (28.7)Systemic lupus erythematosus45 (21.5)Systemic sclerosis24 (11.5)Sjogren's syndrome15 (7.2)Vasculitis19 (9.1)Myositis9 (4.3)Other3 (1.4)Acknowledgements:NIL.Disclosure of InterestsNone Declared.
ABSTRACT
Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells. The prognosis varies depending on the form of the disease and organ damage. Any organs and systems can be involved in the pathological process in various combinations. A poor response to standard therapy and an unfavorable prognosis are characteristic of patients with a multisystem form of LCH and involvement of organs at risk. Skin lesions are a classic sign of LCH. Purpose - to describe the complexity and duration of diagnosis of LCH with multisystem damage in a boy aged 2 years and 2 months, infected with poliomyelitis and coronavirus. Clinical case. The first clinical manifestations of LCH in the child debuted with an eczematous-seborrheic rash on the scalp with spread to the limbs and trunk. The child was treated for toxicoderma, hemorrhagic vasculitis at the place of residence for 6 months. The boy lost 1.5 kg of body weight in 1 month. At the time of hospitalization, seborrheic-eczematous rashes on the skin with a hemorrhagic component, trophic-inflammatory changes in the nails of the hands, signs of protein-energy deficiency, stomatitis, gingivitis, hepatosplenomegaly, polyserositis, diabetes insipidus, osteolytic foci of the frontal bones were found. Results of the tests: anemia, thrombocytopenia, hypoproteinemia and hypoalbuminemia, coagulation disorders. The patient had the onset of lower flaccid paraparesis, muscle hypotonia. The boy was diagnosed with a number of infectious complications, including poliomyelitis (a derivative of vaccine poliovirus type 2), COVID-19. The child received LCH-III cytostatic therapy with a positive effect. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies.Copyright © 2023 Institute of Physics of the Russian Academy of Sciences. All rights reserved.
ABSTRACT
BackgroundGCA is a critically ischemic large vessel vasculitis, varying in extent, severity and outcomes, hence requires disease stratification using clinical, laboratory and imaging parameters, for targeted management. Although DMARDs are used, the effectiveness in real life, such adjuvants remain un-elucidated. We performed a prospective, multi centre cohort study of new GCA stratified into remitting, relapsing, refractory, ischemic disease.ObjectivesWe assessed prognostic factors and compared critical outcomes such as remission with glucocorticoid (GC) monotherapy versus GC plus DMARDs in the first 12 months.MethodsHAS GCA study (1) recruited consecutive patients with new onset GCA from 7 centres (UK, Italy, Spain, Netherlands). diagnosis was confirmed used a modified GiACTA criteria at 6 months follow up. All underwent ultrasound (bilateral common, parietal, frontal temporal arteries, and axillary arteries) using accepted standard cut-off values [2]. GCA patients had US at baseline,1,3,6,12 months and halo count (HC) and Halo score (Temporal TAHS, axillary AAHS, total THS) assessed [3]. The primary outcome- remission at 12 months (absence of signs/symptoms, CRP<5 mg/dl, prednisolone < 5 mg daily). Results are reported as descriptive statistics.Results229 participants included in the study (GCA- 84 (36.68 %) (Figure 1). Study recruited during Covid pandemic,73 completed,11 lost to follow-up (died -7, withdrawn-4). The deceased/withdrawn patients (compared to completers) were older (80 v74 yrs, p=0.018), preponderantly male (73% v 36%, p=0.043) with visual symptoms (91% v 49%, p=0.010) partial/total sight loss (55% v 21%, p=0.024), lower CRP (21 v 68, p=0.061) and ESR (42 v 62, p= 0.317).Of 73 completers 36 required early DMARDs (<12 weeks) for refractory/relapsing/ischemic/GC related AEs. This group had more LV involvement (50% v 11%, p=0.0003), Remission attained at 12 months 32/36 (89%) in DMARD group was comparable to the remitting GC monotherapy group 33/37 (89%) with comparable cumulative GC doses (Figure 1, Table 1).At 12-months follow up, median TAHS, AAHS and THS reduced from 13 to 3, 12 to 9 and 21.5 to 12, respectively.ConclusionOur study suggests, elderly males with visual symptoms, sight loss, lower CRP are a high-risk group with increased mortality within GCA. Difficult to treat disease is seen in half of all patients especially with LV involvement. This group responds well to early DMARD use achieving remission comparable to the remitting group at 12 months. Current therapies fail to achieve remission in 9.5 % of cases. HS and HC show significant improvement mirroring clinical outcomes during first 12 months of therapy.References[1]Sebastian A et al. BMC Rheum. 2020[2]Schafer VS et al. Rheumatology 2017[3]van der Geest KSM et al. ARD 2020Table 1.comparison between the DMARD-used group and only GC group in all the GCA completed the 12 months follow upPatients' characteristicsGCA with completed follow-up (n=73)GCA treated with DMARD=36GCA not treated with DMARD=37Age, median (range) years73.5 (60-89)76 (60-89)Sex, Females, n (%)23 (64)24 (65)US halo score (HS)/IMT median (range)Temporal artery HS11 (0-23)13 (1-22)Axillary artery HS12 (0-21)12 (0-18)Axillary artery IMT (mm)0.77 (0.33-2.6)0.82 (0.39-1.21)Total HS22.5 (2-41)21 (5-40)Clinical features, n (%)Temporal headache25(69)30 (81)Scalp tenderness17 (47)19 (51)Jaw & Tongue claudication22 (61)24 (65)Polymyalgic symptoms21 (58)13 (35)Constitutional symptoms21 (58)18 (49)Any visual disturbance15 (42)21 (57)Partial or complete vision loss8 (22)7 (19)History of PMR6 (17)3 (8)Exam findings, n (%)Temporal artery abnormality24 (67)30 (81)AION/ CRAO8 (22)6 (16)Ocular nerve palsy1 (3)3 (8)Lab markers at baseline, median (range)CRP mg/dL,72.2 (6.4-292)59 (6-206)ESR mm/hr67 (9-130)57 (2-120)GC treatment, median (range)GC starting dose, (baseline)45 (0-60)50 (0-60)GC dose at 12m,5 (0-25)2.5 (0-10)Cumulative GC dose at 12m4627.5 (2600-10260.5)4622.5 (944-10737.5)Remission with prednisolone dose ≤5 mg at 12m, n (%)32 (89)33 (89)Acknowledgements:NIL.Disclosure of InterestsBhaskar Dasgupta Consultant of: Roche, Chugai, Sanofi, Grant/research support from: Roche, Sanofi, AbbVie, and GlaxoSmithKline, Kornelis van der Geest Speakers bureau: Roche, Grant/research support from: AbbVie, Alessandro Tomelleri: None declared, Pierluigi Macchioni: None declared, Giulia Klinowski: None declared, Carlo Salvarani: None declared, Abdul Kayani: None declared, Mohammad Tariq: None declared, Diana Prieto-Peña: None declared, Edoardo Conticini: None declared, Muhammad Khurshid: None declared, Sue Inness: None declared, Jo Jackson: None declared, Alwin Sebastian: None declared.
ABSTRACT
BackgroundVaccination against SARS-CoV2 had a critical role in the fight against COVID19 pandemic.A weaker humoral response to COVID19 vaccine has been found in rheumatic patients treated with Rituximab (RTX) or Mycophenolate Mofetil (MMF)[1]. Despite the evidence that anti-SARS-CoV-2 mRNA vaccines can elicit a T-cell response [2], some data show that even the cellular immunity could be impaired in rheumatic patients [3] but COVID19 serology is the only parameter that is feasible to measure in daily practice.Tixagevimab+cilgavimab are two human-derived monoclonal antibodies administered parenterally and authorized by regulatory agencies in February 2022 for pre-exposure prophylaxis (PrEP) against COVID19 from different virus variants in fragile patients.ObjectivesTo demonstrate safety and effectiveness of tixagevimab+cilgavimab.MethodsPatients with autoimmune rheumatic diseases undergoing immunosuppressive treatment with RTX or MMF during the vaccination campaign were enrolled between April and June 2022. All patients must have anti-spike antibody levels below the protective threshold (defined by anti-spike IgG titre <250 BAU/ml) after receiving at least 2 vaccine doses.Patients were monitored with a questionnaire every month about COVID19 symptoms (including respiratory and gastrointestinal symptoms, anosmia and ageusia, skin rash and potential contact with COVID19+ subjects) and were checked for anti-spike and anti-nucleocapside antibodies titres every 2 months for a total of a 6 month follow-up. MMF dose was reduced at 1 g/die at the time of vaccine administration.ResultsFifteen patients were enrolled: 9 participants had a connective tissue disease (CTD;1 dermatomyositis, 3 anti-syntethase syndrome, 4 systemic sclerosis, 1 systemic lupus erythematosus) and 6 had vasculitis (all granulomatosis with polyangiitis). 12 of them received RTX in the preceding 12 months and 3 were taking MMF.About safety, the therapy was very well tolerated and only 4 patients (26%) reported a non-severe adverse event in the 2 weeks following drug administration (2 myalgia, 1 headache, 1 fatigue), none of them requiring hospitalization nor pharmacologic treatment.Regarding effectiveness, 3/15 patients contracted SARS-Cov2 infection (20%) with mild symptoms and no need for hospitalization nor oxygen therapy. Only 1 of them received an antiviral drug (nirmatrelvir+ritonavir). All infected patients had a CTD diagnosis. No significant correlation was observed between the type of rheumatic disease and the risk of infection or response to tixagevimab+cilgavimab.ConclusionNone of our patients developed severe adverse events after tixagevimab+cilgavimab administration and, among the 3 SARS-CoV2 infected patients, none required hospitalization nor oxygen therapy.We conclude that in our experience tixagevimab+cilgavimab is a safe and useful complementary immunization strategy to vaccination for COVID19 prophylaxis.These data will be implemented in a larger study, comprehending various immunocompromised patients from several departments.References[1] Furer et al., Ann Rheum Dis, 2021[2] Mangalakumari et al., Nat Rev Immunol, 2020[3] Picchianti-Diamanti et al., Front Immunol, 2021Charateristic of the cohortIdentificativeAgeDiagnosisRTX/MMFSARS-CoV2 InfectionHospitalizationAntiviral drugs180SScMMF-//270ASSDRTX+nono370ASSDRTX+noyes452GPARTX-//551GPARTX-//649SSc+SSjRTX-//768SScMMF-//847LESRTX-//964ASSDRTX-//1068GPARTX-//1123GPARTX-//1258DMRTX+nono1361SScMMF-//1475GPARTX-//1569GPARTX-//Figure 1.Acknowledgements:NIL.Disclosure of InterestsNone Declared.
ABSTRACT
BackgroundAccuracy of diagnosis and prompt therapeutic intervention are the mainstay in patients with ANCA-associated vasculitis(AAV) suffering from life-threatening complications [1].However, there is no definition of therapeutic window in vital AAV, nor its impact on patient outcome regarding length of hospital stay, intensive care unit(ICU) admission or survival.ObjectivesThe aim of the study is to analyze the process of care from the perspective of time management in vital organ involvement AAV patients and to identify potential risk factors for ICU admission.MethodsA retrospective multicenter study identified AAV patients with life-threatening organ involvement, defined as alveolar hemorrhage, rapidly progressive renal failure, myocarditis and cerebral granuloma. Demographic data was collected. Key time frames were recorded, namely the interval from acute symptom onset to hospital presentation, days until imaging(plain X-ray, cardiac ultrasound, CT-scan), time to therapeutic intervention with corticosteroids or biologic/non-biologic immunosuppression(cyclophosphamide or rituximab) and to renal replacement therapy(RRT) or plasmapheresis. Time to ICU admission, hospital length-of-stay, Birmingham Vasculitis Activity Score(BVAS) were also noted. Statistical analysis was performed using SPSS and Chi-square and Pearson correlation tests were applied.Results66 patients with AAV were enrolled, out of which 17 fulfilled inclusion criteria. Mean age in the study group was 58.6±11.1 years old,10 patients(58.8%) were females and 7 (41.2%) males.11(64.7%) patients were c-ANCA positive, while 6 (35.3%) had p-ANCA and all were diagnosed with AAV prior to life-threatening event. Two patients had COVID-19 triggered AAV.In the study group, the most frequent critical organ suffering was rapidly progressive renal failure(12), followed by alveolar hemorrhages(10), 2 cerebral granulomas and one acute myocarditis. Three patients(17.6%) had more than one vital manifestation. Ten patients(58.8%) had more than three additional non-organ-threatening manifestations. Mean interval from AAV diagnosis to emergency admission was 30.1± 61.1 days, median 3 and from severe episode onset to hospitalization 1.65±0.18 days, median 1. There was only one death in the study group. Three patients were admitted in the ICU in 0.59±1.5 days following hospital presentation and required either RRT or plasma exchange within 2.66 days. Imaging examination was performed unanimously the day upon hospital admission. All patients received corticosteroids in the first 5.95±14.3 days, while immunosuppression was given to 13(76.5%) patients within 11.5±15.5 days from hospitalization.12 patients(70.5%) suffered from associated infections. Mean BVAS(13.6±6.76) correlated to ICU admission(p 0.013, r 0.58).Patients in ICU revealed higher BVAS(22±9.53) versus non-ICU(11.8±4.76).Hospital length of stay was 14.7±10.7 days(median 14) and showed no relationship to the type of severe organ involvement. The need for ICU caring was dominant in males(p 0.05) and confirmed in patients with proteinuria(p 0.012) and at least two major organ damage.ConclusionThis study shows that severity risk factors for potential ICU admission for life-threatening AAV appear to be male gender, proteinuria and the number of affected organs.Moreover, BVAS should be considered a useful tool to predict patients' risk for intensive care management since a higher score indicates a more aggressive disease.However, time to investigational or therapeutic intervention did not correlate to patient outcome in AAV.References[1]Geetha, D., Seo, P. (2011). Life-Threatening Presentations of ANCA-Associated Vasculitis. In: Khamashta, M., Ramos-Casals, M. (eds) Autoimmune Diseases. Springer, London. https://doi.org/10.1007/978-0-85729-358-9_8Acknowledgements:NIL.Disclosure of InterestsNone Declared.
ABSTRACT
Objectives: Uncommon presentations of common diseases present a challenge in recognizing the correct diagnosis. Beside uncommon symptoms, uncommon age of onset challenges the pattern recognition abilities of clinicians. Method(s): Here we present a 6 week old boy with acute haemorrhagic edema of infancy in association with COVID-19. The otherwise healthy term born infant presented in our clinic with fever, mild respiratory symptoms and a rash. After establishing a sufficient saturation of oxygen, also during sleep, the infant was discharged. Result(s): Complete resolution of the rash was within days after. On admittance 60 mg prednisolone rectal was applied by the emergency night shift staff also to stabilize a slight wheeze due to COVID-19 but other than that no therapy was needed. Discussion(s): Reviewing the literature the benign nature of this leucocytoclastic vasculitis was commonly reported as well as the common onset during late infancy (about 8 to 23 months). Very few reports target the age group outside this age brackets. Nonetheless it is important to think outside the box when examining a patient in emergency paediatric derm.atology.
ABSTRACT
BackgroundDuring the Coronavirus disease (COVID-19) pandemic, one of the biggest concerns of rheumatologists and rheumatology patients has been whether the risk or severity of the disease will increase with immunosuppressive therapy. Some drugs have been reported to be associated with adverse Covid19 outcomes [1,2]. Cyclophosphamide (CYC) is a drug that has been used in rheumatology practice for many years. There is not enough data in the literature on the frequency or consequences of COVID-19 while receiving CYC therapy.ObjectivesThe aim of this study is to examine the frequency and outcomes of Covid19 in patients who received CYC therapy during the Covid19 pandemic.MethodsThe files of patients who received CYC therapy protocol between March 2020 and March 2022 at Başkent University Faculty of Medicine Ankara Hospital, Rheumatology outpatient clinic were retrospectively reviewed. In our clinic, CYC therapy is administered as an intravenous treatment protocol of 500 mg three times every 10 days, then 500 mg every two weeks. Although the cumulative dose varies depending on the disease and the patient, it is usually planned to be at least three gram. The diagnosis of Covid 19 was made in the patients with clinically compatible radiology and SARS-CoV-2 PCR test results.ResultsA total of 36 patients received CYC during the specified period. CYC indications were ANCA-associated vasculitis in 12 patients, interstitial lung disease associated with undifferentiated connective tissue disease in 5 patients, SLE in 5 patients, scleroderma in 4 patients, Sjögren's syndrome in 4 patients, Behçet's disease in 1 patient, vasculitis associated with sarcoidosis in 1 patient, rheumatoid vasculitis in 1 patient, leukocytoclastic vasculitis in 1 patient, polymyositis in 1 patient and Takayasu disease in 1 patient.The median age (q1-q3) was 62 (52-68) years. Covid19 infection was detected in only 3 patients (8%) during the CYC therapy protocol. The median cumulative CYC dose for these patients was 3.5 g. One out of 3 patient was hospitalized for Covid 19 pneumonia. There was no death due to Covid19.ConclusionIn this study, it has been shown that CYC therapy was safe during the Covid19 pandemic period.References[1]Samanta J, Naidu G, Deo P, Mittal S, Prasad CB, Das D, Dhir V, Sharma SK, Ramachandran R, Rathi M, Nada R, Minz RW, Jain S, Sharma A. Managing ANCA-associated vasculitis during COVID-19 pandemic: a single-center cross-sectional study. Rheumatol Int. 2022 Dec;42(12):2159-2166.[2]Singh N, Madhira V, Hu C, Olex AL, Bergquist T, Fitzgerald KC, Huling JD, Patel RC, Singh JA. Rituximab is associated with worse COVID-19 outcomes in patients with rheumatoid arthritis: A retrospective, nationally sampled cohort study from the U.S. National COVID Cohort Collaborative (N3C). Semin Arthritis Rheum. 2023 Feb;58:152149.Acknowledgements:NIL.Disclosure of InterestsNone Declared.
ABSTRACT
Objectives: Online health boards (OHBs) are web-based forums where patients post publicly about their conditions. We evaluated the extent to which OHB posts could be used to generate meaningful insights into the lived experience of patients. Specifically, we sought to (a) capture patient reactions to the Covid-19 pandemic, (b) determine pathways to diagnosis of ANCA-associated vasculitis (AAV) and (c) develop clinical outcome assessments (COAs) for congestive heart failure (CHF). Method(s): For each use case, a keyword-based search was used to retrieve relevant English language posts from multiple OHBs. A machine learning approach removed all posts except those where a patient was writing about themselves or someone in their care. Computer-assisted coding was applied to these posts and numerical analysis used to extract insights from the coding. Where possible, our findings were compared to those obtained using traditional methods. Result(s): Relevant posts were obtained from 53,134 users for the Covid study, 271 patients for the CHF work and 59 AAV patients. These proved sufficient to reveal meaningful insights across all three studies. For example, it was possible to rank lifestyle impacts of disease (e.g. limitations on exercise ranked highest, cited by 47% of CHF patients), reconstruct pathways to diagnosis (average time to AAV diagnosis was found to be seven years) and track patient concerns during the onset of the Covid-19 pandemic. Findings were broadly comparable with those reported elsewhere. Conclusion(s): Mining OHBs offers an alternative methodology for capturing the patient experience across a range of applications. Its strengths are the immediacy with which insights can be acquired, the size of cohorts that can be studied and the ability to retrospectively perform longitudinal studies. However, this approach is limited by the inability to probe beyond initial post content, the reliance on patients to proactively share their experience, and the inability to evidence their diagnosis.Copyright © 2023