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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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Background/Aims Cases of new autoimmune and autoinflammatory conditions have been reported among COVID-19 survivors. A literature review on newonset autoimmune connective tissue diseases (ACTDs) following infection with COVID-19 is lacking.This systematic literature review aimed to evaluate the potential association between COVID-19 infection and the development of new-onset ACTDs in adults. Methods Articles published until September 2022, investigating the association between COVID-19 infection and new-onset ACTDs were included. The ''population'' searched was patients with disease terms for autoimmune connective tissue diseases, including (but not limited to) systemic lupus erythematosus (SLE), Sjogren's syndrome, systemic sclerosis (SSc), any idiopathic inflammatory myositis (IIM), antisynthetase syndrome, mixed CTD and undifferentiated CTD (and related MeSH terms), with ''intervention'' as COVID-19 and related terms. For terms for COVID-19, a dedicated search strategy developed by the National Institute for Clinical Excellence was used.Medline, Embase, and Cochrane databases were searched, restricted to English-language articles only. Eligible articles were: case reports and series (of any sample size), observational studies, qualitative studies and randomised controlled trials. Patients developing ACTDs without prior COVID-19 or reporting flares of existing ACTDs were excluded. Information was extracted on patient demographics, new ACTDs' onset time, clinical characteristics, COVID-19 and ACTD treatment, and COVID-19 and ACTDs outcomes. The protocol was registered in PROSPERO (CRD42022358750). Results After deduplication, 2239 articles were identified. After screening title and , 2196 papers were excluded, with 43 proceeding to fulltext screening. Ultimately, 28 articles (all single case reports) were included. Of the 28 included patients, 64.3% were female. The mean age was 51.1 years (range 20-89 years). The USA reported the most cases (9/28). ACTD diagnoses comprised: 11 (39.3%) IIM (including 4 cases of dermatomyositis);7 (25%) SLE;4 (14.3%) anti-synthetase syndrome;4 (14.3%) SSc;2 (7.1%) other ACTD (one diagnosed with lupus/MCTD overlap). Of eight, four (14.3%) patients (including that with lupus/MCTD) were diagnosed with lupus nephritis. The average onset time from COVID-19 infection to ACTD diagnosis was 23.7days. A third of the patients were admitted to critical care, one for ACTD treatment for SLE with haemophagocytic lymphohistiocytosis (14 sessions of plasmapheresis, rituximab and intravenous corticosteroids) and nine due to COVID-19. The majority (80%) of patients went into remission of ACTD following treatment, while two (10%) patients died- one due to macrophage activation syndrome associated with anti-synthetase syndrome and two from unreported causes. Conclusion Our results suggest a potential association between COVID-19 infection and new-onset ACTDs, predominantly in young females, reflective of wider CTD epidemiology. The aetiology and mechanisms by which ACTDs arise following COVID-19 infection remain unknown and require more robust epidemiological data.
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Case Report: A 28 year old male with a past medical history of hypothyroidism and positive ANA presented to an outpatient dermatology clinic with a diffuse pruritic rash two weeks after the administration of his first Moderna COVID booster vaccine. He denied any other accompanying symptoms such as fever or chills as well as any similar rashes to prior doses of the Moderna COVID vaccine. The rash consisted of pink erythematous minimally scaly papules, thin plaques and patches involving the left and right dorsal hands, forearms, wrists, face, neck and left shoulder. The remainder of the patient's skin including the bilateral lower extremities, the eyelids, conjunctiva and oral mucosa was clear. The patient denied any similar rashes in the past. The patient denied any allergies to medications, or food or environmental allergies. He denied any notable contact allergen exposures, including to soaps, lotions, and cosmetic products. The patient also denied any significant family history or past surgical history. The patient was on Armour Thyroid for hypothyroidism and testosterone for low levels since age eighteen. The patient was started on cetirizine 10 mg once daily for the rash with minimal improvement. Autoimmune workup for the rash was notable for an elevated anti-RNP and as the patient's past medical history included Raynaud's phenomenon and ANA positivity for ten years, the patient was diagnosed with mixed connective tissue disease (MCTD). Autoimmune conditions can often have an indolent course, where symptoms progressively develop and worsen. MCTD is an autoimmune overlap syndrome that can consist of the following three connective tissue diseases: systemic lupus erythematosus, scleroderma, and polymyositis. Millions of individuals across the world are receiving COVID vaccines to protect themselves and members of their community, and it is of utmost importance that we continue to investigate adverse events. Although of low incidence, these rare effects have the ability to impact large numbers of people within both healthy and immunocompromised populations. It is critical that we examine and document them in a rigorous manner, to ensure safe vaccine delivery and reassure the public about vaccine safety overall.
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SARS-CoV2 primarily affects the respiratory system but a hyperinflammatory response leading to multisystem inflammatory syndrome - children (MIS-C), immune dysfunction and various autoimmune manifestations has also been noted. Autoimmunity depends on various factors, including genetic predisposition, environmental factors, immune dysregulation and infections acting as triggers like Epstein-Barr virus, cytomegalovirus, human immunodeficiency virus, hepatitis B. Molecular mimicry, bystander T-cell activation and persistence of viral infection are the main mechanisms behind these manifestations. We present here 3 cases of newly diagnosed connective tissue disease with high titers of COVID19 immunoglobulin G antibody in children. A 9-year-old girl with fever, oliguria and malar rash (prior history of sore throat) and a 10-year-old girl with fever for 2 weeks and choreoathetoid movements were diagnosed as systemic lupus erythematosus (SLE) nephritis (stage 4) and neuropsychiatric SLE, respectively as per European League Against Rheumatism / American College of Rheumatology 2019 criteria. An 8-year-old girl with fever, joint pain and respiratory distress (a recent contact with a positive COVID19 patient) presented with altered sensorium, Raynaud's phenomenon noted, and eventually diagnosed as mixed connective tissue disease as per Kusukawa criteria. The immune-mediated manifestations post-COVID infection are a de-novo phenomenon which necessitates further workup as not many studies exist in the pediatric population.
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Background. Several studies and cohorts with adult populations with rheumatic diseases (RD) were performed since pandemic outbreak. RD patients were more susceptible to infections and may develop severe forms of COVID-19, since they present immunosuppressive mechanisms inherent to the disease itself and to its treatment. Healthy children and adolescents seem to be less infected and present milder diseases. However, juvenile dermatomyositis patiets and immunosuppressed children have not been extensively studied. The objectives of the study are to evaluate asymptomatic SARS-CoV-2 infection in pediatric RD patients, to identify the risk factors related to contagion and to describe demographics and the profile of COVID-19 in juvenile dermatomyositis (JDM) patients followed. Methods. A cross-sectional study was conducted in March 2021, including 77 pediatric RD patients followed at a Brazilian tertiary hospital and 45 healthy controls. Data was obtained through a questionnaire applied to outpatients during the month of March 2021, before the vaccine, and contained demographic data, symptoms compatible with COVID-19 over the past year, and contact with people with confirmed COVID-19. Patients' medical records were reviewed to access data regarding disease and current medications. A qualitative immunochromatographic SARS-CoV-2 test was performed in all participants. All patients who were using rituximab or intravenous human immunoglobulin, or had symptoms of COVID-19, were excluded. Results. Patients' group included 11 (14.3%) JDM patients, 31 (40.2%) JIA, 25 (32.4%) JSLE, six patients with vasculitis, two with SS, one MCTD and one with autoinflammatory syndrome. Patients and controls were similar in terms of female gender (70.1% vs. 57.8%, p=0.173), median age (14 vs. 13 years, p=0.269) and SARS-CoV-2 serology positivity (22% vs. 15.5%, p=0.481). 80.5% of rheumatic patients were in use of immunosuppressive drugs, 27.3% of them using corticosteroids, 33.3% in high doses, and 7.8% on immunobiologicals. No statistical differences were found between positive (n=17) and negative serology (n=60) patients regarding demographic/socioeconomic data, contact with people with confirmed COVID-19, use and number of immunosuppressive drugs, use and dose of corticosteroids, use of hydroxychloroquine and immunobiological drugs (p>0.05). Regarding the profile of JDM patients, 6/11 (54%) were female, the median age was 13 years (range 9-17) and 3/11 (27%) presented COVID-19 serology positivity. 2/11 were in immunosuppressive treatment, however none of them were in use of glucocorticoids and biologic agents. Conclusions. Pediatric JDM and other rheumatic diseases patients were infected at the same rate as healthy ones. Neither the underlying pathology nor its treatment seemed to interfere with the contagion risk.
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Case Report: Acute motor and sensory axonal neuropathy (AMSAN) syndrome is a rare subtype of Guillain-Barre syndrome (GBS) with poor recovery [1]. While respiratory and gastrointestinal infections may precipitate AMSAN, an underlying autoimmune disorder is seldom reported in literature. We herein report the complex case of a patient with undiagnosed, asymptomatic mixed connective tissue disease (MCTD) who developed AMSAN syndrome. Case: A 44-year-old Asian male without medical history presented with progressively worsening weakness of both upper and lower extremities and inability to perform daily activities. His symptoms started 12 weeks prior with difficulty standing from a seated position. He felt subjectively better for some time until a week prior, when he became bedbound. He had diarrhea 6 months ago, with 5-6 loose bowel movements a day for a few weeks. Vital signs on admission was normal. On neurological examination, he was alert and oriented, with bilateral upper and lower extremity flaccid paralysis, diffuse muscle atrophy, bilateral hand and foot drop, negative Hoover sign, diffuse areflexia, and intact sensation. Cerebrospinal fluid (CSF) analysis showed WBC 0 and protein level 136. MRI cervical, thoracic, and lumbar spine were normal. EMG revealed sensory involvement with positive sharp waves in proximal muscles along with fibrillations. Intravenous immunoglobulin (IVIG) was initiated at 0.4 mg/kg for 5 days. Infectious workup for COVID-19, stool culture, HIV, TB, RPR and campylobacter jejuni antibody (Ab), was negative. ANA was positive in a speckled pattern with titres 1:1280, with a positive RNP Ab, SS-A, and RF IgM, IgG and IgA. Rest of the autoimmune workup (anti-dsDNA, anti-CCP, SS-B, aldolase, anti-Jo-1, anti-Scl-70, p-ANCA, c-ANCA, anti-GM1, anti-GQ1b, and anti-GD1a ganglioside Ab) was negative. The myositis specific 11 Ab panel was negative. Despite IVIG therapy, he developed dysphagia, respiratory distress, with a negative inspiratory force of -0, requiring intubation. He had a tracheostomy and PEG tube placed and remains quadraplegic nearly 120 days later. Discussion(s): The authors report a unique case of a patient who became progressively weak over 3 months, leading to complete quadriplegia. Interestingly, this is more consistent with chronic inflammatory demyelinating poly-neuropathy (CIDP), as AMSAN typically develops over a short period of 2 to 4 weeks [2]. Despite having negative anti-GM1 and anti-GD1a Ab (in which positive Ab are pathognomonic but not always present in AMSAN syndrome), the patient had weakness that began in the lower extremities, progressing to paralysis, along with albuminocytological dissociation on CSF analysis, pointing to a GBS diagnosis [3]. He had sensory involvement in the EMG, thus making the diagnosis as AMSAN. He had an undiagnosed, asymptomatic autoimmune process most consistent with MCTD. Whether the two disease processes are related to each other is a concept that has not yet been investigated. Pediatric Clinical Case Reports Concurrent Session Saturday February 4, 2023 1:00 PM Copyright © 2023 Southern Society for Clinical Investigation.
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Background: According to newspaper Bernama, 87.6% of adolescents in Malaysia aged between 12 and 17 have completed their vaccination and 97.7% of the adult population have completed theirs as of 2nd January 2022.The acceptance of patients with rheumatic diseases on Covid-19 vaccination are crucial in the successful long term protection against Covid-19 infection. We conducted a phone interview to determine the acceptance of Covid-19 vaccination amongst adolescents with underlying rheumatic diseases. Objective(s): To determine the acceptance of Covid-19 vaccination amongst adolescents with underlying rheumatic diseases. Method(s): This was a phone survey. The electronic medical records of all rheumatology patients follow up in rheumatology clinic Hospital Sultan Ismail, Malaysia from 1st January 2012 to 31th December 2021 were reviewed and patients with age group from 12 to 21 were identified. Demographic and diagnosis of the patients collected. Result(s): Phone survey was done after data extracted from medical records. For those under the age of 18, guardian of the patients was interviewed. A total of 50 patients were identified. 36 of them were having systemic lupus erythematosus (SLE), 5 of them were having juvenile idiopathic arthritis (JIA),2 of them were having psoriatic arthritis (PSA) and another 2 of them were having Rheumatoid arthritis (RA), followed by rheumatoid arthritis (RA) overlapped SLE, juvenile dermatomyositis, Henoch-Schonlein purpura, SLE overlapped with JIA and mixed connective tissue disease, 1 each respectively. Most of the patients were female (46/50) and majority of them were Malay (33/50). This was followed by Chinese (10/50), Indian (4/50) and others (3/50). The mean age group was 18 (range from 13 to 21). Majority of them patients are keen or already completed Covid-19 vaccination with the acceptance rate as high as 92% (46/50). Only 8% of them not keen for vaccination with the reason of worrying the risk of myocarditis post vaccination. Conclusion(s): The overall acceptance rate of Covid-19 vaccination amongst adolescents with rheumatic diseases are very encouraging with the percentage of >90% despite of lacking knowledge about vaccine Covid-19. This result can assist our Ministry of Health plan for future battle to improve vaccine uptake that hopefully can lead to herd immunity against COVID-19 infection.
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Background/Purpose: Concomitant systemic lupus erythematosus (SLE) and human immunodeficiency virus (HIV) infection cases are rare worldwide. It is established that SLE patients have an increased risk of opportunistic infection due to immune dysregulation, as well as in HIV. Method(s): A case of a 25-year- old Filipino man with systemic lupus erythematosus admitted due to a 1-week intermittent fever associated with headache, loss of appetite, and generalized body weakness was reviewed in a tertiary hospital in the Philippines. Result(s): An initial diagnosis was made from the clinical presentation of Raynaud's phenomenon, an elevated antinuclear antibody (1:320;nuclear, speckled), 2+ proteinuria, thrombocytopenia, and nail fold capillaroscopy findings consistent with mixed connective tissue disease. Patient was started on hydroxychloroquine and prednisone. He was admitted as a case of Streptococcus bacteremia with COVID-19 pneumonia after initial diagnosis, presenting as fever, and thrombocytopenia as low as 23.000/mul. Patient presented with a scaly erythematous annular lesion at his left wrist since December 2021 where a skin punch biopsy showed findings consistent with dermatophytosis. Direct immunofluorescence staining showed deposition of granular IgM (+3), C3 (+1), Fibrinogen (+3), and C1q (+1) in the basement membrane zone consistent with Lupus Erythematosus. Additional findings were oral thrush, dermatophytosis, and Pneumocystis pneumonia. Patient was started on antibiotics, remdesivir, and antifungal medications. Being severely immunocompromised, work up for HIV was initiated. Rapid HIV screening was positive, CD4 count revealed 7 (3.14%), and subsequent confirmatory western blot was positive. Additional treatment included hydroxychloroquine, methylprednisolone pulse therapy, and platelet concentrate transfusion. He was referred for CD4 monitoring, and ARV treatment enrollment, however, the patient expired a month after his discharge. Conclusion(s): This case is thereby reported to document a rare case of systemic lupus erythematosus (SLE) male patient with concomitant HIV, SARS-CoV- 2, and opportunistic infections secondary to AIDS. Diagnosis becomes challenging in patients with autoimmune diseases and multiple infectious diseases as clinical presentations tend to overlap and may show similar manifestations. In this setting, skin biopsy utilizing direct immunofluorescence can help establish an accurate diagnosis especially when clinical features and histopathology are overlapping.
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Introduction: Viral infections have been implicated in the initiation of the autoimmune diseases. Recent reports suggest that a proportion of patients with COVID-19 develop severe disease with multiple organ injuries. We evaluated the relationship between COVID-19 severity, prevalence and persistence of antinuclear and other systemic and organ specific autoantibodies as well as SARS-CoV-2 infection specific anti-nucleocapsid (N) IgG antibodies and protective neutralizing antibody (Nab) levels. Methods: Samples from 119 COVID-19 patients categorized based on their level of care and 284 healthy subjects were tested for the presence and persistence of antinuclear and other systemic and organ specific autoantibodies as well as SARS-CoV-2 and neutralizing antibody levels. Results: The data shows significantly increased levels of anti RNP-A, anti-nucleocapsid and neutralizing antibody among patients receiving ICU care compared to non-ICU care. Furthermore, subjects receiving ICU care demonstrated significantly higher nucleocapsid IgG levels among the RNP-A positive cohort compared to RNP-A negative cohort. Notably, the expression of anti RNP-A antibodies is transient that reverts to non-reactive status between 20 and 60 days post symptom onset. Conclusions: COVID-19 patients in ICU care exhibit significantly higher levels of transient RNP-A autoantibodies, anti-nucleocapsid, and SARS-CoV-2 neutralizing antibodies compared to patients in non-ICU care.
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Myasthenia gravis (MG) is an autoimmune illness that causes neuromuscular junctions to be damaged by anti-acetylcholine receptor antibodies. It is a very rare condition that is more common among women. Fatigable fluctuating diplopia or ptosis is the characteristic early appearance of this condition. Dysphagia or dysphonia may be present in rare cases. This illness can affect any group of skeletal muscles, including those in the neck and upper limbs. It can also affect the muscles that help you breathe, which can lead to breathing failure. We present a case of a 20-year-old female diagnosed with mixed connective tissue disease presenting with acute respiratory failure as the initial presentation of MG. Clinicians have to have a high index of suspicion for myasthenia when patients arrive with fatigable muscle weakness. This will cut down on the amount of money spent on investigations and the risk of morbidity.
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SESSION TITLE: Unusual Pneumonias SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/19/2022 12:45 pm - 1:45 pm INTRODUCTION: Invasive pulmonary aspergillosis (IPA) commonly occurs in the setting of immunosuppression. Underlying lung disease is a well-known risk factor for IPA;however, interstitial lung disease (ILD) has not been recognized as a risk factor for IPA[1]. CASE PRESENTATION: A 40-year-old male with a history of a failed kidney transplant now on hemodialysis (HD), Juvenile Rheumatoid Arthritis, Mixed Connective Tissue Disease, Aspergilloma led to right lower lobectomy a year ago, COVID-19 infection three months ago, chronic lung disease (CLD) thought to be due to Nonspecific interstitial pneumonia (NSIP) presented with dyspnea. He had several hospitalizations for respiratory failure needing intubation or NIPPV, broad-spectrum antibiotics, steroids, and HD with improved respiratory status, eventually discharged. Bronchoalveolar lavage fluid culture grew aspergillus terreus but was negative for Pneumocystis (PCP), bacteria, acid-fast bacilli, and Nocardia. The transbronchial biopsies showed mixed inflammatory type and fungal forms in one specimen. Additionally, the initially negative galactomannan converted into a serial rise in galactomannan (>3.75 Index) along with a rise in beta d-glucan (>500 pg/ml). Unfortunately, he had gaps in antifungals and was readmitted similarly. Micafungin was added for dual fungal coverage and was planned for surgical lung biopsy to characterize ILD further once his respiratory status allows. DISCUSSION: He has multiple risk factors for developing IPA, such as high-dose steroids for ILD and recent COVID infection. Initially, respiratory failure was thought to be due to exacerbation of ILD, and suspicion for IPA was low because of lack of neutropenia, negative fungal biomarkers, lack of classic findings on lung imaging, and in-hospital clinical improvement with steroids. However, the eventual course of recurrent respiratory failure while on high-dose steroids, along with gaps in antifungal therapy and continued growth of Aspergillus, made IPA the most likely diagnosis. For IPA, the mainstay of treatment is both adequate antifungal therapy and reduction in immunosuppression to the extent possible[2];however, it is unclear if his underlying ILD can tolerate steroid taper. He will need a lung transplant after adequately treating IPA. CONCLUSIONS: There are no current guidelines on simultaneously treating IPA and NSIP. It is challenging to balance reduction in immunosuppression as tolerated for ILD and concurrently maintain antifungal therapy. During this patient's hospitalization, there have been considerations of using a steroid-sparing agent for his suspected NSIP, however, in the setting of active infection, its benefit is debatable.[3] Reference #1: Matsuyama H, Miyoshi S, Sugino K, et al. Fatal Invasive Pulmonary Aspergillosis Associated with Nonspecific Interstitial Pneumonia: An Autopsy Case Report. Intern Med. 2018;57(24):3619-3624. doi:10.2169/internalmedicine.1144-18 Reference #2: Thomas F. Patterson, George R. Thompson, III, David W. Denning, Jay A. Fishman, Susan Hadley, Raoul Herbrecht, Dimitrios P. Kontoyiannis, Kieren A. Marr, Vicki A. Morrison, M. Hong Nguyen, Brahm H. Segal, William J. Steinbach, David A. Stevens, Thomas J. Walsh, John R. Wingard, Jo-Anne H. Young, John E. Bennett, Practice Guidelines for the Diagnosis and Management of Aspergillosis: 2016 Update by the Infectious Diseases Society of America, Clinical Infectious Diseases, Volume 63, Issue 4, 15 August 2016, Pages e1–e60, https://doi.org/10.1093/cid/ciw326 Reference #3: Mezger, M., Wozniok, I., Blockhaus, C., Kurzai, O., Hebart, H., Einsele, H., & Loeffler, J. (2008). Impact of mycophenolic acid on the functionality of human polymorphonuclear neutrophils and dendritic cells during interaction with Aspergillus fumigatus. Antimicrobial agents and chemotherapy, 52(7), 2644–2646. https://doi.org/10.1128/AAC.01618-07 DISCLOSURES: No relevant relationships by Nasir Alhamdan No relevant relati nships by Parth Jamindar No relevant relationships by Harshitha Mergey Devender No relevant relationships by Abira Usman No relevant relationships by Vishruth Vyata
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Background: Since the COVID-19 vaccination campaign was launched all over Europe, there has been general agreement on how benefts of SARS-CoV2 vaccines outweigh the risks in patients with rare connective tissue diseases (rCTDs). Yet, there is still limited evidence regarding safety and efficacy of such vaccines in these patients, especially in the long-term. For this reason, in the framework of ERN-ReCONNET, an observational long-term study (VACCINATE) was designed in order to explore the long-term outcome of COVID-19 vaccination in rCTDs patients. The consent form was developed thanks to the involvement of the ERN ReCONNET ePAG Advocates (European Patients Advocacy Group). Objectives: To evaluate the safety profile of COVID-19 vaccination in rCTDs patients and the potential impact on disease activity. Primary endpoints were the prevalence of adverse events (AEs) and of disease exacerbations post-vaccination. Secondary endpoints were the proportion of serious adverse events (SAEs) and adverse events of special interest for COVID-19 (adapted from https://bright-oncollaboration.us/wp-content/uploads/2021/01/SO2-D2.1.2-V1.2-COVID-19- AESI-update-23Dec2020-review-fnal.pdf) Methods: The frst ad-interim analysis of the VACCINATE study involved 9 ERN-ReCONNET Network centres. Patients over 18 years of age with a known rCTD and who received vaccine against COVID-19 were eligible for recruitment. Demographic data and diagnoses were collected at the time of enrolment, while the appearance of AEs and potential disease exacerbations were monitored after one week from each vaccination dose, and then after 4, 12 and 24 weeks from the second dose. A disease exacerbation was defned as at least one of the following: new manifestations attributable to disease activity, hospital-ization, increase in PGA from previous evaluation, addition of corticosteroids or immunosuppressants. Results: A cohort of 300 patients (261 females, mean age 52, range 18-85) was recruited. Systemic lupus erythematosus (44%) and systemic sclerosis (16%) were the most frequent diagnoses, followed by Sjogren's syndrome (SS,12%), idiopathic infammatory myositis (IMM,10%), undifferentiated connective tissue disease (UCTD,8%), mixed connective tissue disease (MCTD,4%), Ehlers-Dan-los's syndrome (EDS,4%), antiphospholipid syndrome (APS,2%). AEs appearing 7 days after the frst and second doses were reported in 93 (31%) and 96 (32%) patients respectively, mainly represented by fatigue, injection site reaction, headache, fever and myalgia. Otitis, urticaria, Herpes Simplex-related rash, stomatitis, migraine with aura, vertigo, tinnitus and sleepiness were reported with very low frequency. Less than 2% of patients experienced AEs within 24 weeks from the second dose. No SAEs or AEs of special interest were observed in the study period. There were 25 disease exacerbations (8%), 7 of which severe. The highest number of exacerbations was observed after 4 weeks from the second dose (12 within week 4, 6 within week 12 and 7 within week 24). Disease exacerbation was most frequent in patients with EDS (33%) and MCTD (25%). Conclusion: This preliminary analysis shows that COVID-19 vaccination is safe in rCTDs patients. AEs appear most often early after vaccination and are usually mild. Disease exacerbations are not frequent, but can be potentially severe and tend to occur most frequently within the frst month after vaccination. Exacerbations can also occur 3-6 months after vaccination, although a causal relationship with the vaccination remains to be established. Our present data underline the importance of long-term observational studies.
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Background: According to Statista, 78.5% of the population in Malaysia have completed their vaccination as of 6th january 2022 [1].The acceptance of patients with rheumatic diseases on Covid-19 vaccination is crucial in the long term protection against Covid-19 infection. We conducted a survey to determine the acceptance of Covid-19 vaccination amongst patients with underlying rheumatic disease. Objectives: To fnd out the reasons of vaccination refusal amongst rheumatology patients. Methods: This was an interview survey. All rheumatology patients who were follow up in rheumatology clinic Hospital Sultan Ismail, Malaysia from 26th April 2021 to 25th July 2021 (total 3 months) were interviewed. Demographic and diagnosis of the patients were collected. Results: A total of 952 patients were identified. 83.7% of them were female patients (797/952) and majority of them were Malay (46.4%). This was followed by Chinese (36.1%), Indian (16.3%) and others (1.2%). The mean age group was 48 (range from 13-85). 97.6% of the respondents were categorized as having inactive disease during the interview sessions. 36.6% of the patients were diagnosed to have rheumatoid arthritis and 29.1% of them were having systemic lupus erythematosus. These were followed by psoriatic arthritis (10.9%), mixed connective tissue disease (5.5%), systemic sclerosis (2.9%), gout (2.6%), Sjogren syndrome (1.9%), ankylosing spondylitis (1.6%), myositis (1.5%), vascu-litis (1.3%), osteoarthritis (1.2%), antiphospholipid syndrome (0.9%), non-specific arthralgia (0.8%), juvenile idiopathic arthritis (0.8%), seronegative spondyloarthropathy (0.8%), undifferentiated connective tissue disease (0.7%), adult onset still's disease 0.5%) and others (< 0.5% each for Ig G 4 related disease, soft tissue rheumatism and fibro-myalgia). 87.3% of them were keen or have already received Covid-19 vaccination. 12.7% of them were not keen for the vaccination with various reasons. 48.8% of them were worrying about worsening clinical condition, 12.4% of them were not keen as they concerned about side effects (3 worry about fever, 1 worry about hepatitis, 1 for nausea, 1 for dizziness, 1 for breathlessness, and 7 for non-specific reasons). 10.7% of them were not keen due to pregnancy, 5.79% of them were not keen as worried about allergic reactions, 4.9% of them were worrying about sudden cardiac death, 4% were not keen as on chemotherapy treatment, 3 % of them doubted the efficacy of vaccination, 2.5% were not keen as they worried about heart disease, 2.5% worried about increase risks of infection and others (2 for old age, 2 for thrombotic event, 2 for drug interaction and 1 patient due to hemodialysis). Conclusion: The overall acceptance rate of Covid-19 vaccination amongst patients with rheumatic diseases is very encouraging with the percentage of >85% despite of lacking knowledge about vaccine Covid-19. This result can assist our Ministry of Health to plan for future battle to improve vaccine uptake that hopefully can lead to herd immunity against COVID-19 infection. More counseling sessions are required to clear up the doubts of vaccination and increase the vaccination rate amongst rheumatic patients.
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We report the systemic and ophthalmic findings in a female patient with mixed connective tissue disease (MCTD) who subsequently developed retinal vasculitis following coronavirus disease 2019 (COVID-19) reinfection. The patient was a known case of MCTD maintained in remission on immunosuppressive treatment. She subsequently developed retinal vasculitis with areas of capillary non-perfusion in the right eye. This was a finding not seen previously. She was started on an enhanced immunosuppressive regimen along with scatter laser photocoagulation. COVID-19 has been reported to lead to the development of autoimmune disease, both de novo as well as the worsening of pre-existing disease. The onset of retinal vasculitis may potentially be due to a post-COVID-19 exacerbation of her pre-existing MCTD. Physicians should be aware of this possibility and screen for the same.
ABSTRACT
Background/Aims According to newspaper Bernama, 6.5% of adolescents in Malaysia aged between 12 and 17 have completed their vaccination and 89.7% of the adult population have completed theirs as of 9th October 2021. The acceptance of patients with rheumatic diseases on Covid-19 vaccination are crucial in the successful long-term protection against COVID-19 infection. We conducted a phone survey to determine the acceptance of COVID-19 vaccination amongst adolescents with underlying rheumatic disease. Methods This was a phone survey. The electronic medical records of all rheumatology patients follow up in rheumatology clinic Hospital Sultan Ismail, Malaysia from 2019 to 2021 were reviewed and patients with the age group between 12 to 21 were identified. Demographic data, diagnosis of the disease and outcome of the survey were collected and analysed. Results Phone survey was done after data extracted from medical records. For those under the age of 18, guardian of the patients was interviewed. A total of 50 patients were identified. Most of the patients were female (46/50) and majority of them were Malay (33/50). This was followed by Chinese (10/50), Indian (4/50) and others (3/50). The mean age group was 18 (range from 13 to 21). 36 of them were having systemic lupus erythematosus (SLE), 5 of them were having juvenile idiopathic arthritis (JIA) ,2 of them were having psoriatic arthritis (PSA) and another 2 of them were having rheumatoid arthritis (RA), followed by RA overlapped SLE, juvenile dermatomyositis, Henoch-Schönlein purpura, SLE overlapped with JIA and mixed connective tissue disease about 1 each respectively. Majority of them patients are keen or already completed COVID-19 vaccination with the acceptance rate of as high as 92% (46/50). Only 8% of them are not keen for vaccination with the only reason of worrying the risk of myocarditis post vaccination (1 SLE patient and 1 PSA patient). Conclusion The overall acceptance rate of COVID-19 vaccination amongst adolescents with rheumatic diseases are very encouraging with the percentage of>90% despite lacking knowledge about vaccination for COVID-19. This result can assist our Ministry of Health in planning for future battles to improve vaccine uptake that hopefully can lead to herd immunity against COVID-19 infection.
ABSTRACT
We describe an interesting rare case of a 61-year-old woman who was admitted to our hospital for exertional dyspnea, non-productive cough, and generalized weakness of six months duration. Her computed tomography was significant for ground-glass opacities combined with bibasilar consolidations and numerous pulmonary cysts. There can be a significant overlap in imaging findings of post-coronavirus disease 2019 (COVID-19) lung disease and interstitial lung disease from autoimmune diseases. We review in extensive detail the differential diagnosis for these imaging findings from a pulmonologist's perspective and discuss investigations required for further workup. Our patient underwent transbronchial biopsy and was eventually diagnosed with lymphocytic interstitial pneumonia with Sjogren predominant mixed connective tissue disease. We also review in detail the current literature and prognosis for this interesting disease.