Experiences of Covid19 Disease and Vaccination in Hungarian Myositis Patients: Anti-Jo-1 Is a Risk Factor for Hospitalization

Background. Pandemic caused by severe acute respiratory syndrome coronavirus 2 (COVID19) raises a smash barrier for clinicians and patients since 2 years. Limited information is available on disease course after COVID19 infection or vaccination in patients with idiopathic inflammatory myopathies (IIM). Objective(s): The primary goals of the current research were to assess frequency and outcome of COVID19 disease and to determine the vaccination rate and effect in our IIM cohort. Secondary objectives were to search for risk factors of infection, predictive factors of hospitalization and to assess incidence of pots-vaccination adverse events and breakthrough infections. Methods. We identified the confirmed COVID19 positive patients and assessed disease course and outcome on 01/06/2021 in our cohort then patients were prospectively followed. Incidence and complications of infection and vaccination were determined by questionnaires and using the database. Anti-SARS-CoV-2 spike protein electrochemiluminescent immunoassay has been used to assess seroconversion. Disease activity was determined by physician global activity. Results. A total of 176 patients were screened and 101 participated in the study. By 01/06/2021, the COVID infection rate was 34.7%, which was significantly higher than the national prevalence at that time (8.2%). A third of these infections occurred asymptomatically or mild, but 20% of the infected patients were hospitalized, one patient died. Longer disease duration (8.67 vs. 17.87 years;p=0.003) and higher incidence of anti-Jo-1 antibody (57% vs. 10%;p=0.018) were significantly associated with hospitalization. All patients became seropositive after COVID19 infection regardless of immunosuppressive therapy or symptoms severity, meanwhile 72.3% of patients became seropositive after vaccination. Different vaccines induced various titer of antibody against the spike protein. Significantly higher antibody titers was detected after Pfizer-BioNTech (177.1 U / ml vs. 81.1 U / ml;p=0.001) and numerically lower ones after AstraZeneca (45.05 U/ml vs. 126.93 U/ml p=0.054) vaccination compared to others. Patients receiving steroid therapy had significantly decreased post-vaccination antibody response compared to those without steroid treatment (94.03 U/ml vs. 165.6 U/ ml;p=0.008). We did not found short term vaccine related major adverse events. Long term data by 15/02/2022 revealed more infections (42.57%), where anti-Jo1 positivity still showed significant association with hospitalization (50% vs. 9%;p=0.0103). Breakthrough infection was detected in 9,25 % of the vaccinated patients, which was significantly more often after Astra Zeneca vaccination (40 % vs. 7%, p=0.017). All the fatal (n=3) COVID infections occurred in patients with seronegativity to anti-spike protein regardless vaccination. We identified 7,4 % post vaccination disease relapse needing therapy changes and 24,7% new autoantibody positivity. Conclusions. Based on our results, myositis may be associated with an increased risk of COVID19 disease. Independent risk factor for hospitalization for unvaccinated people is anti-Jo1 positivity. Anti-SARS-CoV2 vaccines are safe, tolerable, could prevent complicated infections and strongly recommended for IIM population. Further investigation is required to assess clinical significance of post-vaccination disease flare.

Rituximab in the Treatment of Idiopathic Inflammatory Myopathies with Interstitial Lung Disease, Long-Term Follow-Up

Background. Interstitial lung disease (ILD) is the common internal organ manifestation of idiopathic inflammatory myopathies (IIM) that can severely affect the course and prognosis of the disease. Rituximab (RTX) has been used to treat IIM, including variants with ILD. Objectives. To describe the course of disease in IIM patients with ILD, treated with RTX in long-term follow-up. Methods. Our prospective study included 35 pts with IIM fulfilling Bohan and Peter criteria and having ILD. The mean age was 51.8+/-11.9 years, female-26 pts (74%);24 (68.5%) with antisynthetase syndrome, 5 (14.3%) dermatomyositis (DM), 5 (14.3%) with a-Pm/Scl overlap myositis and 1 (2,9%) with a-SRP necrotizing myopathy were included. 25 (71,4% ) patients had nonspecific interstitial pneumonia, 9 (25,7%) organizing pneumonia (OP) and 1 (2,9%) OP, transformed to diffuse alveolar damage. All pts had the standard examination including manual muscle testing (MMT), creatinkinase (CK) anti-Jo-1 antibodies (anti-Jo-1) assay;forced vital capacity (FVC) and carbon monoxide diffusion capacity (DLCO) evaluation as well as high-resolution computed tomography (HRCT) scanning of the chest were performed at baseline, and 36 and more months. The median disease duration was 3.2 [0.16-18] years, 21 (60%) of pts were positive for a-Jo-1 antibody. All pts received prednisolone at a mean dose of 24.3+/-13 mg/day, immunosupressants at inclusion received 25 (71%) pts: cyclophosphamide 18 , mycophenolate mofetil 6 and comdination 1;Rituximab (RTX) was administered in case of severe course of disease and intolerance or inadequate response to GC and other immunosuppressive drugs. Results. The mean follow-up period after the first infusion of RTX was 47.2+/-11.9 months. Pts received 1-11 courses of RTX . The cumulative mean dose of RTX was 4.6 +/-2.5g. MMT 8 increased from 135.8+/-13.5 to 148.75+/-3.5 (p=0.000001). CK level decreased DELTACK - 762 u/l(median 340;25th% 9;75th% 821). anti-Jo-1 decreased from 173.4+/-37 to 96.5+/-79 u/ml (p=0.00002), FVC increased from 82+/-22.6 to 96,9+/-22% (p=0.00011). DLCO increased from 51.4+/-15.2 to 60+/-77.8% (p=0.0001). The mean prednisone dose was reduced from 24.3+/-13 to 5.7+/-2.4 mg/day. 3 pts died: ILD progression was the cause of death in 1 case, 1 bacterial pneumonia and COVID19 pneumonia. Conclusions. The results of this study confirm the positive effect of RTX in IIM patients with ILD (increase of muscle strength and improve lung function, decrease in anti-Jo-1 levels) and also its good steroid-sparing effect. RTX could be considered as an effective drug for the complex therapy of IIM patients with ILD when standard therapy is ineffective or impossible.

Acute motor and sensory axonal neuropathy in a patient with undiagnosed mixed connective tissue disease

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.

Dermatomyositis following SARS-CoV- 2 2 vaccination

Purpose: To report a case of a 51-year- old male who developed dermatomyositis following the second dose of coronavirus disease (COVID-19) vaccine. Method(s): Case report Result: Case: We report a case of 51-year- old male who developed erythematous maculopapular rash on the upper anterior chest and upper back along with symmetric proximal muscle weakness two months after receiving his second dose of CoronaVac vaccine. His symptoms were followed by edema in the periorbital area which later involved the upper and lower extremities. He had dysphagia and weight loss. He had no known family history of autoimmune diseases. Physical examination revealed macular erythema over the lower anterior neck and upper back. Heliotrope rash and hyperkeratotic pink scaly papules on bilateral lateral second digits (mechanic's hands) were seen. Symmetric proximal muscle weakness in the upper and lower extremities was objectified. Blood tests showed elevated muscle enzymes (total CK: 3899 U/L, CK MB mass: 15.4 ng/mL, LDH: 683, AST: 232 U/L, ALT: 66 IU/L) elevated ESR (36) and normal CRP. Anti Jo 1 and anti U1 RNP were negative. Work up for systemic infection, thyroid function and malignancy were unremarkable. Diagnosis: Diagnosis of dermatomyositis was made based on clinical history and physical exam findings of symmetric proximal weakness, presence of heliotrope rash, V sign and shawl sign. Laboratory tests revealed elevated total CK, CK MB mass, LDH, AST, ALT and ESR consistent with an inflammatory myositis. Intervention(s): Hydrocortisone 1 mg/kg/day was started. Azathioprine was commenced on the 3rd hospital day. Ethical consideration: Informed consent for both written and photographic content was secured and patient confidentiality was observed. Conclusion(s): This case highlights the possible association between COVID 19 vaccine and this rare autoimmune disease. We hypothesize that among patients with genetic predisposition, the possibility of vaccines triggering and unmasking an autoimmune event is possible. (Figure Presented).

A case of morphea following the inactivated COVID-19 vaccine

Background: The emergence of the coronavirus disease (COVID-19) prompted pharmaceutical companies to develop effective vaccines to address the problem. While studies prove the vaccines are safe, rare systemic side effects remain possible. All types can cause various vaccine-related adverse reactions which are continuously being monitored. This paper aims to highlight new data on immunologic reactions to COVID-19 vaccines. Morphea demonstrated after COVID-19 vaccination is rare. Herein, we report a case of morphea that was most likely triggered by the immune response against inactivated COVID-19 vaccine. Method(s): A case of morphea was reviewed at the clinic in a tertiary hospital in the Philippines. Result(s): A 48 year old Japanese male had no underlying co-morbidities and no previous COVID-19 infection. He had his first dose of inactivated COVID-19 vaccine, coronaVac (sinovac) with no untoward reactions. After a month, he had his second dose. One week later, the patient started to have a red plaque on his upper back, palpable, tender on palpation and pruritic. Review of systems was unremarkable. The patient denied any insect bites or skin trauma. No medications applied or taken. No known allergies to food, medications or vaccines. He is a 32 pack years smoker. No family history of any autoimmune diseases. In five months, the skin lesion insidiously progressed, thickened and now spreading to the left side of the back. The patient sought consult with a dermatologist and rheumatologist. Physical examination revealed thickened skin and subcutaneous tissue on the upper back with post-inflammatory hyperpigmentation. Work-up showed normal complete blood count, normal chest x-ray, non-reactive Hepatitis B antigen. Antinuclear antibody (ANA) was positive with 1:80 titer and nuclear speckled pattern. Anti-double stranded DNA (anti-dsDNA), anti-smith, antinuclear ribonucleoprotein (anti-RNP), anti-SSA, anti-SSB and anti-Jo- 1 were all negative. The patient's skin biopsy to the reticular dermis showed findings that are consistent with Morphea. The patient was then started on Methotrexate. Conclusion(s): People should be educated about the possible outcomes of COVID-19 vaccines. One of these are immune-related diseases, such as morphea. The underlying mechanism of morphea is multifactorial but one hypothesis highlighted that the spike glycoprotein from vaccination drives these skin reactions. Other studies demonstrated molecular mimicry to viral epitopes. Discussing this cutaneous manifestation secondary to COVID-19 vaccine stressed the importance of this clinical condition, in order to provide a proper diagnosis and therapeutic management. Although there are novel case reports of morphea induced by COVID-19 mRNA vaccine, inactivated COVID-19 vaccine-related morphea has not been reported yet.

ANTISYNTHETASE SYNDROME PRESENTING AS INTERSTITIAL LUNG DISEASE: A CASE OF ANCHORING BIAS DURING THE COVID-19 PANDEMIC

SESSION TITLE: Dyspne Mysteries SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 01:35 pm - 02:35 pm INTRODUCTION: Anti-synthetase (AS) syndrome is characterized by interstitial lung disease (ILD), arthritis, myositis, fever, or Raynaud's phenomenon in the presence of an AS autoantibody (1). At least 70% of patients with AS syndrome develop ILD (2), and it represents the major cause of mortality in these patients with a 10 year survival rate of 73%. In a small cohort study, the anti-PL-12 antibody subtype was found to be strongly associated with ILD (3). CASE PRESENTATION: A 35 year old female with a history of tobacco use disorder presented to the hospital with three months of recurrent subjective fevers, non-productive cough, and dyspnea on exertion. She denied arthralgias, muscle weakness and hemoptysis. She initially presented to her primary care physician with these symptoms and was prescribed amoxicillin for streptococcal pharyngitis. The patient continued to be symptomatic and was treated empirically for COVID-19 pneumonia twice despite two negative COVID-19 tests and without any significant clinical improvement in her respiratory status. On admission, she was febrile, tachycardic, and had a new oxygen requirement with bilateral coarse breath sounds on exam. She had no leukocytosis and her COVID-19 test was negative. CT angiography of the chest showed extensive mixed reticular and airspace opacities with peribronchial predilection and peripheral sparing (figure 1). A bronchial alveolar lavage was notable only for neutrophilia (19%) and eosinophilia (4%). Rheumatological workup revealed elevated rheumatoid factor, positive antinuclear antibody (1:40), weakly positive anti–Sjögren's-syndrome-related antigen A antibody (50 AU/ml), undetectable anti-Jo-1 antibody and positive anti-PL-12 antibody. Pulmonary function testing revealed a TLC of 40% and DLCO of 28%, consistent with a restrictive pattern. Considering the patient's organizing pneumonia, positive antibodies, and findings of "mechanic's hands,” the patient was diagnosed with anti-synthetase syndrome with ILD. She was started on oral prednisone and mycophenolate mofetil. On follow-up, she was noted to have symptomatic improvement and stable hypoxia without clinical signs of disease progression. DISCUSSION: During the coronavirus pandemic, the resemblance of COVID-19 pneumonia to other diseases, in the absence of conscious suspicion for other etiologies, can lead to anchoring and availability bias thereby delaying diagnosis and appropriate treatment. Additionally, anti-synthetase syndrome should be considered in the differential diagnosis of ILD even in the absence of arthritis and myositis, as respiratory symptoms are often the first presenting signs. CONCLUSIONS: Increased responsibility is required of diagnosticians to exercise due diligence and active recognition of COVID availability and anchor bias to avoid missing crucial diagnoses. Reference #1: Cojocaru, Manole, Inimioara Mihaela Cojocaru, and Bogdan Chicos. "New insights into antisynthetase syndrome.” Maedica 11.2 (2016): 130. Reference #2: Marco, Joanna L., and Bridget F. Collins. "Clinical manifestations and treatment of antisynthetase syndrome.” Best Practice & Research Clinical Rheumatology 34.4 (2020): 101503. Reference #3: Kalluri, Meena, et al. "Clinical profile of anti-PL-12 autoantibody: cohort study and review of the literature.” Chest 135.6 (2009): 1550-1556. DISCLOSURES: No relevant relationships by Mario Flores No relevant relationships by David Jackson No relevant relationships by Lisa Saa No relevant relationships by Abu Baker Sheikh

IDIOPATHIC INFLAMMATORY MYOPATHIES WITH INTERSTITIAL LUNG DISEASE TREATED WITH RITUXIMAB AND LONG-TERM FOLLOW-UP

SESSION TITLE: Treatment Debates in Critical Care SESSION TYPE: Rapid Fire Original Inv PRESENTED ON: 10/17/2022 12:15 pm - 1:15 pm PURPOSE: Interstitial lung disease (ILD) is the common internal organ manifestation of idiopathic inflammatory myopathies (IIM) that can severely affect the course and prognosis of the disease. Rituximab (RTX) has been used to treat IIM, including variants with ILD. OBJECTIVES:To describe the course of disease in IIM patients with ILD, treated with RTX in long-term follow-up. METHODS: Our prospective study included 35 pts with IIM fulfilling Bohan and Peter criteria and having ILD. The mean age was 51.8±11.9 years, female-26 pts (74%);24 (68.5%) with antisynthetase syndrome, 5 (14.3%) dermatomyositis (DM), 5 (14.3%) with a-Pm/Scl overlap myositis and 1 (2,9%) with a-SRP necrotizing myopathy were included. 25 (71,4% ) patients had nonspecific interstitial pneumonia, 9 (25,7%) organizing pneumonia (OP) and 1 (2,9%) OP, transformed to diffuse alveolar damage. All pts had the standard examination including manual muscle testing (MMT), creatinkinase (CK) anti-Jo-1 antibodies (anti-Jo-1) assay;forced vital capacity (FVC) and carbon monoxide diffusion capacity (DLCO) evaluation as well as high-resolution computed tomography (HRCT) scanning of the chest were performed at baseline, and 36 and more months. The median disease duration was 3.2 [0.16-18] years, 21 (60%) of pts were positive for a-Jо-1 antibody. All pts received prednisolone at a mean dose of 24.3±13 mg/day, immunosupressants at inclusion received 25 (71%) pts: cyclophosphamide 18, mycophenolate mofetil 6 and comdination 1;Rituximab (RTX) was administered in case of severe course of disease and intolerance or inadequate response to GC and other immunosuppressive drugs. RESULTS: The mean follow-up period after the first infusion of RTX was 47.2±11.9 months. Pts received 1-11 courses of RTX. The cumulative mean dose of RTX was 4.6 ±2.5g. MMT 8 increased from 135.8±13.5 to 148.75±3.5 (p=0.000001). CK level decreased ΔCK – 762 u/l(median 340;25th% 9;75th% 821). anti-Jo-1 decreased from 173.4±37 to 96.5±79 u/ml (p=0.00002), FVC increased from 82±22.6 to 96,9±22% (p=0.00011). DLCO increased from 51.4±15.2 to 60±77.8% (p=0.0001). The mean prednisone dose was reduced from 24.3±13 to 5.7±2.4 mg/day. 3 pts died: ILD progression was the cause of death in 1 case, 1 bacterial pneumonia and COVID19 pneumonia. CONCLUSIONS: The results of this study confirm the positive effect of RTX in IIM patients with ILD (increase of muscle strength and improve lung function, decrease in anti-Jo-1 levels) and also its good steroid-sparing effect. CLINICAL IMPLICATIONS: RTX could be considered as an effective drug for the complex therapy of IIM patients with ILD when standard therapy is ineffective or impossible DISCLOSURES: No relevant relationships by Lidia Ananyeva No relevant relationships by Maria Aristova No relevant relationships by Oxana Desinova No relevant relationships by Liudmila Garzanova No relevant relationships by Anna Khelkovskaya-Sergeeva No relevant relationships by Olga Koneva No relevant relationships by Dmitry Kulikovsky

A CASE OF ANTISYNTHETASE SYNDROME: THE IMPORTANCE OF HISTORY IN DISTINGUISHING ETIOLOGY OF INTERSTITIAL LUNG DISEASE IN A COVID-19 WORLD

SESSION TITLE: Systemic Diseases Causing Pulmonary Havoc SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: In the coronavirus disease 2019 (COVID-19) era, the etiology of interstitial lung disease (ILD) should remain broad to ensure accurate diagnosis and the proper treatment of patients. Vital to the art of medicine is taking a comprehensive history, and anchoring on a common diagnosis such as COVID-19 can result in early dismissal of alternate etiologies that physicians have an obligation to explore. CASE PRESENTATION: A 58-year-old male with a history of diabetes, hypothyroidism, and hypertension presented to the emergency department (ED) with dyspnea and fever. Initial CT chest imaging was significant for reticular and fibrotic changes with peripheral ground-glass and solid nodular opacities, some with areas of central clearing. Despite negative PCR testing, he was diagnosed with COVID-19 and discharged on oxygen with pulmonary follow-up. He continued to have arthralgias, proximal muscle weakness, low-grade fevers, and weight loss. He re-presented to the ED and was admitted for hypovolemia and further exploration into a potential autoimmune etiology of his symptoms. Labs were significant for a creatine kinase of 3,381 U/L, positive autoimmune antibodies [ANA (1:320), Jo-1 (>8.0 U), and SS-A/Ro (1.4 U)], and elevated ESR and CRP (30 mm/hr and 81 mg/L). Repeat CT revealed persistent parenchymal changes. Bronchoscopy was performed without anatomical abnormalities, and bronchoalveolar lavage (BAL) fluid was normal in appearance and negative for infectious etiologies. Though the patient was a farmer and possessed risk factors for hypersensitivity pneumonitis, lack of lymphocytic predominance on BAL, negative hypersensitivity panel, and uncharacteristic CT findings helped exclude this diagnosis. The patient was diagnosed with antisynthetase syndrome and treated with pulse dose intravenous solumedrol before transitioning to prednisone with resolution of muscle weakness and radiographic improvement in lung infiltrates. Muscle biopsy was deferred given the rapid clinical response and serum markers consistent with the diagnosis. DISCUSSION: Antisynthetase syndrome is a rare cause of ILD and often presents with myositis, arthritis, skin changes, Raynaud's phenomenon, and fever [1]. These symptoms, combined with the aminoacyl-tRNA synthetase antibody—most commonly the Jo-1 antibody—help confirm the diagnosis [2]. Due to a lack of established diagnostic criteria, muscle biopsy is often used to exclude other causes of myositis [3]. The ILD associated with antisynthetase syndrome is a significant cause of morbidity and mortality, and delay in diagnosis can lead to progression of lung injury. CONCLUSIONS: Chest imaging findings in COVID-19 are nonspecific, and post-COVID lung disease often presents similarly to other ILDs [1]. Because of this, history and physical exam remain crucial tools to reflect on alternate diagnoses for ILD and will continue to be necessary as we evolve through this COVID-19 era. Reference #1: Devi HG, Pasha MM, Padmaja MS, Halappa S. Antisynthetase Syndrome: A Rare Cause for ILD. Journal of Clinical and Diagnostic Research : JCDR. 2016;10(3):OD08. doi:10.7860/JCDR/2016/16872.7361 Reference #2: Cavagna L, Trallero-Araguás E, Meloni F, et al. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course. Journal of Clinical Medicine. 2019;8(11). doi:10.3390/jcm8112013 Reference #3: Schmidt J. Current Classification and Management of Inflammatory Myopathies. J Neuromuscul Dis. 2018;5(2):109-129. doi: 10.3233/JND-180308. PMID: 29865091;PMCID: PMC6004913. DISCLOSURES: No relevant relationships by Dustin Norton No relevant relationships by Alyssa Simon No relevant relationships by Kang Rui Xiang

DERMATOMYOSITIS AFTER COVID-19 PNEUMONIA

SESSION TITLE: Unique Inflammatory and Autoimmune Complications of COVID-19 Infections SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/19/2022 12:45 pm - 1:45 pm INTRODUCTION: Viral infections can induce an immune cascade which may incite varied autoimmune disease to take action. One such disease is dermatomyositis, a rare inflammatory disease with multisystemic involvement. As we enter the post pandemic era, several unique complications related to COVID-19 are now surfacing. Here we present a case of a 57-year-old female who developed dermatomyositis after recent Covid-19 infection. CASE PRESENTATION: Patient is a 57-year-old female who presented to our pulmonary clinic with complaints of cough and shortness of breath (SOB). Patient reported feeling ill since contracting COVID-19 in November 2020. Two months after being diagnosed with COVID-19 she started to experience generalized muscle and joint pain;she underwent extensive rheumatological workup which was consistent with Sjogren disease for which she was started on hydroxychloroquine. A month after initiation of medication she started to experience worsening cough and SOB and underwent pulmonary function testing (PFT) in our clinic which showed evidence of restrictive lung disease. Chest CT was consistent with interstitial changes, therefore a diagnosis of ILD (interstitial lung disease) due to connective tissue disorder was made. Further assessment revealed positive CPK and anti-Jo1 antibodies indicative of dermatomyositis as a cause of her ILD. She was started on oral steroids which helped improve her symptoms. DISCUSSION: Several viruses including EBV, Hepatitis C, Rubella, HTLV-1 and Parvovirus have been associated with development of autoimmune diseases. Viral infections, like COVID-19 have shown to trigger an intense immune response which in turn may lead to autoimmune activity against host antigen. SARS-CoV2 has been found to enter muscle cells through ACE-2 receptors, allowing for transfer of genetic material and skeletal muscle damage. Another proposed mechanism of COVID induced myopathy has been T-cell clonal expansion by the virus up regulating TLR4 receptors increasing expression of ACE2, therefore facilitating entry of viruses leading to further inflammation. Identification of very specific T cell receptor epitopes for SARS-COv2 in patients with dermatomyositis has suggested COVID-19 as a trigger for CD8-T cells which leads to dermatomyositis in these patients. Autoimmune reactions occur from varying mechanisms like epitope spreading and bystander activation to molecular mimicry triggered by viral infections. CONCLUSIONS: SARS-COv2 presented with several challenges in the field of medicine. As we enter the post COVID period in medicine, we will continue to face several challenges proposed by the inflammatory surge caused by this disease. It is therefore important clinicians recognize and report these rare cases to increase awareness regarding several post covid diseases. Reference #1: HUSSEIN, H. M.;RAHAL, E. A. The role of viral infections in the development of autoimmune diseases. Critical Reviews in Microbiology, [s. l.], v. 45, n. 4, p. 394–412, 2019. DOI 10.1080/1040841X.2019.1614904. Disponível em: https://search-ebscohost-com.proxy.lib.wayne.edu/login.aspx?direct=true&db=a9h&AN=138199390&site=ehost-live&scope=site. Acesso em: 4 abr. 2022. DISCLOSURES: No relevant relationships by Kevser Akyuz No relevant relationships by Ranim Chamseddin No relevant relationships by Padmini Giri No relevant relationships by verisha khanam No relevant relationships by Emad Shehada No relevant relationships by Abdullah Yesilyaprak

A CHALLENGING DIAGNOSIS OF CELLULAR NONSPECIFIC INTERSTITIAL PNEUMONIA (NSIP) WITH ORGANIZING PNEUMONIA IN A PATIENT WITH ANTI-JO 1 NEGATIVE DERMATOMYOSITIS

SESSION TITLE: Autoimmune Diffuse Lung Disease Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Nonspecific interstitial pneumonia (NSIP) is an interstitial lung disease (ILD) that can be idiopathic or associated with connective tissue disorders (CTDs). The two subtypes of NSIP are cellular interstitial pneumonitis (CIP) and fibrotic, with CIP being less common. Subtypes can be distinguished through pathology and imaging. NSIP constitutes 14-36% of cases of idiopathic interstitial pneumonias. ILD-associated DM has a higher mortality, making diagnosis crucial. In specific, fibrotic NSIP has a high 10-year mortality rate, making differentiation relevant. CASE PRESENTATION: A 36-year-old female presented with fatigue and erythematous papular lesions on her face, palms, shoulder, and neck for one month. She also developed a fever and dry cough a week before. She denied recent travel or sick contacts. COVID-19 was negative. On exam, she was tachypneic and tachycardic with a maculopapular rash. A pulmonary exam revealed bilateral fine crackles. CXR showed dense left and mild right-sided patchy consolidations. Labs revealed elevated inflammatory markers (ESR-63, CRP-1.9, LDH-982). CPK was high - 517. CBC and procalcitonin were normal. CT showed extensive patchy and confluent areas of opacification of the left lower lobe, including a mass-like area measuring 3.3cm. Infectious workup was negative. Autoimmune testing ( Anti-Jo 1 Ab, ANA, etc) was negative. Bronchoscopic left lower lobe biopsy showed cellular interstitial inflammation composed of lymphocytes, plasma cells, rare eosinophils, and foci of intra-alveolar fibrinous exudates, suggestive of CIP and OP. She was treated successfully with corticosteroids and was discharged on prednisone. Repeat autoimmune antibody workup was negative. Skin biopsy showed a lichenoid lymphocytic infiltrate and necrotic keratinocytes consistent with dermatomyositis. Mycophenolate and rituximab were initiated;prednisone was tapered off. Follow-up chest CT showed cleared infiltrates with symptomatic improvement. DISCUSSION: CIP is an uncommon form of NSIP. On CT, bilateral ground-glass opacities are the most common feature. CIP is characterized histologically by interstitial thickening due to the presence of inflammatory cells and type-II pneumocyte hyperplasia with preserved lung architecture. Treatment is corticosteroids. The prognosis is excellent. ILD associated with DM is strongly associated with a positive Anti-Jo Ab, which was negative here making diagnosis challenging. She was diagnosed with dermatomyositis using histological findings from a skin biopsy. She responded to steroids at acute presentation and treatment was tailored once DM was diagnosed leading to complete recovery. CONCLUSIONS: ILD is not uncommon in CTD, however it is usually associated with a positive Anti-Jo 1 antibody. Our case is unique as the patient had negative Anti- Jo 1 Ab, however was found to have cellular NSIP with DM responding well to treatment following diagnosis. Reference #1: https://ard.bmj.com/content/63/3/297 Reference #2: https://www.ncbi.nlm.nih.gov/books/NBK518974/ Reference #3: https://pubmed.ncbi.nlm.nih.gov/33916864/ DISCLOSURES: No relevant relationships by Nawal Ahmed No relevant relationships by TAIKCHAN LILDAR No relevant relationships by Namratha Shripad No relevant relationships by David Wisa

RHABDOMYOLYSIS AS A VARIANT OF COVID-19 MYOSITIS

SESSION TITLE: Management of COVID-19-Induced Complications SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/19/2022 12:45 pm - 1:45 pm INTRODUCTION: Myalgias are one of the most common manifestations of a COVID infection. Myositis is much less reported with the spectrum of presentation ranging from asymptomatic elevation of creatinine kinase (CK) to rhabdomyolysis. Further understanding is required to formulate evidence based protocols for management and prognostication. CASE PRESENTATION: A 25-year-old male smoker, unvaccinated for COVID presented to the hospital with fever, weakness and myalgias and tested positive for COVID. Examination showed mild tenderness in the proximal muscles of the lower extremities. Labs were significant for metabolic acidosis, hypocalcemia, hyperkalemia, acute kidney injury, AST 6178, ALT 1340, CK > 36000 and CPK > 60000 and gross hematuria. Electrolyte abnormalities were corrected and he received aggressive hydration with intravenous fluids containing bicarbonate. Oxygen requirements increased and he received dexamethasone and Baricitinib for COVID. His creatinine continued to increase despite downtrending transaminases and CK. Ultrasound liver was normal. He developed bilateral pleural effusions and mild ascites suspected secondary to volume overload in the setting of acute renal failure. Hemodialysis was initiated and he received a total of 6 sessions of hemodialysis over the next week. Creatinine, BUN and GFR significantly improved. AntiJo1 Ab ordered to rule out polymyositis was negative. Transaminitis and raised CK levels downtrended alongside the COVID inflammatory markers and oxygen requirements as the patient was weaned to room air. DISCUSSION: The spectrum of COVID myositis reported thus far covers asymptomatic elevation of muscle enzymes, myasthenia, paraspinal myositis, dermatomyositis and rhabdomyolysis (1). The pathophysiology of COVID myositis has been hypothesized to be through ACE2 receptor mediated viral entry into muscle fibers leading to activation of innate and adaptive immunity. Other proposed mechanisms include the release of inflammatory cytokines and molecular mimicry with cross reaction of the antiviral antibodies. Myositis was most reported most commonly among males aged 33–87 (1). Symptoms when present include fevers, cough, shortness of breath, myalgias and proximal, lower limb–dominant, acute, and symmetric weakness. Peak CK values as high as 33,000 U/L have been reported (2). In general, patients diagnosed with rhabdomyolysis appear to have negative myositis-specific autoantibodies and higher CK levels than those without, highlighting the need for close monitoring of CK levels. Rhabdomyolysis associated fatality was reported to be as high as 45% (4 of 9 reported) over a short follow-up duration (1). Our case documents a recovery period in days-weeks with hydration and hemodialysis (3). CONCLUSIONS: Areas for exploration include factors predisposing patients to rhabdomyolysis, utility of checking enzyme levels and impact of vaccination on disease severity. Reference #1: Saud A, Naveen R, Aggarwal R, Gupta L. COVID-19 and Myositis: What We Know So Far. Curr Rheumatol Rep. 2021 Jul 3;23(8):63. doi: 10.1007/s11926-021-01023-9. PMID: 34216297;PMCID: PMC8254439. Reference #2: Husain R, Corcuera-Solano I, Dayan E, Jacobi AH, Huang M. Rhabdomyolysis as a manifestation of a severe case of COVID-19: A case report. Radiol Case Rep. 2020 Jul 7;15(9):1633-1637. doi: 10.1016/j.radcr.2020.07.003. PMID: 32690987;PMCID: PMC7340044. Reference #3: Byler J, Harrison R, Fell LL. Rhabdomyolysis Following Recovery from Severe COVID-19: A Case Report. Am J Case Rep. 2021 May 8;22:e931616. doi: 10.12659/AJCR.931616. PMID: 33963170;PMCID: PMC8127859. DISCLOSURES: No relevant relationships by Asim Amjad No relevant relationships by Sarasija Natarajan No relevant relationships by Pius Ochieng No relevant relationships by Yamini Patel

Myositis and Myocarditis With Anti- Jo-1 Antibodies Following SARS-CoV-2 mRNA Vaccination Or COVID-19 Infection

Background: Myocarditis without myositis has been described following mRNA SARS-CoV-2 vaccination. The literature on post-vaccine antibody mediated myositis is limited and to date no case series have been reported with a distinct clinical syndrome and a single myositis specific antibody, related to SARS-CoV-2 mRNA vaccination or COVID-19. Over a 6-month period in 2021, 54 patients were referred to our tertiary referral centre for suspected myositis. Out of 25 patients with a diagnosis of myositis, we identified three patients with a distinct clinical syndrome with myositis and myocarditis with anti-Jo-1 antibodies, following SARS-CoV-2 mRNA vaccination (BNT162-Pfizer-BioNtech;n=2) or following a mild COVID-19 infection (n=1). Results: Three patients (one woman, two men;49, 50 and 58 years old) developed progressive muscle weakness and muscle pain following either vaccination (patient 1 and 2) or mild COVID-19 infection (patient 3). Patients 2 and 3 had a history of anti- CCP positive rheumatoid arthritis (RA), which had been untreated for three years in patient 2. Both post-vaccine cases had severe pitting edema of the legs, patient 2 also had arthritis. None of the patients had mechanic's hands, Raynaud's phenomenon, or interstitial lung disease (ILD). The time interval between the SARS-CoV-2 trigger and the onset of progressive muscle weakness was between 10 and 14 days (patient 1 and 3) and was estimated cybetween three and seven days in patient 2. Laboratory tests showed highly elevated CK levels (17-32 times upper limit of normal (ULN)) and troponine T levels (14-34 times ULN). In patient 2, in addition, troponin I was tested (42 times ULN), which is more specific for myocardial involvement. In patient 1 supraventricular tachycardia, unspecific ST- and Twave abnormalities and elevated NTproBNP were found. In all patients, testing for myositis specific antibodies (MSAs;EUROline myositis 16 Ag. lineblot assay) showed anti-Jo-1 antibodies (semi-quantitatively in the highest possible range). Muscle MRI showed widespread muscle edema in all patients and extensive fascial and subcutaneous edema in the legs in the post-vaccine cases (figure 1). Muscle biopsies showed inflammatory myopathy. Cardiac MRI showed abnormalities in all patients: Pericardial effusion and/or late contrast enhancement of the epicardial myocardium (figure 1). All patients showed major improvement in response to immunosuppressive therapy and could discontinue highdosed steroids after three and six months. Discussion: In conclusion, we report three patients with a distinct clinical picture of anti-Jo-1 myositis and myocarditis without ILD, following SARS-CoV-2 mRNA vaccination or COVID-19. Although it is difficult to determine a causal relationship between SARS-CoV-2 and anti-Jo-1 myositis based on these small numbers, we suspect a SARSCoV- 2 trigger of anti-synthetase syndromes given the typical combination of symptoms and previously demonstrated association with antecedent viral infections. In addition, we have collected nationwide data on myositis specific antibodies (MSAs) in 2019 (pre- COVID-19) and 2021 (during COVID-19) from six medical centers in the Netherlands. We are currently analysing these data to examine whether the proportion of positive MSAs in 2021 is higher as compared to 2019. The results will be presented at the ICNMD.

THE EFFECT of COVID-19 PANDEMIC on IDIOPATHIC INFLAMMATORY MYOSITIS PATIENTS-A SINGLE CENTRE'S EXPERIENCES

Background: Pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-Cov2/COVID19) determines the life of clinicians and patients since 2 years. Limited information is available on the nature, prognosis, and complications of SARS-Cov2 virus infection in patients with idiopathic infammatory myopathies (IIM). There are also few data on triggered humoral response, side effects and disease course after COVID19 infection or vaccination in IIM. Objectives: The primary goals of the current research were to assess frequency and outcome of COVID-19 disease and to determine the vaccination rate and effect in our IIM cohort. Secondary objectives were to search for risk factors of infection, predictive factors of hospitalization and to assess incidence of vaccination adverse events, complications and post vaccination breakthrough infections. Methods: We retrospectively identifed the confrmed COVID19 positive patients and assessed the symptoms, disease course and outcome on 01/06/2021 then patients were prospectively followed. Incidence and complications of vaccination were determined by questionnaires. Anti-SARS-CoV-2 S enzyme electrochemilu-minescent immunoassay has been used to assess seroconversion, which measures total antibody (IgM and IgG) to the SARS-CoV2 S protein and SARS-CoV2 N protein. Disease activity was determined by physician global activity. Results: A total of 176 patients were screened and 101 participated in the study. By 01/06/2021, the COVID infection rate was 34,7% (mean age: 49.54 years, 72.72% women), which was signifcantly higher than the national prevalence at that time (8.2%). A third of these infections occurred asymptomatically or mild but 20% of the infected patients were hospitalized, one patient died. Longer disease duration (8.67 vs. 17.87 years;p=0.003) and higher incidence of anti-Jo-1 antibody (57% vs. 10% p=0.018) were signifcantly associated with hospitaliza-tion. All of COVID infected patients became seropositive regardless of immu-nosuppressive therapy or symptoms severity. 53,4 % of the patients received anti-COVID19 vaccine, 75,9 % choose the mRNA type. The titer of antibodies against the spike protein induced by vaccines showed high variance, but 72,3% of patients became seropositive after vaccination. Higher antibody titer against spike protein was detected after Pfzer-BioNTech vaccination compared to others (177,1 U/ml vs. 81.1 U/ml;p <001). Patients receiving steroid therapy had decreased post-vaccination antibody response compared to those without steroid treatment (94,03 U/ml vs. 165.6 U/ml;p = 0.008). With the follow-up of vaccinated patients, we did not found short term vaccine related major adverse events, but long term data revealed 7, 4 % post vaccination disease relapse. Breakthrough infection was detected in 9.25 % of the vaccinated patients, one cancer associated patient without post vaccination seroconversion died due to COVID pneumonia. All the fatal COVID infections occurred in patients with seron-egativity to anti-SARS-CoV2 S protein. Conclusion: Based on our results, myositis may be associated with an increased risk of infection with SARS-CoV-2. Independent risk factor for hospitalization is anti-Jo1 positivity and longer disease duration. Anti-SARS-CoV2 vaccines are safe, tolerable and strongly recommended for IIM population, but further investigation is required to assess clinical signifcance of post-vaccination disease fare.

Scleroderma Renal Crisis and COVID—Is There an Association?

Scleroderma renal crisis (SRC) is a rare but potentially devastating complication of systemic sclerosis as it is associated with significant morbidity and mortality. We present an interesting case of a patient who developed SRC following infection with COVID-19. A 37-year-old female presented with new-onset hypertension, AKI, anemia and thrombocytopenia. She had a history of diffuse cutaneous systemic sclerosis diagnosed 8 years ago, that had been well controlled with immunosuppression. The patient had contracted COVID-19 infection about 2 weeks ago but had remained largely asymptomatic except for a sore throat. Urinalysis revealed sub-nephrotic proteinuria but was otherwise bland. Peripheral blood smear was notable for 12-15 schistocytes per HPF. ADAMTS13 and complement levels were normal. Serologies for ANA, ANCA, anti-Scl70, anti-Jo1, anti-Sm, lupus anticoagulant, anti-beta2-glycoprotein I, anti-RNA polymerase III, RF, cryoglobulin, RPR, hepatitis and HIV, all returned negative. Renal biopsy revealed an arterial predominant thrombotic microangiopathy (TMA) (Figure) consistent with a diagnosis of SRC. The patient was treated with anti-hypertensives including an ACE-inhibitor, but her AKI continued to worsen, ultimately leading to dialysis dependence. SRC classically develops in patients with early or progressive diffuse cutaneous disease or positivity for anti-RNA polymerase III antibodies. Our patient did not have any such risk factors and rather developed SRC following infection with COVID-19. COVID-19 has been reported to cause TMA by inducing immune dysregulation via an overactive complement system. It is plausible that infection with COVID-19 triggered an exaggerated immune response, in turn leading to the development of SRC in our patient. COVID-19 may trigger SRC in patients with systemic sclerosis in the absence of other risk factors. (Figure Presented)

NOT EVERYTHING IS COVID

CASE: 45-year-old African American female with history of hypertension, hypothyroidism and prior tobacco abuse was admitted to hospital with shortness of breath and hypoxia. She was diagnosed with COVID-19 pneumonia due to her respiratory symptoms, CT scan findings of bilateral pulmonary infiltrate and positive COVID IgG antibodies although a PCR test was negative. The patient was discharged and later seen in pulmonary clinic where on further questioning she complained of fatigue, bilateral wrist and knee pain, and exertional dyspnea. On auscultation, bilateral rales were noted in the lower lung fields. She was noted to desaturate upon ambulation. PFTs (pulmonary function tests) revealed severe restrictive spirometry and severe gas transfer defect. A HRCT revealed bilateral infiltrates suggestive of organizing pneumonia. CPK was elevated at 449. Serologies were positive for ANA and antijo1 and negative for other connective tissue diseases. The patient was diagnosed with anti-synthetase syndrome (ASS). She was treated with oxygen, steroids and tacrolimus with reported improvement in her symptoms. IMPACT/DISCUSSION: ASS is a rare chronic systemic autoimmune disorder that predominantly affects females with a median age of 50. It is characterized by autoantibodies against aminoacyl-tRNA synthetase enzyme. The role of these autoantibodies in the development of ASS is not fully understood. Several autoantibodies have been identified including anti-Jo1, anti-EJ, antiOJ, anti-PL7, anti-PL12, anti-SC, anti-KS, anti-JS, anti-HA, anti-YRS. Among them, anti-Jo1 is the most common. The ASS is characterized by myositis, interstitial lung disease( ILD), arthritis, fever, Raynaud's phenomenon, mechanic's hand plus positive serologic testing of the Anti- aminoacyl-tRNA synthetase enzyme. The majority of the patients with Anti- Jo-1 antibodies develop ILD. An organizing pneumonia pattern can be seen in the settings of connective tissue disease and is commonly found in those with the ASS. ILD may be the first manifestation of the disease. CONCLUSION: We present a case of a 45 year old female mistakenly diagnosed with COVID pneumonia who on further evaluation was found to have ILD secondary to Antisynthetase Syndrome, a form of inflammatory myositis. An organizing pneumonia pattern on HRCT can be found in many settings other than COVID pneumonia. Careful attention to the history, physical examination, lab findings and COVID test results remain important in identifying etiologies other than COVID 19 for a patient's respiratory symptoms during the pandemic. Delays in diagnosis can be quite harmful to patients.

A Case of Rapidly Progressive Interstitial Lung Disease Due to Antisynthetase Syndrome

Introduction: Antisynthetase syndrome is a rare autoimmune disease. Clinical characteristics include interstitial lung disease (ILD), myositis, Raynaud's phenomenon, mechanic's hands, and arthritis. The condition is characterized by antibodies targeting an aminoacyl transfer RNA synthetase. Compared to other inflammatory myopathies, there is a higher prevalence and increased severity of ILD. Case Report: A 34-year-old female with a history of polycystic ovarian syndrome presented with progressive dyspnea during her third trimester of pregnancy. Initial computed tomography angiography (CTA) chest showed widespread multifocal and multilobar ground-glass opacities and nodular areas of consolidation. COVID-19 testing was negative. She went into preterm labor and delivered her baby at 30 weeks. About 10 days after delivery, her respiratory symptoms worsened. Transbronchoscopic lung biopsy was nondiagnostic. She subsequently underwent surgical lung biopsy which revealed organizing pneumonia and interstitial fibrosis. Laboratory studies revealed a high Jo-1 antibody of 1033U (normal less than 20U), positive ANA, creatine kinase 186 U/L, as well as aldolase 22.3 U/L leading to a diagnosis of antisynthetase syndrome. The patient continued to be dyspneic and developed increased oxygen requirements. Treatment was initiated with 1 dose of 125 mg of methylprednisolone followed by 1 g of methylprednisolone for 3 days, after which she was continued on oral prednisone. She was additionally started on 250 mg of mycophenolate mofetil. Despite these therapies she continued to have increased oxygen requirements, eventually requiring noninvasive positive pressure ventilation and ultimately intubation with mechanical ventilation. Chest x-ray demonstrated worsening bilateral patchy infiltrates. Given her clinical deterioration, she underwent 5 treatments of plasma exchange after which she received 1000 mg of rituximab. The patient improved on this therapy and was able to be extubated after 3 days. Her oxygen requirements subsequently decreased and she was discharged on a prednisone taper;mycophenolate with a goal dose of 1000 mg twice daily;and plan for continued rituximab infusions. At 2 months follow-up, the patient was doing well without the need for supplemental oxygen. Discussion: This case demonstrates a rare disease in a peripartum patient. A high suspicion for antisynthetase syndrome is required to initiate autoimmune testing, particularly since there can be ILD predominant phenotypes without significant evidence of a myositis. Treatment is not standardized but typically consists of corticosteroids and other immunosuppressive agents. In severe cases of antisynthetase syndrome that are refractory to initial corticosteroid therapy, therapeutic plasma exchange can be performed.

Antisynthetase Syndrome Post Shingrix and Pneumovax Vaccinations, Possible Correlation.

This is a case report of a patient who developed acute progressive shortness of breath that started two days following the administration of Shingrix and Pneumovax vaccinations. Eight days after the onset of his symptoms he was diagnosed with acute interstitial pneumonitis based on CT scan of the chest which later appeared to be consistent with the diagnosis of antisynthetase syndrome in light of findings consistent with mechanic's hands on examination, elevated Anti-Jo-1 antibody titers and aldolase on laboratory studies.

TRACKING DOWN AUTOANTIBODIES

Background/Aims Vaccine-associated autoimmunity is not infrequent, pertaining to either the cross-reaction between antigens or the action of adjuvant. This issue is more inexplicable to the COVID-19 vaccine, because of nucleic acid formulation and the hastened development process inflicted by the urgent pandemic condition. Here we are presenting a young patient who developed a significant abnormal autoimmune profile immediately post covid vaccination. Methods A 31-year-old IT engineer was referred to Rheumatology with postvaccine arthralgia. He had a history of recent aortic root aneurysm repair after having chest pain on exertion. Echocardiography showed dilated aortic root with significant aortic regurgitation, CT aortogram confirmed spiral type A dissection. He underwent an emergency cardiothoracic surgery in October 2020, followed by an uneventful recovery. He received the first dose of Pfizer COVID-19 vaccine on 2nd February, the very next day he developed painful ankles, knees, left hip, and right shoulder. Blood tests showed elevated CRP of 45, ESR 34, rheumatoid factor positive at 92, anti-CCP >340, ANA 13, ds-DNA 202, U1RNP positive, anti-SM antibody positive, Ro and La antibodies positive, antiJo1 antibody positive, with normal complements. He denied any swelling of the joints. No history of hair loss, photosensitive skin rashes, Raynaud's, sicca symptoms, oro-genital ulceration, or cracking of the skin. There were no constitutional symptoms, chest pain, or bowel issues. He was previously labeled as asthmatic, which is stable after surgery. He doesn't smoke or drinks alcohol. There was no family history of autoimmune conditions. On examination, he has tenderness across both hands and wrists with palmar erythema but no synovitis. He has painful right shoulder abduction with left hip pain on flexion and extension. Cardiovascular and GI examination was unremarkable apart from sternotomy scar and metallic valvular heart sounds. His dipstick urinalysis was negative for blood and protein. In recent x-rays hands and feet were normal. We agreed on a trial a tapering course of prednisolone started with 20mg daily. Three weeks later in follow-up, he reported partial response to steroids. His inflammatory markers were coming down. We have started azathioprine as a steroid-sparing agent. Results This gentleman with negative autoimmune screening prior to cardiothoracic surgery expressed florid newly detected autoantibodies straightaway after the COVID-19 vaccine. This is suggestive of undifferentiated connective tissue disease with the likelihood of overlap syndrome between rheumatoid arthritis and SLE. Conclusion COVID-19 vaccination showed a beacon of light to end the pandemic by achieving herd immunity. There is an excusable socioeconomic rush towards mass vaccination without long-term safety analysis, however, it is also crucial that any vaccine licensing process should entail meticulous scrutiny of the human proteome against vaccine peptide sequences. This will minimize the risks of acute autoimmune reactions to inoculation and future chronic autoimmune pathology.

The antisynthetase syndrome as a diagnostic challenge: A case report

Background: Idiopathic inflammatory myopathies are a heterogeneous group of pathological conditions characterized by muscle inflammation, and sometimes by inflammatory involvement of other organs, such as skin (in dermatomyositis) and lungs (pulmonary interstitial disease). Description of the case: 54-year-old woman, turns to the Emergency Room of Miulli Hospital in March 2021 for worsening dyspnea. In medical history: previous recent SARS-CoV-2 infection. She is admitted to Medicine Unit, where the characteristic signs and symptoms of myositis were objected: myasthenia affecting the proximal muscles, myalgias, plantar desquamation of the feet (climber's foot) and fingers (mechanic's hands), Gottron's papules. There are also: pericardial effusion, signs of pulmonary hypertension on echocardiogram, consolidating pulmonary parenchymal changes with fibrotic evolution on HR chest CT. She is transferred to the Rheumatology Unit of the Policlinico Di Bari to perform specific autoantibody panel (positivity of ANA, anti Jo- 1, anti Ro52), electromyography (signs of myogenic suffering), MRI of the thighs (hypotrophy and adipose replacement of the muscles of the posterior lodge) and muscle biopsy, indicative of muscle inflammation. Therapy with steroid boluses and cyclosporine 5mg/kg/day, shows quick effectiveness, and she is discharged with the diagnosis of antisynthetase syndrome. Conclusions: The diagnosis of antisynthetase syndrome is not always easy, due to the clinical heterogeneity of the disease and the need for specific instrumental and laboratory tests.