Clinical Outcome of Systemic Lupus Erythematosus during the Covid-19 Omicron Variantwave Compared to Previous Periods: Results from a Single Center Cohort of Puerto Rico

Objectives: Variant-related differences of SARS-CoV-2 have been reported such as higher transmissibility but less disease severity in omicron sublineages when compared to other variants. Although some studies have examined the outcomes of COVID-19 in systemic lupus erythematosus (SLE), most were conducted during the initial waves. Thus, we sought to compare the clinical outcomes of SLE patients with COVID-19 during the omicron and pre-delta/delta periods. Method(s): A cohort of adults with SLE from a single center in Puerto Rico was studied. SARS CoV-2 infection was confirmed by polymerase chain reaction or antigen tests. The pre-delta/delta variants period was defined as March 2020 to November 2021 and the omicron period as December 2021 to October 2022. Demographic parameters, cumulative SLE manifestations, disease activity, disease damage, lupus treatments, comorbidities, COVID-19 symptoms, SLE exacerbations, and hospitalizations were compared between the study periods using bivariate and multivariate analyses. Result(s): Of the entire SLE cohort (n = 347), 151 patients (43.5%) had COVID-19. In those with COVID-19, the mean (SD) age was 46.7 (12.5) years and 96.0% were women. Overall, clinical outcomes were favorable with low rates of hospitalizations (2.6%), lupus flares (3.3%), and mortality (0.7%). In 14.6% of cases, COVID-19 occurred during the pre-delta/delta period and in 85.4% during the omicron wave. Patients that had COVID-19 during the predelta/ delta period were younger and had a significantly higher proportion of oral ulcers, psychosis, anti-Smith antibodies, coronary artery disease, and chronic kidney disease compared to those during the omicron wave. Among COVID-19 symptoms, runny nose, cough, and sore throat were more common in the omicron period, whereas anosmia and anorexia were more frequent in the pre-delta/delta period. In the multivariable analyses adjusted by age, all variables retained significance except for psychosis, anti-Smith antibodies, and coronary artery disease. No significant differences were observed for other variables. Conclusion(s): In this group of Puerto Ricans with SLE, a higher proportion had COVID-19 during the omicron wave compared to previous periods. No differences were seen for severe outcomes such as hospitalizations, lupus flares, and mortality. Furthermore, COVID-19 did not appear to have a negative impact on the short-term clinical outcomes of these patients, regardless of the variant period examined.

Systemic lupus erythematosus in a 36-year old Filipino male after SARS-COV- 2 vaccination

Background: Systemic lupus erythematosus (SLE), a multisystem autoimmune disease more common in females, is associated with autoantibodies against different autoantigens forming immune complexes. Inadequate removal of these complexes from the host triggers inflammatory response which causes tissue damage. Some antiviral vaccines have been associated with the onset of SLE. Few cases of SLE occurring after SARS-CoV- 2 vaccines have been reported. Herein, we describe a case of new-onset SLE associated with COVID-19 vaccine. Case Summary: A previously well 36-year- old male with unremarkable family history of autoimmune disease started to develop muscle and joint pains, hair thinning, and ecchymoses 2 months after receiving second dose of inactivated SARS-CoV- 2 vaccine. He was subsequently admitted after consultation due to thrombocytopenia (platelet count of 58). He was given high dose steroid with tapering dose during the entire 14 days admission with significant increase of platelet count after 72 hours of repeat complete blood count. He went consult at rheumatology clinic a month after due to persistent joint and muscle pains, and progression of hair fall with associated facial rash, oral ulcers, easy fatigability and weight loss. Physical exam disclosed an ambulatory well-built male with normal vital signs, alopecia, malar rash, oral ulcers, joint tenderness and no objective muscle weakness. Complete blood counts and Anti-smith were within normal. Urinalysis, Antinuclear antibody (ANA), Anti-SSA, Anti-SSB, complement factor 3 (C3), and Anti-dsDNA were positive. He was managed with tapering prednisone and hydroxychloroquine with significant improvement at time of this report. Conclusion(s): Development of autoimmune reaction following COVID-19 vaccine has been described extensively;however, evidence of autoimmunity following vaccination seems to be lacking at present. Pathomechanisms include defective elimination and/or control of self-reactive lymphocytes resulting in over-stimulation of the immune system leading to clinical manifestations strikingly similar to the infection itself. Management approach to these autoimmune reactions address the immune hyper-stimulation with immunosuppressive or immuno-modulating agents including steroids and hydroxychloroquine.

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.

NEW-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS AND STEVEN JOHNSONS SYNDROME WITH SEVERE ARDS HERALDED BY POST-COVID-19 INFECTION MULTISYSTEM INFLAMMATORY SYNDROME IN AN ADULT (MIS-A)

SESSION TITLE: Autoimmune Diseases Gone Wild: Rare Cases of Pulmonary Manifestations SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 01:35 pm - 02:35 pm INTRODUCTION: Post-Covid-19 Multisystem Inflammatory Syndrome (MIS) is a severe hyperinflammatory syndrome associated with either the acute or recovery phase of covid-19 infection affecting multiple organ systems requiring hospitalization. This syndrome has been described in both children (MIS-C) and adults (MIS-A). Several case reports and systematic reviews have reported an association between post-covid-19 MIS-A and several autoimmune diseases. CASE PRESENTATION: We herein report a case of a 27-year-old female with no known chronic medical condition and a non-contributory family history who was diagnosed with post-covid-19 multisystem inflammatory syndrome in adults (MIS-A). She presented with generalized partial thickness erythematous skin ulcerations with tender blistering and painful erosion of her mucus membranes (oral and vaginal mucosa). This was diagnosed as Steven Johnsons syndrome. She was pulsed with intravenous methylprednisone. During this therapy, she progressed to severe acute respiratory distress syndrome (ARDS) requiring mechanical ventilation (fig 1). Bronchoscopy revealed mild pulmonary hemorrhage fig 2a&b). Serological testing heralded a new onset systemic lupus erythematosus in light of positive antinuclear antibodies, anti Ds DNA and anti Smith antibodies. Her course was complicated by significant proteinuria and an active renal cast suggestive of lupus nephritis. This necessitated further treatment for active lupus. She was successfully extubated and discharged home. DISCUSSION: We arrived at the diagnosis of post-covid-19 multisystem inflammatory syndrome in adults (MIS-A) in light of her presenting with fever, hypotension, persistent sinus tachycardia and new onset atrial fibrillation), acute pancreatitis, acute kidney injury, elevation in transaminases, new onset skin rash, elevated inflammatory markers and a recent history of positive SARS-CoV-2 infection. Covid-19 has been reported to induce wide spread vasculitis resulting in MIS-A or MIS-C by triggering type 3 hypersensitivity (1). Also, multiple case reports and systemic reviews have reported a direct association between MIS-A and several autoimmune diseases including SLE, SJS (2). The patient recovered with high dose corticosteroid and supportive therapy indicating her severe ARDS was most likely due associated to SJS, SLE and MIS-A. Clinicians should also keep in mind that SARS-CoV-2 PCR swab may be negative at the time patient presents with symptoms of MIS-A as the infection might have occurred about 4-5weeks prior just as in our patient(3) CONCLUSIONS: We cannot underscore enough the importance of clinicians having a high index of suspicion for this syndrome in patients with acute or recent covid-19 infection, with or without a positive PCR covid-19 test. Early involvement of a multidisciplinary approach and appropriate management is essential to mitigate morbidity and mortality in these patients. Reference #1: Roncati L, Ligabue G, Fabbiani L, Malagoli C, Gallo G, Lusenti B, et al. Type 3 hypersensitivity in COVID-19 vasculitis. Clin Immunol Orlando Fla. 2020 Aug;217:108487. Reference #2: Gracia-Ramos AE, Martin-Nares E, Hernández-Molina G. New Onset of Autoimmune Diseases Following COVID-19 Diagnosis. Cells [Internet]. 2021 Dec 20 [cited 2022 Mar 22];10(12):3592. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8700122/ Reference #3: Morris SB. Case Series of Multisystem Inflammatory Syndrome in Adults Associated with SARS-CoV-2 Infection — United Kingdom and United States, March–August 2020. MMWR Morb Mortal Wkly Rep [Internet]. 2020 [cited 2022 Mar 22];69. Available from: https://www.cdc.gov/mmwr/volumes/69/wr/mm6940e1.htm DISCLOSURES: No relevant relationships by Isaac Ikwu No relevant relationships by Anthony Lyonga Ngonge No relevant relationships by Alem Mehari No relevant relationships by Noordeep Panesar no disclosure on file for Vis al Poddar;No relevant relationships by Emnet Yibeltal

COVID-19 PNEUMONIA: A CAUSE OF CAVITARY LUNG LESIONS

SESSION TITLE: Pathologies of the Post-COVID-19 World SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Cavitary lung lesions are a relatively common finding on imaging with a vast differential diagnosis. CASE PRESENTATION: A 36-year-old female with history of gestational diabetes, preeclampsia and obesity presented to an outside hospital for evaluation of acute onset shortness of breath and chest pain. The patient tested positive for COVID19 two weeks prior. She initially developed some mild symptoms including headache, nasal congestion, generalized body aches, cough with mild sputum production and shortness of breath. Despite improvement in these symptoms, she suddenly developed chest discomfort that radiated to the back and was worse with inspiration and cough. The patient had been fully vaccinated against COVID-19 and had received a booster dose as well. Upon evaluation in the emergency room, she was afebrile, respiratory rate was 20, blood pressure was 144/90 mmHg, heart rate was 88 and her oxygen saturation was 99% on room air. Her physical exam was unremarkable. Basic laboratory work up including CBC and chemistry was normal. EKG and troponins were normal as well. A chest x-ay was performed which showed bilateral nodular densities with possible cavitation. A CT chest was later performed and showed multiple bilateral subsolid pulmonary nodules, some with apparent central cavitation. The patient was admitted for further work up at our institution. The patient's subsequent evaluation was largely unrevealing. An autoimmune panel testing for SLE, rheumatoid arthritis, ANCA vasculitis, and Goodpasture's syndrome revealed only a weakly positive ANA with negative anti-DNA and anti-Smith antibody. HIV and QuantiFERON tests were negative. Blood cultures were negative as well. Unfortunately, the patient was not able to expectorate and therefore no sputum cultures were obtained. Due to the patient's clinical improvement and absence of hypoxemia, diagnostic bronchoscopy was deferred, and the patient was subsequently recommended to undergo short interval chest imaging. Repeat chest computed tomography scan one month later showed complete resolution of the previously seen cavitary pulmonary nodules. At the time of outpatient clinical follow up, the patient remained asymptomatic from a respiratory perspective. DISCUSSION: Cavitary pulmonary nodules on chest imaging is an atypical presentation of COVID19 pneumonia that has been rarely described in the literature. In our patient, the temporal correlation between her pulmonary nodules and COVID19 infection as well as her negative work up for other common infectious and inflammatory causes known to cause cavitary lung lesions, makes COVID19 the most plausible cause for her findings. The pathophysiology of these findings remains unclear but may be explained by endothelialitis and small vessel vasculitis may be implicated in the formation of these lesions. [1] CONCLUSIONS: We presented a rare case of cavitary pulmonary nodules due to COVID19 pneumonia. Reference #1: Ackermann M, Verleden SE, Kuehnel M, Haverich A, Welte T, Laenger F, Vanstapel A, Werlein C, Stark H, Tzankov A, Li WW, Li VW, Mentzer SJ, Jonigk D. Pulmonary Vascular Endothelialitis, Thrombosis, and Angiogenesis in Covid-19. N Engl J Med. 2020 Jul 9;383(2):120-128. DISCLOSURES: No relevant relationships by Karim Anis No relevant relationships by Carolyn Garcia

Arterial Thrombosis as a Presentation of Systemic Lupus Erythematosusin a Young Child

Introduction: Systemic lupus erythematosus (SLE) is a rare diagnosis in children and can present with nonspecific symptoms. Similarly, arterial thromboses in children without risk factors is also rare. Together, they present a diagnostic challenge which may lead to a delay in proper management. Our objective is to emphasize the importance of having high suspicion for SLE in children, especially when presenting with arterial thrombosis. Case Description: Our patient is a 6-year-old female who presented with a 3-week history of severe right foot pain with a 1-week history of discoloration in the same foot. She sought care multiple times prior to presentation. She initially received the diagnosis of “COVID toes” despite a negative COVID PCR. Prior to presentation, she developed fevers, worsening foot pain, increasing discoloration, decreased oral intake, weight loss, and fatigue. Further lab work showed acute kidney injury, elevated inflammatory markers, and coagulopathy. She was also found to have elevated troponins and QTc prolongation. Additionally, a lower extremity Doppler demonstrated an acute partially occluding thrombus in her right popliteal artery. She was transferred to the pediatric intensive care unit (PICU) and started on a heparin infusion. An Echocardiogram with bubble study showed no interatrial shunting. Thrombolysis was considered but held due to concerns regarding thrombus chronicity. COVID-19 PCR and antibodies were negative, so Infectious Disease team determined this was unlikely related to an acute or remote COVID-19 infection or multisystem inflammatory syndrome in children (MIS-C). Her hemoglobin and platelets began to downtrend and Coombs was positive, raising concern for autoimmune hemolytic anemia. Autoimmune and coagulopathy work-up was significant for positive ANA titer, low complement levels and elevated anticardiolipin, anti-dsDNA, anti-Smith, anti-chromatin, and anti-RNP antibodies. She also had a chest X-ray that showed small non-infectious pleural and pericardial effusions suggestive of serositis. The constellation of these findings eventually led to the diagnosis of SLE. Discussion: There have been several cases of thrombotic or thromboembolic events reported in pediatric patients with SLE, with a majority being venous related events. Only two reports involving cerebral arteries have been published. For several of these patients, thrombosis was the presenting symptoms of their disease. In our literature review, we did not find any cases of arterial thrombosis outside the central nervous system related to SLE. Conclusion: Arterial thrombosis can be a presenting symptom for SLE in children. Despite being a rare presentation, rheumatologic diseases such as SLE should always be considered to prevent a delay in diagnosis and management. In our case, our patient experienced a notable delay in care due to a misdiagnosis related to the COVID-19 pandemic. While it is extremely important to consider sequelae of COVID-19, we would like to emphasize the importance of ensuring consideration of other diagnoses as well.

SLE After SARS-COV-2 Infection

SARS-COV-2 infection is often associated with exaggerated immune response, also referred to as a ‘cytokine storm’. There is growing concern that it may be linked to autoimmunity, with many cases of autoimmune diseases either triggered by or related to SARS-COV-2 having been reported, ranging from Guillain-Barre syndrome, Graves’ disease, multiple sclerosis, Kawasaki-like disease. Our patient was a 20-year-old female with a history of hidradenitis who presented with malaise, feet and ankle swelling, asthenia, anorexia, weight loss of 50 Ibs of 4 months. She had COVID pneumonia 7 months prior and was also seen in the ER thrice afterwards for ankle pain and fatigue managed with antibiotics and analgesics. Exam findings included tender bilateral lower extremity edema, diffuse hyperkeratotic and hyperpigmented purpuric rashes and bilateral suppurative axillary swellings. She was admitted for protein-energy malnutrition. Blood work showed WBC 13.5, low Hb 9.3, AST 509, ALT 104, BUN 29, Creatinine 0.9, Protein 7.5, albumin 1.5 (globulin gap of 6). Urine assay showed 3+ proteinuria Hb 3+ with RBC 3-10/hpf, absent nitrite, LE 1+, protein/creatinine ratio was 2949 mg/g. Blood cultures returned negative. US showed trace pericardial effusion and normal kidneys. Infectious workup returned negative for anti-streptolysin O, HIV, hepatitis B and C. Two days after, she developed AMS, fever, tachycardia and neck stiffness concerning for possible meningoencephalitis. CT head was normal. Lumbar puncture was performed. IV vancomycin and piperacillin-tazobactam was started. CSF fluid analysis revealed total protein of 125mg/dl, elevated IgG 79.8, concerning for an underlying inflammatory pathology. EEG was unremarkable. She became oliguric with creatinine and BUN both peaking at 2.6 and 58 respectively. Renal ultrasound revealed medical renal disease. Urine microscopy showed granular cast and no dysmorphic RBCs. ANA, anti-smith SSA, SSB, DS-DNA, RF, smooth muscle, anti-histone, anti-centromere, JO-1 and RNP antibodies were markedly elevated. She was unstable for CT trocar biopsy of the kidney. She subsequently went into cardiac arrest multiple times about a week into admission, before eventually expiring. Though causation was not established in our patient, SARS-COV-2 infection causing exaggerated immune response may unmask SLE or be associated with SLE.

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)

Subacute cutaneous lupus erythematosus after COVID-19 vaccination

Numerous cutaneous reactions have been reported secondary to COVID-19 vaccinations. The most commonly reported include local site reactions, delayed large local reactions, urticaria and morbilliform eruptions. Here we report a case of de novo subacute cutaneous lupus erythematosus (SCLE) after COVID-19 immunization. A 56-year-old woman presented with a 3-month history of a rash. The onset was 1 week following the first dose of the AstraZeneca COVID-19 vaccine. She reported lesions characterized by erythema, pruritus and a burning sensation. She also described mouth dryness. Examination revealed scaly annular erythematous plaques on the chest, arms, legs and scalp. Blood results were positive for anti-Ro antibodies and strongly positive for anti-nuclear antibodies (1: 2560 titre). Anti-Smith, anti-centromere and double- stranded DNA antibodies were negative. Skin biopsy revealed the histological appearance of an interface of dermatitis. Direct immunofluorescence was negative. These clinical and histopathological findings are consistent with a diagnosis of SCLE. The patient was treated with hydroxychloroquine, a weaning course of prednisolone, topical steroids and topical tacrolimus. Her hydroxychloroquine dose was 200 mg twice daily for the first 3 months and then increased to 400 mg twice daily. This resulted in an improvement of her presentation although she has yet to achieve complete remission. It has been suggested that enhanced interferon responses with COVID-19 vaccination and interactions of the SARS-CoV-2 spike protein with cytoplasmic RNA-binding proteins could contribute to disease flares in lupus. There are two other recent reports of SCLE developing or being exacerbated by COVID-19 vaccination. More research is required to determine how COVID-19 vaccinations affect patients with autoimmune skin diseases.

A Case of Transverse Myelitis After Moderna SARS-CoV-2 Vaccination

Objective: This case report describes a longitudinally-extensive transverse myelitis after Moderna SARS-CoV-2 vaccination. Background: Transverse myelitis (TM) is an inflammatory spinal cord syndrome presenting with acute-to-subacute neurological deficits. A lesion spanning three or more vertebral segments on imaging is considered “longitudinally-extensive.” The TM differential is broad-- among these etiologies, vaccination is a rare but recognized entity. Design/Methods: 60-year-old, right-handed man with chronic right hemisphere stroke with residual left hemiparesis admitted for four days of bilateral lower extremity numbness progressing to weakness and urinary and bowel incontinence 10 days after receiving his second Moderna SARS-CoV-2 vaccination. Examination showed hypotonic lower extremities, proximal greater than distal weakness, a T9 dermatome sensory level, perineal numbness, mildly-reduced rectal tone, and preserved reflexes. MRI spine revealed a longitudinallyextensive, non-enhancing T2-hyperintense lesion spanning T8-T12. CSF analysis demonstrated 5 white blood cells, 1271 red blood cells, 124 glucose, and 55 protein. Aside from mildly elevated ESR and CRP, extensive serum and CSF work-up for other causes of myelopathy, including nutritional/toxic (copper, zinc, heavy metals), infectious (RPR/VDRL, HIV, HTLV, HSV, VZV, West Nile), and rheumatologic (anti-Jo, anti-Mi-2, anti-Ro/La, anti-smith, anti-Scleroderma, anti-dsDNA, anti-ribosomal P, anti-RNP), were unremarkable. Anti-NMO and anti-MOG antibodies were negative. He improved with methylprednisolone 1000 mg daily for five days suggesting an autoimmune etiology. Results: NA Conclusions: Transverse myelitis has a broad presentation and differential, requiring detailed history-taking to determine the cause as management differs between etiologies. SARS-CoV-2 and post-vaccination are known etiologies for TM. Given the timing of our patient's symptom onset after vaccination and thorough exclusion of other causes, we postulate a potentially novel case of TM associated with the Moderna SARS-CoV-2 vaccine. Though post-vaccination myelopathy is potentially debilitating if untreated, it is rare, and the benefits of vaccination appear to outweigh the risks.

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.

LEUKOCYTOCLASTIC VASCULITIS IN MITRAL VALVE ENDOCARDITIS

Background: Vasculitis is a known, although not commonly observed, manifestation of bacterial endocarditis. It is imperative that diagnosis is made promptly and appropriately treated, as vasculitis can often be painful and uncomfortable for patients. Case: 75-year-old male is admitted to the hospital for Coronavirus Disease 2019 (COVID-19). Several weeks after recovering from his respiratory infection, patient developed a diffuse, purpuric rash that began on his forearms and gradually spread throughout his bilateral upper extremities to his hands and fingers, as well as to his shoulders and lateral chest. Skin biopsy was performed and revealed findings suggestive of leukocytoclastic vasculitis. Blood work revealed Methicillin Resistant Staph Aureus (MRSA) bacteremia, sensitive to Vancomycin. Transthoracic echocardiogram revealed native mitral valve endocarditis. Transesophageal echocardiogram was not performed due to patient's underlying comorbidities and high risk. Decision-making: Patient was diagnosed with leukocytoclastic vasculitis secondary to bacterial endocarditis. Rheumatologic workup, including antineutrophil cytoplasmic antibodies, antinuclear antibodies, serum complement levels, anti-smith antibodies and double stranded deoxyribonucleic acid, was negative. Patient was ultimately discharged on a prolonged course of Vancomycin and his diffuse rash resolved one month later. Conclusion: There are only a few case reports describing the direct association between leukocytoclastic vasculitis and infective endocarditis. It is important to consider the association of vasculitis and endocarditis in order to effectively treat because immunosuppression, particularly with steroids, is the gold standard treatment for vasculitis. Our patient experienced near complete resolution of the rash after completion of antibiotics and no other therapy was deemed necessary.

Identifying systemic lupus erythematosus presenting as hemorrhagic oral bullae in a covid positive pediatric patient

Case Report A previously healthy 13 year-old female presented with painful, oral mucosal bullae filled with sanguinous fluid. She was initially (mis)diagnosed with angina bullosa haemorrhagica (ABH) and was provided symptomatic treatment. After a CBC demonstrated severe thrombocytopenia, anemia, and leukopenia, the patient was admitted for further workup including Coombs and COVID-19 PCR, which were both positive. Given a remote family history of Lupus and increasing right knee pain, further diagnostic testing was ordered. These results demonstrated a positive ANA, anti-Smith, anti-chromatin, anti-RNP, increased dsDNA and increased SM/RMP, confirming Lupus as the etiology of this patient's presentation. A form of Blistering Systemic Lupus Erythematosus (BSLE) was likely responsible for the patient's oral manifestations. The patient was discharged on Prednisone 30 mg twice per day after receiving 60 g IVIG and 3 days of high dose pulse corticosteroids. Discussion This outlines the case of a thirteen yearold girl with SLE with an initial presentation of blood-filled oral mucosal lesions. The patient's COVID-19 positive status, young age, and atypical presentation added to the intricacy of her case. After presenting with blood-filled bullae in the oral cavity, the patient was initially suspected of having Angina Bullosa Haemorrhagica (ABH). ABH is a rare condition that presents with painful or painless blood-filled oral vesicles or bullae that rupture spontaneously and heal without scarring. The patient's abnormal CBC ruled out ABH and suggested a diagnosis of Evan's syndrome, a disorder in which cytopenias are present in two or more cell lines. Before SLE was determined to be the cause of the patient's cytopenias, the etiology of her Evan's syndrome was attributed to her COVID-19 positive status. Rarely, Lupus has been reported to present with vesicles and bullae in a syndrome known as BSLE. Upon an extensive review of the literature, only four articles mentioned oral bullae in a pediatric patient with SLE and not a single article mentioned hemorrhagic bullae in pediatric SLE patients. Conclusion A deeper understanding of the variety of cutaneous manifestations of SLE is essential for disease diagnosis and management. The present study details the first ever reported case of SLE presenting with blood-filled oral bullae in a pediatric patient. Novel presentations of SLE such as this reinforce the need for a collaborative, inter-specialty approach to diagnosis and treatment of autoimmune disease. This case reinforces the utility of a centralized database for recording unique autoimmune manifestations in order to aid in physicians' diagnosis and expediency of treatment. Lastly, this case should support an increase in a clinician's degree of suspicion for underlying autoimmune disease when dealing with unique cutaneous presentations of autoimmune diseases like SLE.

Not your typical presentation: A case of pediatric systemic lupus erythematous

Case Report Pediatric Systemic Lupus Erythematous (pSLE) is a multi-system autoimmune disease with varied clinical presentations. Although rare, it can be associated with significant morbidity and mortality. We report a case of a newly diagnosed pSLE patient who's presenting symptoms were concerning for viral pericarditis. Our 17-year-old African American female presented with non-radiating substernal chest pain and shortness of breath for three days. Pain was described as a constant pressure, exacerbated by lying down, and improved with leaning forward. Shortness of breath was most notable with ambulation. Other associated symptoms included subjective fever, chills, fatigue, two-week history of mild cough, intermittent headaches, nausea, emesis, pedal edema, myalgias, arthralgias, and a 10-pound weight loss. Detailed enquiry revealed a long-standing history of lymphadenopathy, neutropenia, microcytic anemia, and migraine headaches. Previous evaluation included normal thyroid studies, Hemoglobin A1c, complete metabolic panel, HIV testing, monospot, and a reassuring peripheral blood smear. Initial evaluation for her chest pain revealed normal EKG and chest x-ray. On physical exam at our facility, she was afebrile with normal vital signs. Soft, mobile, non-tender, <2 cm posterior cervical lymph nodes were palpated. Cardiac exam was unrevealing and no arthritis or rashes were noted. Her lungs were clear, but she had conversational dyspnea. Initial differential diagnoses included systemic processes such as rheumatologic, oncologic, and infectious. Testing revealed elevated troponin, proteinuria, pancytopenia, and elevated inflammatory markers. Coombs test and ACE were negative. D-dimer and creatinine kinase to evaluate for deep vein thrombosis and myositis were normal. EKG was concerning for ST elevation in anterolateral leads, and an echocardiogram revealed a small, globally distributed pericardial effusion. COVID PCR was positive concerning for pericarditis secondary to multisystem inflammatory syndrome in children (MIS-C). Though she met diagnostic criteria for MIS-C with her history of fever, elevated inflammatory markers, and multisystem involvement (cardiac and abdominal), the presence of her symptoms over several months was more concerning for a chronic process. Further evaluation into an underlying etiology revealed low C3/C4, positive ANA, positive ds-DNA, and positive SS-A, SS-B, chromatin, Anti-Smith, and RNP antibodies. The immunologic profile along with her clinical presentation was consistent with pSLE. Treatment with high-dose intravenous steroids resulted in complete resolution of her chest pain and she was discharged on oral hydroxychloroquine. pSLE is a multi-faceted and diagnostically challenging disease. Our case highlights the importance of obtaining a thorough history and a low threshold of suspicion for this complex autoimmune condition.

A rare case of cardiogenic shock due to lupus myocarditis in a patient with HIV

INTRODUCTION: Lupus Myocarditis is a rare and severe manifestation of systemic lupus erythematosus. We describe a patient with Human Immunodeficiency Virus (HIV) presenting with cardiogenic shock due to lupus myocarditis. DESCRIPTION: A 33 year old man with history of congenital HIV infection on anti-retroviral therapy, CD4 count 338/ mm3 and undetectable viral load, recurrent Pneumocystis jirovecii pneumonia, disseminated zoster and chronic kidney disease stage 3 presented with shortness of breath for 2 weeks and hypotension with cold extremities and leg edema. Transthoracic echocardiogram demonstrated acute severe biventricular dysfunction with ejection fraction of 10%. CXR showed ground glass opacities with bibasilar consolidation. He was subsequently intubated for acute hypoxic respiratory failure and admitted to the cardiac intensive care unit for management of cardiogenic shock mixed with sepsis due to presumed multifocal pneumonia. He was treated with high dose vasopressors, inotropes and empiric antibiotics. Infectious work up revealed methicillin-resistant Staphylococcus aureus (MRSA) in respiratory culture and negative viral infection including SARS-CoV-2. His course was complicated by worsening renal function with proteinuria and refractory metabolic acidosis required continuous venovenous hemofiltration and he suffered pulseless electrical activity (PEA) arrest with return of spontaneous circulation in 5 minutes. Coronary angiogram was normal. Auto-immune work up revealed elevated serologies: anti-Ds DNA >300 IU/ ml, Anti-Smith Ab: 1 (0-0.9 AI), Anti-chromatin Ab >8 (0 to 0.9 AI) with markedly low complement levels. Endomyocardial biopsy revealed lymphocytic infiltrate in endocardium and myocardium with no granulomas or thrombi. Based on these findings, he was diagnosed with lupus myocarditis and lupus nephritis. The patient clinically improved after treatment with pulse dose steroids and cyclophosphamide. His renal function recovered and cardiac function improved. He was weaned off from the ventilator and discharged to rehabilitation facility. DISCUSSION: Lupus Myocarditis requires urgent clinical attention as it may progress to heart failure and fatal cardiogenic shock. Early diagnosis with high index of suspicion and treatment with steroids and immunotherapy are the keys for better outcome.

Livedo reticularis as a late sign of COVID-19 temporally related multisystem inflammatory syndrome (MIS-C): A case series

Introduction: In children, the dermatologic features appear to occur early with other COVID-19 manifestations. Dermatologists play a key role in the early diagnosis of COVID-19. Multi-system inflammatory syndrome in children (MIS-C) shows a presentation mimicking Kawasaki Disease (KD), with mucocutaneous signs. However, late onset dermatological signs are poorly described. Objectives: to evaluate children with MIS-C during the follow-up and to describe late dermatological signs in these patients. Methods: We followed 14 children (3M;11 F) with MIS-C, with clinical, biochemical, imaging data. Autoantibodies, D-Dimer, CRP, ESR, C3, C4, ferritin, serum amyloid, IgA, IgM, IgG were detected 1-2 months after the resolution of the clinical manifestations of MIS-C. Results: 8/14 children (58%) showed livedo reticularis at the legs, arms, trunk. The livedo was more evident at the legs in all the patients. The livedo started at the remission, after normalization of CRP, ESR, D-Dimer;the sign lasted also for 1-2 months after the discontinuation of steroids and the normalization of haematochemical parameters. 4/8 showed low-title positive autoimmune tests (ANA in 2;ENA anti-Sm in 2;anti-cardiolipin IgG in 1;ASCA in 2). Conclusion: In our series, 8/14 patients showed a livedo reticularis, more marked in the legs, however in some cases with a wide distribution to arms and the trunk. Low-title autoantibodies were transiently positive in 50% of these cases, negative in later detections. Livedo reticularis was a late sign, linked to MIS-C related vasculitis, persisting 1-2 months after the resolution of MIS-C. A different treatment regimen (IVIG plus steroids at 1-2 or 30 mg/Kg/day) did not influence the progress of this clinical manifestation. In 50% of children we documented a transient autoimmune response.