LANGERHANS CELL HISTIOCYTOSIS IN A CHILD WITH MULTISYSTEMIC LESIONS ASSOCIATED WITH INFECTED POLIOMYELITIS AND SARS-COV-2, FEATURES OF DIAGNOSTICS. CLINICAL CASE.

Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells. The prognosis varies depending on the form of the disease and organ damage. Any organs and systems can be involved in the pathological process in various combinations. A poor response to standard therapy and an unfavorable prognosis are characteristic of patients with a multisystem form of LCH and involvement of organs at risk. Skin lesions are a classic sign of LCH. Purpose - to describe the complexity and duration of diagnosis of LCH with multisystem damage in a boy aged 2 years and 2 months, infected with poliomyelitis and coronavirus. Clinical case. The first clinical manifestations of LCH in the child debuted with an eczematous-seborrheic rash on the scalp with spread to the limbs and trunk. The child was treated for toxicoderma, hemorrhagic vasculitis at the place of residence for 6 months. The boy lost 1.5 kg of body weight in 1 month. At the time of hospitalization, seborrheic-eczematous rashes on the skin with a hemorrhagic component, trophic-inflammatory changes in the nails of the hands, signs of protein-energy deficiency, stomatitis, gingivitis, hepatosplenomegaly, polyserositis, diabetes insipidus, osteolytic foci of the frontal bones were found. Results of the tests: anemia, thrombocytopenia, hypoproteinemia and hypoalbuminemia, coagulation disorders. The patient had the onset of lower flaccid paraparesis, muscle hypotonia. The boy was diagnosed with a number of infectious complications, including poliomyelitis (a derivative of vaccine poliovirus type 2), COVID-19. The child received LCH-III cytostatic therapy with a positive effect. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies.Copyright © 2023 Institute of Physics of the Russian Academy of Sciences. All rights reserved.

Acute hemorrhagic edema of infancy in a 6 week old

Objectives: Uncommon presentations of common diseases present a challenge in recognizing the correct diagnosis. Beside uncommon symptoms, uncommon age of onset challenges the pattern recognition abilities of clinicians. Method(s): Here we present a 6 week old boy with acute haemorrhagic edema of infancy in association with COVID-19. The otherwise healthy term born infant presented in our clinic with fever, mild respiratory symptoms and a rash. After establishing a sufficient saturation of oxygen, also during sleep, the infant was discharged. Result(s): Complete resolution of the rash was within days after. On admittance 60 mg prednisolone rectal was applied by the emergency night shift staff also to stabilize a slight wheeze due to COVID-19 but other than that no therapy was needed. Discussion(s): Reviewing the literature the benign nature of this leucocytoclastic vasculitis was commonly reported as well as the common onset during late infancy (about 8 to 23 months). Very few reports target the age group outside this age brackets. Nonetheless it is important to think outside the box when examining a patient in emergency paediatric derm.atology.

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

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

Dermatomyositis with Specific Autoantibodies Appearing after Sars-Cov-2 Infection or Mrna Vaccination: Hints at a Shared Pathogenetic Mechanism

Background. Environmental factors such as infections and vaccines are known to trigger dermatomyositis (DM), and during the recent SARS-CoV-2 pandemic this has become even clearer. SARS-CoV-2 infection may share features with anti-MDA5 DM, such as rapidly progressive lung involvement, cutaneous lesions and cytokine release syndrome. A few case reports of DM following SARSCoV-2 vaccination have been published, suggesting the onset of an aberrant immune response leading to DM with specific autoantibody signatures and severe organ impairment. Methods. Clinical and laboratory data of the 2 case reports were obtained from electronic clinical charts in Humanitas Research Hospital (Rozzano, Milan, Italy). Autoantibody analysis was performed by protein-immunoprecipitation for anti-MDA5 and immunoblot for anti-Ro52 and TIF1gamma antibodies as per protocol. Results. Case report 1 is a 71-year-old woman who developed fever, cough, and anosmia, which resolved spontaneously in two weeks, but did not undergo a nasopharyngeal swab, while her relatives were diagnosed with SARS-CoV-2 infection. When symptoms improved, she developed arthralgia and skin lesions on her face, chest, and hands for which she started topical treatment, with negative SARSCoV-2 nasopharyngeal swab and positive serum test for IgG against SARS-CoV-2 spike protein. For the persistence of the skin rash and arthralgia, she was admitted to our Department in March 2021. Blood tests showed mild elevation of C reactive protein (2.1 mg/L -normal value NV<5), aspartate (84 UI/L) and alanine aminotransferase (133 UI/L -NV<35), ferritin (595 ng/ml -NV<306), troponin I (19 ng/L -NV<14), and BNP (251 pg/ml -NV<100) with normal complete blood cell count, creatine kinase, C3 and C4. IgG antibodies for SARS-CoV-2 spike protein were confirmed to be elevated (96 AU/ml -NV<15). Autoantibodies associated with connective tissue diseases were tested and only anti-MDA5 antibodies were positive at immunoprecipitation. A punch biopsy of a Gottron-like lesion on the left hand showed leukocytoclastic vasculitis. We observed reduced capillary density with neoangiogenesis and ectasic capillaries at the nailfold capillaroscopy. EKG and ecocardiography were normal, while cardiac magnetic resonance detected abnormalities in the parametric sequences, consistent with signs of previous myocarditis. A lung CT scan revealed pulmonary emphysema while respiratory function tests demonstrated reduced volumes (FVC 82%, FEV1 64%, inadequate compliance CO diffusion test). Based on the biochemical and clinical findings, a diagnosis of anti-MDA5-associated DM with skin and heart involvement was made and treatment with low-dose methylprednisolone (0.25 mg/kg daily) and azathioprine 100 mg was started, then switched to mycophenolate because not effective on skin lesions. Case report 2 is an 84-year-old woman with history of colon cancer (surgical treatment) and oral lichen treated with low doses steroids in the last 2 years. After the 2nd dose of SARS-CoV-2 mRNA vaccination, in March 2021 she developed skin rash with V-sign, Gottron's papules, periungueal ulcers, muscle weakness and fatigue, thus she performed a rheumatologic evaluation. Blood tests showed mild elevation of creatine kinase (484 UI/L, NV <167), CK-MB (9.6ng/ml, NV <3.4), BNP (215 pg/ml -NV<100) with normal values of complete blood cell count, C3 and C4. Anti-Ro52kDa and TIF1gamma were positive at immunoblot, thus we confirmed a diagnosis of DM. The clinical evaluation also showed active scleroderma pattern at nailfold capillaroscopy, normal echocardiography, bronchiectasia but not interstitial lung disease at lung CT, and normal respiratory function tests (FVC 99%, FEV1 99%, DLCO 63%, DLCO/VA 81%). A PET-CT scan was performed to exclude paraneoplastic DM, and treatment with steroids and mycophenolate was started. Conclusions. SARS-CoV-2 may induce mechanisms for escaping the innate immunity surveillance and causing autoimmune diseases, but more clinical and functional studies are needed to demonstrate this possible association.

'Breaking' news: A pediatric case of leukocytoclastic vasculitis after SARS-CoV-2 vaccination

Case report In May 2021, the European Medicines Agency (EMA) approved the administration of the mRNA BNT162b2 vaccine (Pfizer/BioNTech) in adolescents aged 12-15 years, in the form of a two-doses-primary course three weeks apart, with a booster dose after five months. Few adverse events have been observed in vaccinated children -mainly fever, myalgia, or local edema at the injection site. Conversely, delayed cutaneous reactions are little reported in adults and even rarer in pediatric age. A 12-year-old boy, with no previous cutaneous or autoimmune diseases nor allergic reactions to previous vaccines or drugs, came to our attention because of a cutaneous rash, which started on his right arm two days after his first dose of Pfizer/BioNTech vaccine. The rash was flat, erythematous, and purpuric, with subsequent bruise appearance and spontaneous resolution. Lesions took over on the front side of his arms and shoulders until a month after the vaccine. Urine and blood tests -including blood cell count, flow cytometry, plasma biochemistry, inflammatory index, autoantibodies, coagulation, and platelets aggregation test -did not show significant alterations. Grass pollen monosensitization was detected. Biopsy of the lesions showed 'modest perivascular lymphohistiocytic infiltrate and focal infiltration of vascular walls with swollen endothelium' with 'occasional eosinophils, scattered neutrophil granulocytes, and erythrocytes in interstitial areas,' compatible with leukocytoclastic vasculitis. Epidermidis was undamaged, and the direct immunofluorescence showed fibrinogen deposits in superficial-to-middle dermis vessels. Further tests highlighted a high immune response to the vaccine without previous Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection. No therapies were needed, and the boy experienced no other side effects undergoing his second dose. Few leukocytoclastic vasculitis cases after SARS-CoV-2-vaccination have been reported in adults, primarily flares of previously known vasculitis. A role for induced spike glycoprotein as a pseudovirion docking to specific vascular receptors has been suggested, too. To our knowledge, no similar cases were described currently, neither in such young patients nor with such atypical, focal lesions. Further studies are needed to identify the pathogenesis and possibly prevent the onset of this adverse reaction.

Leukocytoclastic vasculitis following immunization with COVID-19 vaccine

Case report Introduction: Vaccines have been recognized as agents associated with development of different forms of vasculitis. We describe the case of a leukocytoclastic vasculitis which developed after immunization with inactivated COVID-19 vaccine. Case presentation: A 68-years old female patient presented with painful purpuric papules and plaques, and areas of necrosis, dominantly localized on her feet (Figure 1), developing 12 days after she received first dose of inactivated COVID-19 vaccine (BBIBP Cor-V). There were no other symptoms except mild fatigue and low-grade temperature of 37.3degreeC. Skin biopsy showed damaged vessel wall with perivascular, neutrophilic inflammatory infiltrate, leucocytoclasia and erythrocytes extravasation (Figure 2). Thorough work-up, including broad immunoserological and virological analysis didn't reveal any other potential trigger. Three months before vaccination the patient recovered from COVID-19 pneumonia. After the four weeks course of prednisone (initially 20 mg/day) with tapering a complete resolution of skin changes was achieved. The patient was followed for one year with no relapses. Conclusion(s): COVID-19 immunization should be considered as a potential trigger for development of cutaneous vasculitis.

Cutaneous vasculitis after COVID-19 vaccination in a 41-year-old male.

Leukocytoclastic vasculitis could be a possible adverse event of different SARS-CoV-2 vaccines. Clinicians and manufacturers should be aware of this adverse event for appropriate diagnosis and treatment.

A Case of Iga Vasculitis That Developed Gross Hematuria and Lower-Limb Purpura Following Covid-19 Vaccines

Introduction: There have been some reports on flare-ups of kidney diseases following COVID-19 vaccines such as IgA nephropathy and minimal change disease. However, there have been few reports on those of IgA vasculitis following the vaccines yet. We report a case of IgA vasculitis with a flare-up of gross hematuria and lower-limb purpura following Moderna COVID-19 vaccines. Method(s): The patient is a 16-year-old female with no previous history of abnormal results of urinalyses before April in 2021. She had developed microscopic hematuria, proteinuria and purpura on both of her lower limbs that emerged and then disappeared repeatedly since then. She received Moderna COVID-19 vaccines in August and September in 2021, both of which were followed by gross hematuria lasting for around 10 days. The lower-limb purpura reemerged at the same time as the hematuria. Microscopic hematuria of around 30-49 RBC/HPF, glomerular hematuria of moderate degree and urine protein-to-creatinine ratio (UPCR) of around 0.8 g/gCr had continuously been detected. Skin and kidney biopsies were performed in December in 2021 and in February in 2022 respectively. Result(s): The skin tissue showed formation of leukocytoclastic vasculitis, and the kidney tissue showed that of cellular and fibrocellular crescents and endocapillary hypercellularity. Immunofluorescence staining of both tissues showed deposition of galactose-deficient IgA1(Gd-IgA1) and C3, and she was diagnosed as IgA vasculitis. She received steroid pulse therapy followed by tonsillectomy. The lower-limb purpura has disappeared after she received three courses of the steroid pulse therapy, but microscopic hematuria and UPCR of around 0.8 g/gCr have still continued. Conclusion(s): IgA vasculitis is leukocytoclastic vasculitis characterized by deposition of Gd-IgA1 on microvessel walls in skin and on glomerular capillaries in kidneys. The detailed mechanism of IgA vasculitis has not been fully elucidated yet. Gross hematuria following an upper respiratory infection is considered as a characteristic clinical symptom of IgA vasculitis as well as IgA nephropathy. Post-vaccination gross hematuria of patients with IgA nephropathy has been reported, and it is believed that innate immunity is related to its mechanism. Moderna COVID-19 vaccines, which the patient received, are mRNA vaccines. We estimate that exposure to the mRNA vaccine triggered excess glomerular deposition of Gd-IgA1-containing immune complexes and subsequent gross hematuria by overactivation of innate immunity such as Toll-like receptors that detect RNA. This case suggests that such immune activation by a mRNA vaccine might be related not only to the mechanism of IgA nephropathy but also to that of IgA vasculitis. No conflict of interestCopyright © 2023

A case report of IgA vasculitis following CoronaVac vaccination in a Filipino patient

Introduction: Coronavirus disease 2019 (COVID-19) pandemic is ongoing and new variants of severe acute respiratory syndrome coronavirus type 2 (SARS-CoV- 2) are emerging. There is an urgent need for COVID-19 vaccines to control disease outbreaks by herd immunity. Vaccines can trigger immunity as many vaccine-related immunological adverse events have been described. Case Presentation: A case of a 32 years old Filipino female with no known co-morbidity who presented with rashes on bilateral lower extremities one day after receiving her second dose of CoronaVac-Sinovac vaccine. These were non-pruritic nor painful, which appeared to be initially well-defined round erythematous macules, papules, and plaques, mostly raised. It was associated with colicky abdominal pain and inflammatory arthritis affecting the both knees and ankles. She has no vices but known to have allergy with seafood. There were multiple well defined erythematous round to irregularly shaped purpuric macules, papules and plaques, non-blanching, flat and raised, on arms near the antecubital fossa, abdomen and lower extremities. She underwent skin biopsy and direct immunofluorescence showed interface dermatitis with leukocytoclastic vasculitis and IgA +1 vessel wall, and fibrinogen +2 vessel wall, respectively. There was microscopic hematuria and proteinuria. The Urine protein creatinine ratio was normal at 0.193 gm/gm. She was managed as a case of IgA vasculitis and was given moderate dose of steroid (0.5mg per kilogram per day prednisone equivalent) and omeprazole. She was discharged improved with resolution of rashes evident during follow up at the out-patient consultation. Conclusion(s): We report a case of an adult Filipina developing IgA vasculitis following CoronaVac COVID-19 vaccination. She responded well following initiation of steroid therapy. Autoimmune phenomenon following immunization is possible through different mechanisms. These include molecular mimicry, a hyper-stimulated inflammatory state, and autoimmune syndromes induced by adjuvants. While no strategies have been found to prevent autoimmunity following vaccination, it should be emphasized that vaccine recipients should seek medical care for any untoward events following receipt of any immunization.

Cutaneous vasculitis: Lessons from COVID-19 and COVID-19 vaccination.

Cutaneous vasculitis (CV) is an inflammatory skin-limited vascular disease affecting the dermal and/or hypodermal vessel wall. From the pathogenetic point of view, idiopathic forms are described as well as the induction from various triggers, such as drugs, infections, and vaccines. Following SARS-CoV-2 pandemic outbreak, cases of CV induced by both COVID-19 and COVID-19 vaccinations have been reported in literature. The aim of our work was to collect multiple cases available in the literature and analyze the frequency of the different forms of induced vasculitis, as well as their histological and immunopathological features. Although rare, CV induced by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) and vaccines may provide interesting insights into the pathogenesis of these inflammatory processes that may in the future be useful to understand the mechanisms underlying cutaneous and systemic vasculitis.

Antineutrophil Cytoplasmic Autoantibody (ANCA)-Associated Vasculitis With Mucosal Involvement Following COVID-19 Pneumonia.

Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are a group of inflammatory disorders in which autoantibodies damage small arteries throughout the body, including in the upper and lower respiratory system, kidneys, as well as the skin. AAV may be precipitated by a variety of causes, including infections. In this report, we examine the case of a patient who developed AAV that was suspected primarily based on mucocutaneous hemorrhagic bullae, elevated ANCA levels, and subsequently confirmed by kidney biopsy, while recovering from coronavirus disease 2019 (COVID-19) infection. AAV and COVID-19 infections may present with similar symptoms, rendering an accurate diagnosis challenging. Additionally, only a few other cases describing a similar onset of AAV post-COVID-19 infection have been described in the literature. Initial presenting features of AAV in such cases have varied considerably, which makes the diagnosis even more challenging. We also engage in a review of such cases to assess key similarities, different treatment options, and outcomes. Lastly, the fact that several mechanisms have been proposed for AAV highlights the need for continued research to help clarify the pathophysiology while also identifying the optimal therapy.

Sympathetic Nerve Block in Use Management of Vasculitis-Induced Complex Regional Pain Syndrome: A Case Report

Background: Case Report: Conclusion(s): Leukocytoclastic vasculitis (LCV) can lead to both dermatological and neuropathic symptoms with many patients ultimately meeting criteria for complex regional pain syndrome (CRPS). While there are accepted treatments for both LCV and CRPS, when these treatments fail, there is very limited evidence for next steps in management. A 34-year-old woman with a history of COVID exposure-induced LCV presented to the pain medicine clinic with back and left lower leg pain. The patient failed medical management and initial conservative interventions. Ultimately lumbar sympathetic nerve block resulted in significant and lasting improvement in her symptoms. Sympathetic blockade shows promise in the treatment of refractory vasculitis and chronic pain. More extensive research with a larger sample size and longer patient follow-up is necessary to determine the true efficacy of sympathetic nerve block in both CRPS and vasculitis. Copyright © 2022, American Society of Interventional Pain Physicians.

A RARE CASE OF COVID-19-INDUCED AORTITIS

SESSION TITLE: Post-COVID-19 Infection Complications SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Aortitis is a type of vasculitis that refers to inflammation of the aortic wall. Most common causes are rheumatologic disorders and bacterial infection. Here, we report a viral cause of aortitis induced by COVID-19. CASE PRESENTATION: A 73 year old female with history of coronary artery disease, chronic kidney disease, COPD, hypertension, pulmonary embolism on Eliquis and abdominal aortic aneurysm (AAA) status post repair presented with acute hypoxemia secondary to Covid-19 pneumonia. Of note, patient was vaccinated against COVID-19. CT abdomen at admission demonstrated a known infrarenal AAA with increased degree aortic wall thickening, concerning for aortitis. Aortitis was initially thought to be due to endovascular infection from possible bacteremia rather than surgical site infection as the patient had the AAA repair almost a year prior. Given that bacterial aortitis could result in death, blood cultures were obtained and she was started on Vancomycin and Rocephin. Rapid Plasma Reagin was ordered to rule out syphilis. She had titer of 1-2 which was thought to be false positive as fluorescent treponemal antibody absorption test was negative. After blood cultures and inflammatory markers were negative, antibiotics were discontinued. Aortitis was attributed to COVID. Patient was treated with DEXA-ARDS protocol. Repeat CT abdomen after 8 days no longer showed gross evidence of aortitis. Patient was discharged home with home healthcare. DISCUSSION: Aortitis, a rare complication of COVID-19, has been reported. A proposed mechanism of this pathogenesis involves acute endotheliitis, where endothelial cells infected by virions become infiltrated by neutrophils and mononuclear cells, leading to apoptosis and lymphocytic endotheliitis [1]. Later, these arteries move through the stages of an accelerated karyolysis, accumulation of apoptotic bodies, caspase granules, and fibrinoid substances, leading to leukocytoclastic vasculitis [1]. This inflammatory reaction is followed by deposition of polyclonal antigen-antibody immune complexes, which is a type III hypersensitivity acute vasculitis [2]. Our patient's history of AAA repair predisposed her to increased endothelial dysfunction. After other bacterial infectious causes and post-surgical complications were ruled out, patient was treated with steroids. Most cases of COVID induced aortitis have been treated with prednisone that required treatment for around 1 month [3]. Here, we present a patient treated with DEXA-ARDS with resolution of aortitis. CONCLUSIONS: Due to the novelty, the understanding of exact pathogenesis and long term effects of COVID-19 induced aortitis is limited. However, our case does serve to support prior case reports of COVID-19 aortitis that showed clinical and radiologic response to steroids. Further research is warranted to diagnose and treat aortitis in order to avoid life threating complications. Reference #1: Varga, Zsuzsanna, et al. "Endothelial cell infection and endotheliitis in COVID-19.” The Lancet 395.10234 (2020): 1417-1418. Reference #2: Roncati, Luca, et al. "Type 3 hypersensitivity in COVID-19 vasculitis.” (2020): 108487-108489. Reference #3: Dhakal, Pravash et al. "Aortitis in COVID-19.” IDCases vol. 24 (2021): e01063. doi:10.1016/j.idcr.2021.e01063 DISCLOSURES: No relevant relationships by Jessica Lee No relevant relationships by Thong Ngo No relevant relationships by Marrian Sedrak No relevant relationships by Hena Yagnik

A RARE CASE OF ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (ANCA)-ASSOCIATED VASCULITIS IN THE SETTING OF COVID-19 INFECTION

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: Anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA-AV) is an autoimmune mediated inflammation of small and medium sized vessel walls. The occurrence of this autoimmune vasculitis is typically associated with underlying infection, medications, and genetic predisposition.(1) The objective of this case report is to describe a rare presentation of ANCA-AV in the setting of COVID-19 infection. CASE PRESENTATION: A 67-year-old male presented to the hospital with a three-week history of cough productive of brown sputum, epistaxis, fatigue, decreased appetite, and unintentional weight loss. During the previous week, he experienced worsening dyspnea and bilateral lower extremity swelling. On physical examination, he was hypoxic requiring 4L of supplemental oxygen to maintain saturations greater than 90%. Diffuse and bilateral wheezes were heard on auscultation of his lungs. A tender petechial rash was dispersed over his limbs, trunk, oropharynx, and nasopharynx. A basic metabolic panel revealed a mild, acute renal impairment. Urinalysis showed new onset proteinuria and hemoglobinuria. Nasopharyngeal swab was positive for SARS-COV-2. Contrast-enhanced computed tomography of the chest revealed diffuse, bilateral ground glass opacities and interstitial changes. Therapy with piperacillin-tazobactam was started for presumed superimposed bacterial community acquired pneumonia in the setting of COVID-19 infection. On day three of hospitalization, the petechial rash progressed to hemorrhagic blisters. His oral petechiae were now ulcerated. A punch biopsy of the affected skin showed leukocytoclastic vasculitis. Anti-Proteinase 3 (PR3) antibodies were positive. Subsequent renal biopsy showed pauci-immune focal necrotizing crescentic glomerulonephritis consistent with ANCA-AV. Therapy with intravenous pulse dose corticosteroids led to improvement in his rash and body aches, and he was discharged home on oral steroids ten days after admission. DISCUSSION: This report describes a rare case of ANCA-AV in the setting of recent COVID-19 infection. Differentiation of ANCA-AV, bacterial and COVID-19 pneumonia can be challenging on chest imaging alone.(1) New onset renal impairment, hematuria, proteinuria and the presence of the petechial rash were suspicious for co-existing ANCA-AV in this patient. COVID-19- associated cytokine storm and formation of neutrophil extracellular traps (NETs) is postulated to be the underlying cause.(1-3) NETs present myeloperoxidase (MPO) and PR3 antigens to the immune system. Formation of auto-antibodies to MPO and PR3 lead to the development of ANCA-AV. The findings of NETs on kidney biopsy specimens in patients with ANCA-AV supports this hypothesis.(1,2) CONCLUSIONS: To avoid the misdiagnosis of COVID-19-induced vasculitis, a low threshold to investigate co-existing vasculitis in patients with COVID-19 and associated clinical findings is highly recommended. Reference #1: Izci Duran T, Turkmen E, Dilek M, Sayarlioglu H, Arik N. ANCA-associated vasculitis after COVID-19. Rheumatol Int. 2021;41(8):1523-1529. Reference #2: Uppal NN, Kello N, Shah HH, et al. De Novo ANCA-Associated Vasculitis With Glomerulonephritis in COVID-19. Kidney Int Rep. 2020;5(11):2079-2083. Reference #3: Cobilinschi C, Cobilinschi C, Constantinescu A, Draniceanu I, Ionescu R. New-Onset ANCA-Associated Vasculitis in a Patient with SARS-COV2. Balkan Med J. 2021;38(5):318-320. DISCLOSURES: No relevant relationships by Andrei Hastings No relevant relationships by Jason Lane No relevant relationships by Tanya Marshall No relevant relationships by Palak Rath No relevant relationships by Sterling Shriber No relevant relationships by inderprit Singh No relevant relationships by Samuel Wiles

PRIMARY SJÖGREN'S SYNDROME with JUVENILE ONSET: REPORT of 15 CASES

Background: Primary Sjögren's syndrome (pSS) is a chronic and progressive multisystem autoimmune disease which rarely onset in children and adolescents. Diagnostic delay in large part of patients are common due to the non-specifc and variable symptoms and the slow progression of disease. Objectives: To analyse demographic data, specifc extraglandular, salivary and ocular manifestations, laboratory parameters and therapy of pSS with juvenile onset. Methods: Retrospective study of all patients (pts) with pSS in single center. Results: pSS was verifed in 15 pts (6.7% were boys), which amounted to 23.8% of all pts with SS in our pediatric rheumatologic department. The median age of pSS onset was 8.0 y.o. [IQR 7.0;10.2]. The median of disease duration at the time of pSS verifcation was 2.75 years [2.2;5.6]. All patients had systemic manifestations at onset: constitutional abnormalities-33.3%, nonerosive polyarthritis-64.3%, polyarthralgias-26.7%, lymphadenopathy-73.3%, cutaneous involvement-53.3% (2-xerosis, 2-annular erythema, 1-erythema nodo-sum, 2-Raynaud phenomenon, 2-nonspecifc spotty rashes, 1-hemorrhagic rash). At the time of diagnosis 7 pts (46.7%) had isolated involvement of salivary glands, 8 pts (53.3%)-combined with involvement of lacrimal glands. The decrease in salivary gland function was recorded in 80% of cases, hypolacrimia-in 46.7%, 1 patient had isolated hypolacrimia. Recurrent parotitis was present in 6 pts (40.0%). At time of diagnosis pulmonary involvement had 20.0% of pts, 1 patient had renal tubular acidosis. 8 pts (53.3%) had various hematological disorders: anemia-in 3 pts (20.0%), leukopenia-in 6 (40.0%). ANA Hep-2 were detected in 100% pts (in titer 1/640-4, 1/1280-7, 1/2560-3, 1/20480-1, with mixed patterns in all pts: speckled + homogeneous-9 pts, speckled + homogeneous+cytoplasmic-6 pts), anti-Ro-in 12 pts (80.0%), anti-La-in 8 pts (53.3%), RF+-in 9 pts (60.0%). 6 pts (40.0%) had polyclonal hypergammaglob-ulinemia, max 42%. 2 pts (13.3%) had concomitant autoimmune non-rheumatic disease;1-cutaneous psoriasis, 1-autoimmune thyroiditis. The treatment of each patient was justifed by the main individual manifestations: 93.3% received glucocorticoids, 26.7%-methotrexate, 33.3%-hydroxychloroquine, 6.7%-mycophenolate mofetil. Treatment with biologics (B) was received by 13 (93.3%) pts (7-rituximab (RTM), 6-abatacept (ABA)) with a good response in 10 pts, including improvement in the function of the salivary and lacrimal glands in 7 pts. 1 patient received 2B-RTM and ABA sequentially due to the development of MAS 7 days after 1st RTM infusion. B was discontinued in 3 pts: 1 due to development of hemorrhagic vasculitis 2 days after the 1st RTM infusion, 1-COVID-19 with lung involvement (CT 3-4) 2 weeks after the 1st RTM infusion, 1-inefficiency of ABA during 15 months. Conclusion: In our pediatric rheumatologic department pts with pSS made up less than a quarter of all pts with SS. The diagnosis was verifed delayed in all pts, which can be explained by a wide range of nonspecifc manifestations at the onset. However, the manifestations of SS that were present at the time of diagnosis were brought under control on the background of complex therapy, including the prescription of B, with a good efficacy and safety profile of therapy.

A case of cutaneous vasculitis following third dose of SARS-CoV-2 vaccine in an immunocompromised patient

A 58-year-old man known to dermatology services, established on guselkumab for psoriasis and methotrexate for psoriatic arthritis, attended with an acute onset purpuric rash distributed over both his lower limbs, one day after his third dose of SARS-CoV-2 Pfizer-BioNTech vaccine (booster). He had received his initial vaccinations 6 months prior with no reported reactions. He denied any previous SARS-CoV-2 infection or recent symptoms suggestive of COVID-19. There had been no new recent medications and no systemic symptoms were reported. Examination revealed a nonblanching, palpable, purpuric rash distributed over both lower limbs, clinically in keeping with cutaneous vasculitis. Baseline observations were satisfactory including blood pressure and temperature. Bedside investigations included a urinalysis which revealed no proteinuria or haematuria. Punch biopsies were taken and were consistent with a leucocytoclastic vasculitis (LCV). He was managed symptomatically with potent topical steroids with good clinical response. LCV is classified as a cutaneous, small vessel vasculitis, exclusively characterized by deposition of immune complexes in the dermal capillaries and venules (Baigrie D, Bansal P, Goyal A, Crane JS. Leukocytoclastic vasculitis. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing, 2021). LCV following both first and second SARS-CoV-2 vaccinations has been documented in recent literature with a few reports following a third booster dose, and in particular within an immunocompromised population. This particular case has raised questions regarding delayed immune response following SARS-CoV-2 vaccine in this subgroup. The pathophysiology of SARS-CoV-2 vaccine-induced LCV has not been extensively researched;however, it is felt to be caused by offtarget immune activation after the vaccination (Dicks AB, Gray BH. Images in vascular medicine: leukocytoclastic vasculitis after COVID-19 vaccine booster. Vasc Med 2022;27: 100-1).

Leukocytoclastic vasculitis secondary to COVID-19 vaccine

We present the case of a severe cutaneous reaction following COVID-19 vaccination. A 60-year-old white woman presented to our service with an extensive painful, pruritic rash affecting her bilateral lower limbs. This was on a background of psoriasis, psoriatic arthritis and notably inoculation against COVID-19 with the Johnson & Johnson vaccine hours prior to onset. There was no history of new medications, illicit drug use or infections. On examination, extensive palpable purpura was noted circumferentially at both lower limbs from the knee distally. Tense bullae were described at her bilateral ankles. She was apyrexial. Her cardiopulmonary and gastrointestinal examinations were normal. A punch biopsy taken from her right lower limb demonstrated findings consistent with leucocytoclastic vasculitis (LCV). Direct immunofluorescence demonstrated IgA deposits within the vasculature. IgA LCV secondary to COVID-19 vaccination was proposed on the basis of histological and clinical findings. Treatment consisted of oral steroids, oral antibiotics for secondary infection and wound dressings. Opioid analgesia and nitrous oxide were implemented for severe pain associated with dressing changes. As her urinary protein creatinine ratio was in excess of 100 mg dL-1 and microscopic haematuria was noted on urine microscopy, she was referred to nephrology. We note case reports of patients diagnosed with LCV up to 2 weeks following COVID-19 vaccination (Cavalli G, Colafrancesco, De Luca G et al. Cutaneous vasculitis following COVID- 19 vaccination. Lancet Rheumatol 2021;3: E743-4). In this case, onset of symptoms occurred within hours. While this presentation may have been coincidental, the relationship between immune complex vasculitis, COVID-19 infection (Iraji F, Galehdari H, Siadat AH, Bokaei Jazi S. Cutaneous leukocytoclastic vasculitis secondary to COVID-19 infection: a case report. Clin Case Rep 2020;9: 830-4) and vaccination (Cavalli et al.) has been reported in the literature and represents the most likely diagnosis.