Transient optic neuritis and perineuritis associated with anti-MOG antibody after SARS-CoV-2 mRNA vaccination

Backgrounds: To report the first case of left optic neuritis and perineuritis associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) BNT162b2 mRNA vaccination. Case presentation: A 39-year-old woman was referred and admitted to our hospital due to transient left visual field abnormality with left ophthalmalgia and headache 12 days after the first vaccination dose of SARS-CoV-2 (BNT162b2). On admission (Day 2), she presented with left ophthalmalgia and headache without any other neurological deficits including the movement of eyeballs, visual field, visual acuity, or nystagmus. MRI on Day 2 suggested slight left optic neural swelling;Gadolinium-enhanced MRI on Day 4 revealed left optic perineuritis. Test for serum anti-aquaporin 4 antibody was negative, whereas anti-myelin oligodendrocyte glycoprotein (MOG) antibody was positive. She was diagnosed with left optic perineuritis after SARS-CoV-2 mRNA vaccination. Her visual disturbance never recurred and her ophthalmalgia and headache subsided only with anti-inflammatory agents. Discussion(s): Many cases of optic neuritis associated with vaccinations have been reported except for SARS-CoV-2 BNT162b2 mRNA. To our knowledge, only one neuromyelitis optica case was associated with anti-MOG antibody. Therefore, we propose that SARS-CoV-2 mRNA vaccination may induce transient optic neuritis and perineuritis, associated with anti-MOG antibody in the present case. Conclusion(s): This is the first case of left optic neuritis and perineuritis associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) BNT162b2 mRNA vaccination.Copyright © 2022

How to Evaluate Ultrasound Abnormalities of Salivary Glands in Sjogren Syndrome Using Web Training Session during Covid 19 Period? An International Reliability Exercise

Background. Salivary glands ultrasonography (SGUS) is an emerging tool to evaluate damages in primary sjogren patients (pSS). Up to date, SGUS has demonstrated its metric properties as an outcome measure for diagnosing pSS. As New therapeutics protocols are in developing it seems mandatory to use validated SGUS scoring systems. Objectives. The goal of our study was to evaluate international SGUS reliability exercise before beginning an international SGUS study to evaluate Modification Abnormalities of Salivary glands in pSS According to disease duration (MASAI study). Methods. Fourteen sonographers with different levels of SGUS participated in the exercise, evaluating 60 grey scale static images (30 parotid and 30 submandibular glands). Before the exercise, training was done by videoconferencing showing the different pathological SGUS findings and explaining the new OMERACT scoring system. We evaluated homogeneity (yes/no), location of hypoechogenity (0 to 3), hyperechoic band (0-3), comprehensive OMERACT scoring system (0-3), binary comprehensive OMERACT (0-1 versus 2-3) and diagnosis appreciation (No/yes). Intra-reader and interreader reliability were estimated by computing Cohen's kappa coefficients using SPSS 25.0 (SPSS Inc., Chicago, IL), and was interpreted as follows: slight, 0-0.20;fair, 0.21-0.40;moderate, 0.41-0.60;substantial, 0.61-0.80;and almost perfect, 0.81-1. The most experienced sonographer (P4) was considered as the gold standard. Results. Intra-reader reliability of the most experienced was perfect and substantial for the OMERACT scoring system. Intra-reader reliability of the other sonographers was fair to almost perfect for homogeneity and diagnosis whereas the reliability was fair to substantial for other items. Inter-reader reliability between the two most experienced sonographers (P4 and P12) was almost perfect for homogeneity, substantial for diagnosis and moderate for OMERACT scoring system. Changing OMERACT scoring system in binary items, the reliability of the most experienced sonographer was good 0.65 (9 images with homogeneity and low OMERACT, 42 had heterogeneity and high OMERACT, 9 had no homogeneity but low OMERACT and none had homogeneity with high OMERACT), clearly lower that of homogeneity. Compared to the most experienced sonographer, reliabilities of other sonographers were moderate to almost perfect for both homogeneity and diagnosis but only fair to moderate for OMERACT (Table I, II). Conclusions. According to the updated literature, we confirmed that homogeneity is the most reliable item, very close to diagnosis appreciation. Structural damages evaluations by the OMERACT scoring systems gave lower kappa values but remain still useful for diagnosing and particularly following parenchymal modifications. (Table Presented).

MULTISYSTEM SARCOIDOSIS ASSOCIATED WITH THE COVID-19 MRNA VACCINE

SESSION TITLE: Unusual Presentations of Sarcoidosis SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 01:35 pm - 02:35 pm INTRODUCTION: COVID-19 infection has brought high morbidity and strain on hospitals. Multiple vaccines have been developed against COVID and are now widely available. These vaccines have been linked to various side effects listed by the Centers for Disease Control and prevention (CDC) website- https://www.cdc.gov/coronavirus/2019-ncov/vaccines/safety/adverse-events.html. We present a case of multisystem sarcoidosis after receiving an mRNA COVID-19 vaccine. CASE PRESENTATION: Our patient is a 42-year-old African American woman who reported low grade fever after her first dose of the BNT162b2 mRNA COVID-19 vaccine (Pfizer). After her second dose, the fever continued, and fatigue and lethargy were noted. She denied any respiratory symptoms and other review of systems were negative. She was referred for autoimmune workup by her PCP. Then, she received her booster dose (~6 months after the second dose) and 2 weeks later, she noticed a right posterior calf mass that was firm and non-tender on exam. Rest of the exam was unremarkable. Orthopedic surgery was involved, and an excision-biopsy was done. Pathology demonstrated non-necrotizing granulomatous inflammation (Figure 1A). Her blood counts and metabolic panel were normal. Other labs showed elevated C-reactive protein of 19 mg/L (normal < 5 mg/L), angiotensin-converting enzyme level of 93 U/L (normal is 9-67 ) and Vitamin D level of 8.4 ng/mL (normal is 20-50 ng/mL). She was referred to pulmonary clinic for further evaluation. Pulmonary function test showed mild restrictive physiology. CT chest revealed enlarged mediastinal and hilar lymph nodes (Figure 2) and lung parenchymal involvement (Figure 3). EBUS-guided TBNA was performed and showed granulomatous inflammation. (Figure 1B). DISCUSSION: We present a case of multisystem sarcoidosis with mediastinal and soft tissue compromise in a temporal association with Pfizer mRNA COVID-19 vaccine. This is an uncommon adverse reaction and has not been reported by the CDC. In our case, there is a strong temporal relationship between the vaccination schedule and onset of symptoms, starting with fever and tiredness as common side effects that progressed to mass-like lesion in leg and mediastinal adenopathy. Cutaneous sarcoidosis might occur with COVID-19 vaccines (#1), however only 3 cases of multisystem sarcoidosis have been reported so far. Two cases developed Lofgren syndrome after the COVID-19 vaccination (#2) and one case with uveitis and parotid compromise (#3). To the best of our knowledge, this is the first case reporting sarcoidosis with soft tissue involvement in association with Pfizer mRNA COVID-19 vaccine. CONCLUSIONS: Our patient met criteria for multisystem sarcoidosis and there is a strong temporal relationship between the onset of symptoms/disease and COVID-19 vaccine. Immunological adverse events related to vaccines are uncommon. Our case elucidates to consider the diagnosis in right clinical context. Reference #1: Niebel D, Novak N, Wilhelmi JZ, Wilsmann-Theis D, Bieber T et al. Cutaneous adverse reactions to COVID-19 vaccines: Insights from an immune-dermatological perspective. Vaccines 2021,9,944. Reference #2: Rademacher J, Tampe B and Korsten P. First Report of Two Cases of Löfgren's Syndrome after SARS-CoV-2 Vaccination-Coincidence or Causality? Vaccines 2021, 9, 1313. https://doi.org/10.3390/ vaccines9111313 Reference #3: Matsuo T, Honda H, Tanaka T, Uraguchi K, Kawahara M et al. COVID-19 mRNA vaccine-associated uveitis leading to diagnosis of Sarcoidosis: case report and review of literature. J Investig Med High Impact Case Rep. 2022 Jan-Dec;10: 23247096221086450. DISCLOSURES: No relevant relationships by Chien Chen No relevant relationships by Eleonora Fiorletta Quiroga No relevant relationships by Manish Joshi No relevant relationships by Angel Mitma No relevant relationships by PRACHI SALUJA

Mammary analogue secretory carcinoma presenting with cervical lymphadenopathy: A rare case report with review of the literature.

INTRODUCTION: Mammary analogue secretory carcinoma is a rare malignant tumor of the salivary glands that typically involves the major glands. The aim of the current study is to report a rare case of mammary analogue secretory carcinoma that presented with left cervical lymphadenopathy. CASE REPORT: A 59-year-old lady presented with left cervical lymphadenopathy. Tissue biopsy and immunohistochemistry revealed metastatic carcinoma, favoring ovarian origin. Staging workup was performed and, ultimately, the patient was treated as having a carcinoma of unknown primary. After showing partial response to therapy, left side neck dissection was performed. Based on better assessment of the histologic picture and a broader panel of immunohistochemistry performed on the excision specimen, the final diagnosis was that of mammary analogue secretory carcinoma. DISCUSSION: Mammary analogue secretory carcinoma is usually an indolent salivary gland carcinoma, with the majority of patients presenting with a slow-growing, painless mass measuring approximately 2 cm in size, and a reported duration ranging from 2 months to several years. In certain cases, pain and facial paralysis have been reported. It could also be found incidentally during radiologic assessment for thyroid illness or routine dental screening. CONCLUSION: Diagnosing mammary analogue secretory carcinoma is challenging, and this should be in the differential diagnosis list of metastatic carcinomas to cervical lymph nodes.

Parotid Abscess in A Case of Acute Lymphoblastic Leukemia

Parotid gland enlargement as a presenting manifestation of acute lymphoblastic leukemia (ALL) is very rare, even though it has been reported in acute myeloid leukemia. Here we present a case of parotid abscess in a case of ALL in the presence of Dengue.

COVID-19-associated Parotid Gland Abscess.

BACKGROUND: Human infection with the SARS-CoV-2 virus has caused a pandemic characterized by a plethora of diseases, of which those affecting the internal organs and nervous system can have severe, life-threatening consequences. Among the manifestations of the disease at the viral entry site, diseases in oral mucosa adnexa are rarely reported. This case report describes a COVID-19-associated aggravating parotid gland disease. CASE REPORT: The 47-year-old, SARS-CoV-2-positive male patient, already hospitalized and now in need of ventilation, had been referred for intensive care treatment due to increasing respiratory problems. A unilateral swelling of the cheek was noticed, the cause of which was initially a parotid gland infection. Examination ruled out mechanical causes of the parotid gland. During further treatment, the patient developed a parotid abscess, which was drained extra orally. CONCLUSION: The oral cavity is primarily considered as a reservoir of the pathogen. There is an increasing number of reports detailing inflammation of the major salivary glands associated with SARS-CoV-2 virus. Knowledge of this association facilitates therapeutic decisions.

COVID-19 mRNA Vaccine-Associated Uveitis Leading to Diagnosis of Sarcoidosis: Case Report and Review of Literature.

A 34-year-old Japanese person with male gender identity who had been taking intramuscular injection of methyltestosterone depot for 11 years after bilateral mastectomy noticed blurred vision 5 days after the second vaccination for COVID-19 (Tozinameran; Pfizer-BioNTech) in the interval of 3 weeks following the first vaccination. The patient was diagnosed as granulomatous iritis with mutton-fat keratic precipitates and small iris nodules at the pupillary margin in the right eye and began to have 0.1% betamethasone eye drops with good response. The patient, however, continued to have fever and malaise and showed a high level of serum soluble interleukin-2 receptor (sIL-2R) even 4 weeks after the second vaccination. Computed tomographic scan disclosed mediastinal and bilateral hilar small lymphadenopathy together with limited granular lesion in the right lung. Gallium-67 scintigraphy demonstrated high uptake not only in mediastinal and hilar lymph nodes but also in bilateral parotid glands. Right parotid gland biopsy revealed noncaseating granulomas and proved pathological diagnosis of sarcoidosis. The systemic symptoms were relieved by oral prednisolone 20 mg daily. Even though the causal relationship remains undetermined, this case is unique at the point that vaccine-associated uveitis led to the detection of pulmonary lesions and lymphadenopathy, resulting in clinical and pathological diagnosis of sarcoidosis. In literature review, 3 patients showed sarcoidosis-like diseases after COVID-19 vaccination: 2 patients were diagnosed clinically as Lofgren syndrome with acute onset of erythema nodosum and ankle swelling, with or without mediastinal and hilar lymphadenopathy, whereas 1 patient with mediastinal lymphadenopathy but no uveitis was diagnosed pathologically by biopsy as sarcoidosis.

An unusual case of systemic lupus erythematosus with Sjögren syndrome and recurring episodes of macrophage activation syndrome in a boy - difficulties to diagnose, difficulties to treat

Introduction: systemic lupus erythematosus with juvenile onset (jSLE), especially in boys, can occur with unusual manifestations, including Sjogren's syndrome (SS), accompanied by unexpected complications and difficulties in choosing therapy. Objectives: to present a rare case of successfully application of abatacept in an jSLE+SS in a boy who had two episodes of macrophage activation syndrome (MAS). Methods: Case report. Results: 4-year-old boy admitted to our clinic for the first time in 2010 presenting the 6-month history of disease. There were fever up to 39°C, polyarthritis, anemia, trombocytopenia, hyperIgG-emia, increase TA (2N), CRP (20 mg/l), RF 30 mg/l at the disease onset. Initially JIA, polyarthicular sybtype, RF+ was diagnosed in regional hospital. Treatment with NSAIDs, GC iv 125 mg No3, methotrexati 7,5 mg weekly was started. During the next 3 weeks laboratory results were constantly getting worse (HB 80 τ/π, trombocytes 145000, leucocytes 1000, increase TA 10N) and new clinical symptoms occurred (maculopapular rash with itching;splenomegaly;febrile fever). He received GC iv + per os 1 mg/ kg with a short-term effect. On 1st admission in our clinic in September of 2010, he had general fatigue and tiredness, classic malar rash, severe cutaneous vasculitis (palpable purpura and digital capillaritis), Raynaud's syndrome, enanthema, myopathy, lymphadenopathy, hepatosplenomegaly, polyarthritis. Data of laboratory tests: Hb 96g/l, ESR 27 mm/h, ANA titer 1:640 h+sp+cytopl, anti-SS-A(Ro)>200 U/ml, RF 230 ME/ml, C4 0.07 g/l. The revision of bone marrow biopsy showed changes typical for MAS. So jSLE was diagnosed as with SLICC criteria, 2012 with MAS at onset according the preliminary diagnostic criteria for MAS Complicating SLE. The initial SLEDAI was 29. Patient failed to respond to GC iv repeating courses, GC per os max 0.7 mg/kg, IVIG repeating courses, DMARDs consequentially: cyclophosphamide iv, azathioprine, mycophenolate mofetil (MMF) + hydroxychloroquine. Rituximab (RTX) was introduced in November of 2013 due to inefficiency of prior therapy in dose 375mg/m2 weekly N2 on course. Concomitant therapy: GC per os 0.5 mg/kg, MMF 500 mg/day, hydroxychloroquine 200 mg/day. Treatment with RTX allowed to decrease the dose of GC to 0.2 mg/kg, to reduce the activity of the disease (SLEDAI=4), but relapses of the disease required a repeat of RTX therapy every 6 months (5 courses in total). Patient developed a recurrent episode of MAS on 6th years of disease (the 8th day after RTX - 5th course, 1st infusion). Therapy of RTX was discontinued. In June 2016 Sjogren's syndrome has been verified (according to parotid sialography, unstimulated sialometry in combination with clinical signs of dry mouth, anti-SS-A(Ro)+, RF+). Correction of therapy was carried out at the expense of the change a dose of GC, the dose of MMF was increased to 750 mg per day. Patient received repeat courses of IVIG due to recurrent infections of mouth. Since October 2017 flare due to fever, polyarthritis, skin and mucosal lesions. The dose of GC has been increased to 0.5 mg/kg. In November 2017, abatacept therapy with 10 mg/kg was started with good safety. Inactive status of the disease was achieved after 12 months of therapy. Now boy receives abatacept during 42 months, continues GC 5 mg per day, hydroxychloroquine 100 mg per day, MMF 750 mg per day. In November 2020, he underwent COVID-19 with minimal manifestations (runny nose, fatigue, positive PCR test) without reactivation of rheumatic disease. Conclusion: This clinical case demonstrates efficacy and safety of abatacept in the rare combination of jSLE, SS and recurring episodes of MAS in boy with early onset.

Primary sjögren's syndrome VS MS: Overlap or misdiagnosis?

Case report-IntroductionPrimary Sjögren's syndrome (PSS) is a systemic autoimmune disease that mainly affects exocrine glands. Central nervous system (CNS) involvement in primary SS is extremely rare. In 10-20% of patients diagnosed with PSS, there are lesions in the central nervous system analogous to those presented in multiple sclerosis. We report a case of a 58-year-old female, diagnosed as PSS and multiple sclerosis (MS) (2007), but later, all neurological manifestations turned out to be related to PSS rather MS. This case illustrates how difficult it could be, distinguishing Sjögren's with CNS involvement from MS, even to an expert clinician.Case report-Case descriptionA 58-year-old lady presented to Rheumatology clinic in 2010 with polyarthralgia, sicca symptoms and Raynaud's. Immunology tests (positive anti-RO &anti-LA antibodies) and lymph node biopsy were highly suggestive of primary Sjögren's. She was commenced initially on HCQ and prednisolone. Then Methotrexate was added in because she continued to struggle with inflammatory arthritis.Her Sicca symptoms got gradually worse despite being on Acetylcysteine, Hylo Forte, cyclosporine and Dexamethasone eye drop. Therefore, autologous serum eye drops were tried with good response.Her past medical history included Hypertension and knee OA. She has been under Neurology since 2007 for MS. Her original neurological symptoms were imbalance, dizziness, headaches, and tremor of the right arm which seem to be persistent with no definite relapses. MRI brain and spine were reported as normal with a few non-specific white matter areas, but the lumbar puncture result was positive for unmatched bands in the CSF.Clinical examination revealed action tremor in the right upper limb. She had diminished vibration, pinprick, and cold temperature perception in a stocking distribution.InvestigationsWBC 2.0, lymphocyte 0.62, DsDNA 1, C3 0.061, C4 0.01. CRP <5, PV 1.63, APS screen was negative NCS: evidence of sensory and axonal neuropathy predominantly affecting lower limbs. CTCAP 2018-showed calcification of parotid. No evidence of lymphoproliferative disorder.The latest MRI 2019 showed two new lesions (right corpus &right striatum lesion) which according to Neuro-radiology MDT discussion were not typical of MS and more likely related to underlying CTD.Based on these MRI findings and the recent history of skin vasculitis, the deterioration in her neurological condition was put down to primary Sjögren's. Therefore, her treatment was escalated to cyclophosphamide during the COVID-19 pandemic with a particularly good outcome. She was then switched to MMF and her condition remained stable.Case report-DiscussionNeurological disorders are one of the rare manifestations of primary Sjögren's. The first reports regarding the involvement of the nervous system in PSS were published in 1980. Distinguishing between multiple sclerosis and CNS-SS is not easy.Not only because of similarities of the MRI findings, but also the course of the disease can be like MS, either chronic or relapsing and remitting. This usually leads to missing or delaying in the diagnosis as shown in this case.However, Peripheral neuropathy is far much common in PSS rather MS which can help in differentiating these two conditions. Distal axonal sensory polyneuropathy is the most usual form of neuropathy in PSS as illustrated in this case. Furthermore, up to 75% of patients with SS and active CNS disease have been shown to have concomitant active peripheral vasculitis affecting the skin, muscles, and nerves. Our patient later developed skin vasculitis and peripheral neuropathy which made us think that all the neurological findings including the lesions on the brain are more likely to be related to PSS rather MS.Cognitive disorders are common manifestations of CNS-SS such as attention disorder and memory deficit. Dementia-related to CNS-SS seems to be reversible after immunosuppressive treatment. A second MDT discussion took place and after considering the risk-benefit ratio, the decision was made to give cyclophospham de. Patient was given all the information to make an informed decision. Patient asked for more time to think and discuss with her partner, but eventually, she had decided to have cyclophosphamide despite all the risks and uncertainties around the COVID-19 pandemic. Our patient has noticed significant improvement regarding cognition after completing cyclophosphamide treatment and she was pleased with this outcome.Case report-Key learning points1/Distinguishing between multiple sclerosis and CNS-SS is difficult2/neurophysiological tests should be considered even in asymptomatic patients as they contribute to the detection of early and subtle damage to the nervous system. 3/Successful outcome being achieved with intensive immunosuppression despite all the uncertainties around the COVID-19-19 pandemic.4/This case highlights the importance of communication and openness in shared decisions, especially while confronting uncertainties such as in COVID-19 pandemic.

Evaluating resident involvement and the 'July effect' in parotidectomy.

OBJECTIVE: This study aimed to evaluate the effect of resident involvement and the 'July effect' on peri-operative complications after parotidectomy. METHOD: The American College of Surgeons National Surgical Quality Improvement Program database was queried for parotidectomy procedures with resident involvement between 2005 and 2014. RESULTS: There were 11 733 cases were identified, of which 932 involved resident participation (7.9 per cent). Resident involvement resulted in a significantly lower reoperation rate (adjusted odds ratio, 0.18; 95 per cent confidence interval, 0.05-0.73; p = 0.02) and readmission rate (adjusted odds ratios 0.30; 95 per cent confidence interval, 0.11-0.80; p = 0.02). However, resident involvement was associated with a mean 24 minutes longer adjusted operative time and 23.5 per cent longer adjusted total hospital length of stay (respective p < 0.01). No significant difference in surgical or medical complication rates or mortality was found when comparing cases among academic quarters. CONCLUSION: Resident participation is associated with significantly decreased reoperation and readmission rates as well as longer mean operative times and total length of stay. Resident transitions during July are not associated with increased risk of adverse peri-operative outcomes after parotidectomy.

COVID-19 isolation drape for sialendoscopy-assisted transfacial approach to parotid gland.

Surgical procedures requiring close contact with saliva, such as salivary gland surgery, may determine the risk of spreading the SARS-CoV-2 infection. The use of PPE and isolation settings are mandatory to protect health workers.

Sialadenitis: A Possible Early Manifestation of COVID-19.

Acute sialadenitis may be caused by viruses, including coronaviruses. Although there are anecdotal reports of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) salivary gland infections, there have been no well-documented cases of sialadenitis in patients with COVID-19 described in the literature. We report a case of parotitis and submandibular gland sialadenitis, as well as an isolated case of parotitis, in two patients with concurrent SARS-CoV-2 infections. Computed tomography imaging demonstrated parotid and submandibular gland enlargement with heterogenous enhancement and attenuation, consistent with sialadenitis. Medical management was sufficient for successful resolution of the acute sialadenitis. Laryngoscope, 130:2595-2597, 2020.