To date, around 60% of the world population has been protected by vaccines against SARS-CoV-2, significantly reducing the devastating effect of the pandemic and restoring social economic activity through mass vaccination. Multiple studies have demonstrated the effectiveness and safety of vaccines against COVID-19 in healthy populations, in people with risk factors, in people with or without SARS-CoV-2 infection, and in immunocompromised people. According to the criteria for post-vaccine adverse events established by the World Health Organization, a minority of individuals may develop adverse events, including autoimmune syndromes. The exact mechanisms for the development of these autoimmune syndromes are under study, and to date, a cause-effect relationship has not been established. Many of these autoimmune syndromes meet sufficient criteria for the diagnosis of Adjuvant-Induced Autoimmune Syndrome (ASIA syndrome). The descriptions of these autoimmune syndromes open new perspectives to the knowledge of the complex relationship between the host, its immune system, with the new vaccines and the development of new-onset autoimmune syndromes. Fortunately, most of these autoimmune syndromes are easily controlled with steroids and other immunomodulatory medications and are short-lived. Rheumatologists must be alert to the development of these autoimmune syndromes, and investigate the relationship between autoimmune/inflammatory symptoms and vaccination time, and assess their therapeutic response. 

/pdf/autoimmune-post-covid-vaccine-syndromes-does-the-spectrum-of-1j6n5isy.pdf

Autoimmune post-COVID vaccine syndromes: does the spectrum of autoimmune/inflammatory syndrome expand?

https://doi.org/10.1016/j.fct.2022.113008

Innate immune suppression by SARS-CoV-2 mRNA vaccinations: The role of G-quadruplexes, exosomes, and MicroRNAs

Anti-leucine rich glioma inactivated 1 (LGI1) autoimmune encephalitis (AE) is characterized by cognitive impairment or rapid progressive dementia, psychiatric disorders, faciobrachial dystonic seizures (FBDS) and refractory hyponatremia. Since December 2020, millions of people worldwide have been vaccinated against COVID-19. Several soft neurological symptoms like pain, headache, dizziness, or muscle spasms are common and self-limited adverse effects after receiving the COVID-19 vaccine. However, several major neurological complications, despite the unproven causality, have been reported since the introduction of the COVID-19 vaccine. Herein, we describe a 48 years old man presenting with rapidly progressive cognitive decline and hyponatremia diagnosed with anti LGI1 AE, occurring shortly after the second dose of mRNA COVID -19 vaccine and possibly representing a severe adverse event related to the vaccination. Response to high dose steroid therapy was favorable. As millions of people worldwide are currently receiving COVID-19 vaccinations, this case should serve to increase the awareness for possible rare autoimmune reactions following this novel vaccination in general, and particularly of anti-LGI1 AE.

/pdf/case-report-anti-lgi1-encephalitis-following-covid-19-86n1lyuu.pdf

Case Report: Anti-LGI1 Encephalitis Following COVID-19 Vaccination

Vaccination remains the cornerstone approach to exiting the current global coronavirus disease 2019 pandemic caused by severe acute respiratory syndrome coronavirus 2. The novel messenger ribonucleic acid vaccines offer a high level of protection and are widely used throughout the world. With more people receiving the vaccines, better understanding of their relative safety can be reached. In this report, we describe two patients who developed inflammatory myopathy within 48 hours of receiving the Pfizer BNT162b2 vaccine.Patient A, a 55-year-old South East Asian woman, presented with a 6-week history of pruritic facial and torso rash and a 1-week history of worsening proximal myopathy. Her rash first developed 2 days after receiving the first dose of BNT162b2 vaccine. Patient B, a 72-year-old Caucasian woman, presented with a 2-week history of proximal myopathy a day after receiving the second dose of BNT162b2 vaccine. Both patients had elevated creatine kinase on admission. Patient A tested positive for anti-Mi-2a antibody and anti-Ro-52 antibody, while Patient B was positive for anti-fibrillarin antibody. Magnetic resonance imaging subsequently confirmed generalized acute muscle inflammation and subcutaneous inflammation consistent with inflammatory myositis. Both patients did not have a previous history or family history of autoimmune disease. Patients A and B were diagnosed with dermatomyositis and inflammatory myositis, respectively. They were initially treated with pulsed intravenous methylprednisolone followed by oral prednisolone. However, as their conditions were resistant to corticosteroids, both eventually received and responded well to intravenous immunoglobulin therapy.There are previously reported cases of severe acute respiratory syndrome coronavirus 2-induced and other vaccine-related inflammatory myopathies. However, the precise mechanisms are not elucidated. Without more evidence and convincing pathophysiology, it is not possible to conclude that our patients developed inflammatory myopathy because of the vaccine. However, the timing of the disease onset and the lack of previous history raise an important question of this novel messenger ribonucleic acid therapy. 

/pdf/inflammatory-myopathy-occurring-shortly-after-severe-acute-1l95boq9.pdf

Inflammatory myopathy occurring shortly after severe acute respiratory syndrome coronavirus 2 vaccination: two case reports

As more individuals were coronavirus disease 2019 (COVID-19) vaccinated, unexpected side effects appeared. Herein, we present the case of a 30-year-old man with myopathy in both extremities after the second dose of the Pfizer-BioNTech (BNT162b2) COVID-19 vaccine. Symptoms, swelling and pain, started from the proximal upper and lower extremities and extended to the distal parts. Although he underwent massive hydration, the muscle enzyme level continuously increased. He complained of dysphagia and dysarthria. Microscopically, muscle biopsy showed multifocal or scattered macrophage infiltration and degenerated myofibers. In contrast to general myopathy including inflammatory myositis and rhabdomyolysis, vaccine-induced inflammatory myositis shows a prolonged increase in muscle enzyme levels and multifocal macrophage infiltration with necrosis of the muscle fibers. Symptoms improved with glucocorticoid and immunosuppressive treatment. If vaccinated individuals experience severe and continuous muscle pain and swelling, clinicians should consider vaccine-induced inflammatory myositis, measure the muscle enzyme levels, and perform muscle biopsy for a definite diagnosis.

Clinicopathological Characteristics of Inflammatory Myositis Induced by COVID-19 Vaccine (Pfizer-BioNTech BNT162b2): A Case Report

BACKGROUND AND PURPOSE: Population-based studies suggest that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines may trigger immune-mediated thrombotic thrombocytopenia (VITT) raising concerns for other autoimmune responses. The aim was to characterize neurological autoimmunity after SARS-CoV-2 vaccinations. METHODS: In this single-centre prospective case study patients with neurological autoimmunity in temporal association (≤6 weeks) with SARS-CoV-2 vaccinations and without other triggers are reported. Clinical, laboratory and imaging data were collected with a median follow-up of 49 days. RESULTS: In the study period 232,603 inhabitants from the main catchment area of our hospital (Rhein-Neckar-Kreis, county) received SARS-CoV-2 vaccinations. Twenty-one cases (new onset n = 17, flares n = 4) diagnosed a median of 11 days (range 3-23) following SARS-CoV-2 vaccinations (BNT162b2 n = 12, ChAdOx1 n = 8, mRNA-1273 n = 1) were identified. Cases included VITT with cerebral venous sinus thrombosis (n = 3), central nervous system demyelinating diseases (n = 8), inflammatory peripheral neuropathies (n = 4), myositis (n = 3), myasthenia (n = 1), limbic encephalitis (n = 1) and giant cell arteritis (n = 1). Patients were predominantly female (ratio 3.2:1) and the median age at diagnosis was 50 years (range 22-86). Therapy included administration of steroids (n = 15), intravenous immunoglobulins in patients with Guillain-Barre syndrome or VITT (n = 4), plasma exchange in cases unresponsive to steroids (n = 3) and anticoagulation in VITT. Outcomes were favourable with partial and complete remissions achieved in 71% and 24%, respectively. Two patients received their second vaccination without further aggravation of autoimmune symptoms under low-dose immunosuppressants. CONCLUSIONS: In this study various neurological autoimmune disorders encountered following SARS-CoV-2 vaccinations are characterized. Given the assumed low incidence and mostly favourable outcome of autoimmune responses, the benefits of vaccinations outweigh the comparatively small risks.

Neurological autoimmune diseases following vaccinations against SARS-CoV-2: a case series.

Primary CNS lymphoma (PCNSL) represents an extranodal variant of non-Hodgkin’s lymphoma (NHL) confined to the central nervous system (CNS) at diagnosis.1 Few cases of PCNSL following an initial diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), an inflammatory CNS condition with suspected autoimmune aetiology, have previously been described in the literature.2–4 In a recent study, 13 of 42 presumptive CLIPPERS cases were eventually diagnosed with various CLIPPERS mimics.3 NHL represented the largest subset of CLIPPERS mimics including two cases of PCNSL. Whether PCNSL represents a CLIPPERS mimic or complication remains subject of ongoing discussion. Some authors hypothesised CLIPPERS could be an early phase of CNS lymphomatoid granulomatosis (LYG) with subsequent Epstein-Barr virus (EBV)-associated neoplastic transformation into CNS lymphoma.5 Characteristics of PCNSL following CLIPPERS remain poorly defined. Here, we analysed 10 cases and explored implications for CLIPPERS management and PCNSL outcome.

A comprehensive review on PCNSL following CLIPPERS was independently conducted by two authors using the Medline database in compliance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines in August 2020. ‘CLIPPERS’ was used as the primary search term. Boolean operator ‘and’ was applied together with the secondary search term ‘lymphoma’ to narrow results. A total of 433 articles underwent screening for eligibility. Only cases with a diagnosis of ‘probable’ or ‘definite’ CLIPPERS according to updated diagnostic criteria and a tissue-based diagnosis of PCNSL or high-grade LYG were included.4 Eight articles matched inclusion criteria and were selected for data synthesis. One additional …

https://jnnp.bmj.com/content/jnnp/early/2021/05/13/jnnp-2020-325759.full.pdf

Sofia Doubrovinskaia

Papers

Neurological autoimmune diseases following vaccinations against SARS-CoV-2: a case series.

Primary CNS lymphoma after CLIPPERS: a case series.