Rare Cases of Polymyalgia Rheumatica After Receiving COVID-19 Vaccinations.

Polymyalgia rheumatica (PMR) is a systemic rheumatic inflammatory disease of adults presenting with symmetrical proximal muscle stiffness and pain predominantly involving the shoulders, neck, and pelvic girdle. The coronavirus disease of 2019 (COVID-19) presented as a pandemic causing worldwide morbidity and mortality in large numbers. Rapid scientific research expedited preventative vaccine development and has helped tremendously in cutting down severe illness, hospitalizations, and death from COVID-19, with the messenger ribonucleic acid (mRNA) vaccines outperforming the others. We present two cases that showcase the incidence of polymyalgia rheumatica after receiving COVID-19 vaccination. Patient 1 is a 69-year-old female who developed arm and thigh stiffness a week before the second dose while receiving her primary Moderna vaccine series. She had an elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), so she was started on low-dose steroids, which were weaned down over a five-month period. Three weeks after receiving her Moderna booster, she had a recurrence of the classic polymyalgia rheumatica symptoms and elevated ESR. She responded to prednisone 15 mg with a successful taper over eight months. Patient 2 is a 74-year-old male who received his primary series and booster through Pfizer-BioNTech. Prior to the booster, he was treated for COVID-19 with monoclonal antibody therapy. He presented to the office with hip and shoulder pain and stiffness along with an elevated C-reactive protein. Consequently, he received 20 mg of prednisone but needed to increase his dose to 25 mg total to help with the control of his inflammation. The goal of this article is to prompt physicians about the possibility of PMR incidence after patients receive vaccinations for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). PMR can be debilitating to the quality of life of patients. Knowing this association allows for more timely and competent treatment. PMR following SARS-CoV-2 vaccinations is continuously being observed in the medical field. Increased knowledge may help prevent the recurrence with subsequent doses. Further studies on the follow-up of such cases and the effect on subsequent immunization would be helpful.

Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) in 2023.

In 2011, a syndrome entitled ASIA (Autoimmune/inflammatory Syndrome Induced by Adjuvants; Shoenfeld's syndrome) was first described. ASIA aimed to organize under a single umbrella, the existing evidence regarding certain environmental factors which possess immune stimulatory properties, in order to shed light on a common pathway of autoimmune pathogenesis. Such environmental immune stimulators, or adjuvants, include among others: aluminum salts as in vaccines, various medical implants, as well as various infectious agents. After the launch of the ASIA syndrome, the expansion and recognition of this syndrome by different researchers from different countries began. During the past decades, evidence had been accumulating that (auto)immune symptoms can be triggered by exposure to environmental immune stimulatory factors that act as an adjuvant in genetically susceptible individuals. A panoply of unexplained subjective and autonomic-related symptoms has been reported in patients with ASIA syndrome. The current review summarizes and updates accumulated knowledge from the past decades, describing new adjuvants- (e.g. polypropylene meshes) and vaccine- (e.g. HPV and COVID vaccines) induced ASIA. Furthermore, a direct association between inflammatory/autoimmune diseases with ASIA syndrome, will be discussed. Recent cases will strengthen some of the criteria depicted in ASIA syndrome such as clear improvement of symptoms by the removal of adjuvants (e.g. silicone breast implants) from the body of patients. Finally, we will introduce additional factors to be included in the criteria for ASIA syndrome such as: (1) dysregulated non-classical autoantibodies directed against G-protein coupled receptors (GPCRs) of the autonomic nervous system and (2)) small fiber neuropathy (SFN), both of which might explain, at least in part, the development of 'dysautonomia' reported in many ASIA patients.

Hemophagocytic Lymphohistiocytosis in a Patient With Post-acute COVID-19 Infection.

Hemophagocytic lymphohistiocytosis (HLH) is characterized by immune dysregulation with extensive inflammation and tissue destruction due to abnormal immune activation. Post-COVID-19 patients who have recovered with negative serologic tests may also present with secondary HLH, an unusual finding that our case demonstrates. A 73-year-old male with a notable past medical history of fall COVID-19 infection approximately 11 months prior presented initially to emergency services with a chief complaint of high fevers, lethargy, and progressive changes in mentation gradually progressive over the last 5 months' duration. This presentation was concerning for HLH in view of the patient's high H score and clinical suspicion for HLH, and he was initiated on dexamethasone 20 mg daily. intravenous immune globulin (IVIG) protocol was also trialed, despite which the patient continued to deteriorate before expiring during the course of the hospitalization. sHLH following COVID-19 infection remains a poorly understood phenomenon. The severity of the COVID-19 infection does not appear to be related to one's predisposition to develop sHLH. The mortality of HLH remains high even with appropriate therapy.

SOCS1 Haploinsufficiency Presenting as Severe Enthesitis, Bone Marrow Hypocellularity, and Refractory Thrombocytopenia in a Pediatric Patient with Subsequent Response to JAK Inhibition.

Haploinsufficiency of suppressor of cytokine signaling 1 (SOCS1) is a recently discovered autoinflammatory disorder with significant rheumatologic, immunologic, and hematologic manifestations. Here we report a case of SOCS1 haploinsufficiency in a 5-year-old child with profound arthralgias and immune-mediated thrombocytopenia unmasked by SARS-CoV-2 infection. Her clinical manifestations were accompanied by excessive B cell activity, eosinophilia, and elevated IgE levels. Uniquely, this is the first report of SOCS1 haploinsufficiency in the setting of a chromosomal deletion resulting in complete loss of a single SOCS1 gene with additional clinical findings of bone marrow hypocellularity and radiologic evidence of severe enthesitis. Immunologic profiling showed a prominent interferon signature in the patient's peripheral blood mononuclear cells, which were also hypersensitive to stimulation by type I and type II interferons. The patient showed excellent clinical and functional laboratory response to tofacitinib, a Janus kinase inhibitor that disrupts interferon signaling. Our case highlights the need to utilize a multidisciplinary diagnostic approach and consider a comprehensive genetic evaluation for inborn errors of immunity in patients with an atypical immune-mediated thrombocytopenia phenotype.

Familial mediterranean fever and COVID-19: A monocentric Italian study

Introduction: The severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) is the causative agent of the coronavirus disease 2019 (COVID-19). Its rapid spreading all over the world has caused a pandemia that has a deleterious impact on public health worldwide. The COVID-19 is associated with cytokines dysregulation, increased IL-1β tumor necrosis factor (TNF) and IL-6 release with hyperinflammation and risk of cytokine storm syndrome. Autoinflammatory diseases (AID) are rare, potentially life-threatening conditions caused by pathogenic gene variants encoding for inflammasomes leading to excessive production of pro-inflammatory cytokines. The innate immune system that plays a critical role in the pathogenesis of Familiar Mediterranean Fever (FMF) is also essential in antiviral defense. Colchicine is the main drug therapy for FMF and fulfills its role with the inhibition of microtubule polymerization and pyrin inflammasome. Colchicine effects on COVID-19 have not yet been evaluated in trials, but some studies on COVID-19 patients under colchicine treatment for FMF arouse interest. Anti-SARS-COV-2 vaccination is of key role for COVID-19 eradication worldwide, few data are available for AID patients. Objectives: This study aims to describe the COVID-19 impact on patients under regular treatment for AID and their outcome after anti- SARS-COV-2 vaccination. Methods: An observational monocentric study has been conduct on a cohort of Italian AID patients who were under regular follow up for their disorders. Data have been collected both retrospectively and prospectively. Results: Seventy-eight adult AID patients (65 FMF, 5 PFAPA, 3 FCAS, 2 TRAPS, 2 Behcet Disease, 1 SAPHO,) followed at Center for Primary Immunodeficiency and Autoinflammatory disorders have been considered. Among FMF patients, eight communicated to the center when they got COVID-19 and were followed by regular phone calls. The mean age was 36.9 years, (range 27-60) and they had no other comorbidities. All the patients were on regular colchicine therapy and were on complete remission before COVID-19. They were started with azithromycin (with precaution for colchicine interference) and four were treated also with low-weight heparin prophylaxis. None of the patients need oxygen or hospitalization. Two sisters (MEFV genotype M694V/V726A) had FMF attack relapsing during COVID-19 disease, despite regular colchicine therapy. All the cases recovered from COVID-19 without complications. None of the other AID patients declared to have COVID-19, and all the swabs, that they did when they had symptoms suspected for, were negative. Genetics of FMF patients' with COVID-19 - 2 M694V/V726A - 2 M694V Pediatric Rheumatology 2021, 19(Suppl 1):155 Page 205 of 229 - 1 M694V/M694V - 1 M680IGC/E148Q - 1 R761H/E148Q - 1 -/- Till now 31 AID patients have been vaccinated (22 FMF, 4 PFAPA, 3 FCAS, 2 Behcet disease). No adverse reactions were observed. All the patients who had COVID-19 have been vaccinated (Pfizer/ Biontech or Moderna), two had FMF attack. Two patients with PFAPA had a mild attack. Post-vaccination titre was checked after 4 week since 2nd dose for those patients who were under anti-IL inhibitors (n=5) or anti-TNF (n=1). Conclusion: In this study, the FMF patients who had COVID-19 under chronic colchicine treatment had a mild/moderate disease and recovered without consequences. According to other studies, FMF under colchicine treatment seems not to be a risk factor for severe COVID-19 and colchicine anti-inflammatory effect worths to be considered for treatment in COVID-19. Anti-SARS-COV-2 vaccination in AID patients of this study has been observed to be safe and effective.

The Immunogenicity of Hyaluronic Fillers and Its Consequences.

Hyaluronic acid (HA) is a glycosaminoglycan, a natural component of the extracellular matrix. The identical structure of the molecule in all living organisms is its main advantage, as it translates into the minimal probability of immunogenicity. Therefore, it is the closest to the ideal preparation used as a filler, due to its biocompatibility and stability at the site of implantation. This paper includes the discussion of the potential mechanisms of adverse immune reactions to HA along with the mechanisms of reaction following vaccinations against SARS-CoV-2. Based on the literature, we tried to systematize adverse immune reactions with systemic manifestations to HA. The occurrence of unpredictable reactions to hyaluronic acid indicates that they may not be treated as neutral or non-allergenic. The modifications of the chemical structure of HA, additives and individual tendencies in a patient may be the cause of unpredictable reactions, leading to serious health consequences. Preparations of unknown origin, poorly purified, or including bacterial DNA are particularly dangerous. Therefore, long-lasting follow-up of the patient and the selection of a preparation approved by the FDA or EMA are of high importance. Patients are often unaware of the consequences of cheaper procedures performed by persons without suitable knowledge with the use of unregistered products, so the public should be educated and legal regulations should be introduced.