scholarly journals Ganglionic Acetylcholine Receptor Antibodies and Autonomic Dysfunction in Autoimmune Rheumatic Diseases

2020 ◽  
Vol 21 (4) ◽  
pp. 1332 ◽  
Author(s):  
Michie Imamura ◽  
Akihiro Mukaino ◽  
Koutaro Takamatsu ◽  
Hiroto Tsuboi ◽  
Osamu Higuchi ◽  
...  

Autonomic neuropathy has been reported in autoimmune rheumatic diseases (ARD) including Sjögren’s syndrome, systemic sclerosis, rheumatoid arthritis, and systemic lupus erythematosus. However, the pathophysiological mechanism underlying autonomic dysfunction remains unknown to researchers. On the other hand, autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder, which causes dysautonomia that is mediated by autoantibodies against ganglionic acetylcholine receptors (gAChRs). The purpose of this review was to describe the characteristics of autonomic disturbance through previous case reports and the functional tests used in these studies and address the importance of anti-gAChR antibodies. We have established luciferase immunoprecipitation systems to detect antibodies against gAChR in the past and determined the prevalence of gAChR antibodies in various autoimmune diseases including AAG and rheumatic diseases. Autonomic dysfunction, which affects lower parasympathetic and higher sympathetic activity, is usually observed in ARD. The anti-gAChR antibodies may play a crucial role in autonomic dysfunction observed in ARD. Further studies are necessary to determine whether anti-gAChR antibody levels are correlated with the severity of autonomic dysfunction in ARD.

Rheumatology ◽  
2021 ◽  
Author(s):  
Jun Seok Park ◽  
Seulggie Choi ◽  
Kyuwoong Kim ◽  
Jooyoung Chang ◽  
Sung Min Kim ◽  
...  

Abstract Objective The primary objective is to investigate adverse effects of ambient particulate matter (PM) in various size on the incidence of prevalent autoimmune rheumatic diseases (AIRDs): Rheumatoid Arthritis (RA), Ankylosing Spondylitis (AS), and Systemic Lupus Erythematosus (SLE). Methods We investigated 230,034 participants in three metropolitan cities of South Korea from the National Health Insurance Service-National Sample Cohort (NHIS-NSC). Starting from January 2010, subjects were followed up until the first event of prevalent AIRDs, death, or December 2013. 2008-2009 respective averages of PM2.5 (< 2.5μm) and PMcoarse (2.5μm to 10μm) were linked with participants’ administrative district codes. Adjusted hazard ratios (aHRs) and 95% confidence intervals (CIs) were estimated using Cox regression analysis in one- and two-pollutant model. Results Adjusted for age, sex, region, and household income in two-pollutant model, RA incidence was positively associated with 10μg/m³ increment of PM2.5 (aHR = 1.74, 95% CI: 1.06-2.86), but not with PMcoarse (aHR = 1.27, 95% CI: 0.87-1.85). In one-pollutant model, an elevated incidence rate of RA was slightly attenuated (PM2.5 aHR = 1.61, 95% CI: 0.99-2.61; PMcoarse aHR = 1.13, 95% CI: 0.80-1.61), with marginal statistical significance of PM2.5. RA incidence was also higher in 4th quartile group of PM2.5 compared to 1st quartile group (aHR = 1.83, 95% CI: 1.07-3.11). No adverse effects of PM were found on AS or SLE in one- and two-pollutant models. Conclusion Important components of PM10 associated with RA incidence were fine fractions (PM2.5), while no positive association was found between PM and AS or SLE.


2020 ◽  
pp. jrheum.201017
Author(s):  
Michael D. George ◽  
Shilpa Venkatachalam ◽  
Shubhasree Banerjee ◽  
Joshua F. Baker ◽  
Peter A. Merkel ◽  
...  

Objective To assess concerns and healthcare-related behaviors of patients with autoimmune rheumatic diseases during the coronavirus disease 2019 (COVID-19) pandemic. Methods Adults from the United States with rheumatoid arthritis (RA), psoriatic arthritis (PsA), ankylosing spondylitis (AS), and systemic lupus erythematosus (SLE) from the ArthritisPower Patient-Powered Research Network and CreakyJoints patient community completed surveys. Concerns and behaviors were compared among patients with different autoimmune conditions, disease-modifying antirheumatic drug (DMARD) use, and geographic measures of urban status, income, education, and COVID-19 activity. Results Among 1517 participants (925 RA, 299 PsA, 185 AS, 108 SLE), mean age was 55.1 years, 88.3% were female, and 89.5% were White. COVID-19 concerns were similar across the country and were higher in biologic users (P < 0.001). Avoidance of doctor’s office visits (56.6%) or laboratory testing (42.3%) and use of telehealth (29.5%) were more common in urban areas. Among participants receiving a DMARD without COVID-19 or other respiratory illness, 14.9% stopped a DMARD, with 78.7% of DMARD interruptions not recommended by a physician. DMARD stopping was more common in participants with lower socioeconomic status (SES) and in participants who avoided an office visit (OR 1.46, 95% CI 1.04–2.04) or reported lack of telehealth availability OR 2.26 (95% CI 1.25–4.08). Conclusion In the early months of the COVID-19 pandemic, patients with RA, PsA, AS, and SLE frequently avoided office visits and laboratory testing. DMARD interruptions commonly occurred without the advice of a physician and were associated with SES, office visits, and telehealth availability, highlighting the need for adequate healthcare access and attention to vulnerable populations during the pandemic.


2011 ◽  
Vol 38 (8) ◽  
pp. 1612-1616 ◽  
Author(s):  
SASHA BERNATSKY ◽  
TINA LINEHAN ◽  
JOHN G. HANLY

Objective.To examine the validity of case definitions for systemic autoimmune rheumatic diseases [SARD; systemic lupus erythematosus (SLE), systemic sclerosis (SSc), myositis, Sjögren’s syndrome, vasculitis, and polymyalgia rheumatica] based on administrative data, compared to rheumatology records.Methods.A list of rheumatic disease diagnoses was generated from population-based administrative billing and hospitalization databases. Subjects who had been seen by an arthritis center rheumatologist were identified, and the medical records reviewed.Results.We found that 844 Nova Scotia residents had a diagnosis of one of the rheumatic diseases of interest, based on administrative data, and had had ≥ 1 rheumatology assessment at a provincial arthritis center. Charts were available on 824 subjects, some of whom had been identified in the administrative database with > 1 diagnosis. Thus a total of 1136 diagnoses were available for verification against clinical records. Of the 824 subjects, 680 (83%) had their administrative database diagnoses confirmed on chart review. The majority of subjects who were “false-positive” for a given rheumatic disease on administrative data had a true diagnosis of a similar rheumatic disease. Most sensitivity estimates for specific administrative data-based case definitions were > 90%, although for SSc, the sensitivity was 80.5%. The specificity estimates were also > 90%, except for SLE, where the specificity was 72.5%.Conclusion.Although health administrative data may be a valid resource, there are potential problems regarding the specificity and sensitivity of case definitions, which should be kept in mind for future studies.


2021 ◽  
Author(s):  
Claire T. Deakin ◽  
Georgina H. Cornish ◽  
Kevin W. Ng ◽  
Nikhil Faulkner ◽  
William Bolland ◽  
...  

AbstractDifferences in humoral immunity to coronaviruses, including severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), between children and adults remain unexplained and the impact of underlying immune dysfunction or suppression unknown. Here, we examined the antibody immune competence of children and adolescents with prevalent inflammatory rheumatic diseases, juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM) and juvenile systemic lupus erythematosus (JSLE), against the seasonal human coronavirus (HCoV)-OC43 that frequently infects this age group. Despite immune dysfunction and immunosuppressive treatment, JIA, JDM and JSLE patients mounted comparable or stronger responses than healthier peers, dominated by IgG antibodies to HCoV-OC43 spike, and harboured IgG antibodies that cross-reacted with SARS-CoV-2 spike. In contrast, responses to HCoV-OC43 and SARS-CoV-2 nucleoproteins exhibited delayed age-dependent class-switching and were not elevated in JIA, JDM and JSLE patients, arguing against increased exposure. Consequently, autoimmune rheumatic diseases and their treatment were associated with a favourable ratio of spike to nucleoprotein antibodies.


2020 ◽  
Author(s):  
Nina M de Gruijter ◽  
Meena Naja ◽  
Hannah Peckham ◽  
Anna Radziszewska ◽  
Matthew Kinsella ◽  
...  

Abstract Background: Autoimmune rheumatic diseases (ARDs) are associated with a significant sex-bias which becomes more evident post-puberty. This systematic review aims to elucidate the bidirectional relationship between puberty and ARDs related outcomes.Methods: Studies published in English until October 2019 were identified using a systematic search of endocrinology and rheumatology literature. Information was extracted on study design, sample size, demographics, puberty outcome measures, and main findings. The methodological quality of the studies included was analysed using the Newcastle-Ottawa Scale (NOS).Results: 14 non-randomised studies reporting on the impact of puberty on ARD outcomes (n = 7), ARD impact on puberty-related outcomes (n = 6), or both (n = 1) have been identified. The impact of puberty on ARD outcomes have been investigated in patients with juvenile idiopathic arthritis (JIA)-associated uveitis (n = 1), juvenile systemic lupus erythematosus (JSLE) (n = 5) or in healthy controls who developed adult-onset SLE (n = 1) or had non-specific symptoms (n = 1). The impact of ARD on puberty outcomes was explored in JIA (n = 4) and JSLE (n = 3). Quality assessment of studies showed a small to moderate risk of bias overall (NOS 4–9/9). Due to large heterogeneity of the studies it was not possible to perform a meta-analysis. Multiple studies reported on delayed puberty in patients with JIA/JSLE, menstrual and hormonal abnormalities, and lower height and weight than controls. Earlier (pre-pubertal) onset of JSLE was correlated with more severe disease and more need for systemic treatment.Conclusion: A bidirectional relationship exists between puberty and ARDs; however, more and better research is required to elucidate the complexity of this relationship. We propose puberty-related clinical assessments in patients with ARDs, which can improve patient outcomes and facilitate future research.


Author(s):  
José L. Pablos ◽  
Lydia Abasolo-Alcázar ◽  
José M. Álvaro-Gracia ◽  
Francisco J. Blanco ◽  
Ricardo Blanco ◽  
...  

ABSTRACTBackgroundThe susceptibility of patients with rheumatic diseases, and the risks or benefits of immunosuppressive therapies for COVID-19 are unknown.MethodsWe performed a retrospective study with patients under follow-up in rheumatology departments from seven hospitals in Spain. We matched updated databases of rheumatology patients with SARS-CoV-2 positive PCR tests performed in the hospital to the same reference populations. Rates of PCR+ confirmed COVID-19 were compared among groups.ResultsPatients with chronic inflammatory diseases had 1.32-fold higher prevalence of hospital PCR+ COVID-19 than the reference population (0.76% vs 0.58%). Systemic autoimmune or immune mediated diseases (AI/IMID) patients showed a significant increase, whereas inflammatory arthritis (IA) or systemic lupus erythematosus (SLE) patients did not. COVID-19 cases in some but not all diagnostic groups had older ages than cases in the reference population. IA patients on targeted-synthetic or biological disease-modifying antirheumatic drugs (ts/bDMARD), but not those on conventional-synthetic (csDMARD), had a greater prevalence despite a similar age distribution.ConclusionPatients with AI/IMID show a variable risk of hospital diagnosed COVID-19. Interplay of aging, therapies, and disease specific factors seem to contribute. These data provide a basis to improve preventive recommendations to rheumatic patients and to analyze the specific factors involved in COVID-19 susceptibility.


2010 ◽  
Vol 69 (12) ◽  
pp. 2062-2066 ◽  
Author(s):  
Lars Klareskog ◽  
Peter K Gregersen ◽  
Tom W J Huizinga

Prevention of disease can in principle be accomplished by identification of environmental and/or lifestyle risk and protective factors followed by public health measures (such as for smoking and lung cancer), or by modification of the individual's reactions to disease-inducing factors (such as in vaccinations against microbes). This review discusses both options based on emerging understanding of aetiologies in inflammatory rheumatic diseases such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). The major current opportunity for public health-based prevention lies in avoiding smoking. In RA, recent studies have calculated that, in Sweden (a country characterised by a low frequency of smoking), 20% of all RA cases and 33% of all cases of ACPA-positive RA would not have occurred in a smoke-free society. Smoking is also a major risk factor for SLE but no population attribution is yet available. New avenues for individualised and biology-based prevention are provided by the demonstration that several autoimmune rheumatic diseases are preceded by emergence of subclinical autoimmunity followed by laboratory-based signs of inflammation and finally overt disease. Examples of this process are provided from studies of autoimmunity to citrullinated proteins (in RA), to dsDNA (in SLE in general) and to Ro52 epitopes (in the case of neonatal heart block). The recognition of this sequence of events provides opportunities to intervene specifically and potentially curatively before onset of full-blown disease. Such prevention can be accomplished by modification of inciting antigens (environment), by modification of immunity (more or less specific immunomodulation) or by modification of specific gene functions. In all cases, prevention will be different in different subsets of disease and differ at different time points of disease development. Thus, the road map towards prevention of autoimmune rheumatic diseases includes increased understanding of how genes, environment and immunity interact.


2021 ◽  
Vol 72 (1) ◽  
pp. 1-5
Author(s):  
Božidar Pocevski ◽  
Slavica Pavlov-Dolijanović

Introduction: Silicone breast implants (SBI) have been used since 1962 in the reconstruction of post-mastectomy cases, in augmentation of the breast, or for cosmetic purposes, while fillers with biopolymer (FB) have been used since the 1990s. Today, they are considered adjuvants of the immune system. Most complications of SBI and FB are local in nature, but some patients experience systemic symptoms, which are defined as adjuvant-induced autoimmune inflammatory syndrome (ASIA). Aim: The aim of this study is to demonstrate the possible association of silicone breast implants and FB with the development of immune-mediated inflammatory rheumatic diseases (IMIRD). Material and methods: The research represents retrospective study which involved 15 female patients with immune-mediated inflammatory rheumatic diseases, 6 of whom were patients with implanted silicone breast implants for cosmetic reasons, and 9 patients with placement of fillers with biopolymer on the lips. Results: The average time from silicone implantation to the onset of the first symptoms was 6.10 ± 5.3 (range 6 months to 24 years). The following immune-mediated inflammatory rheumatic diseases were recorded: 3 patients with systemic sclerosis (SSc), 3 patients with undifferentiated arthritis, 3 patients with seropositive rheumatoid arthritis, 1 patient with systemic lupus erythematosus, 2 patients with undifferentiated SCTD, 2 patients with mixed connective tissue disease, and one patient with unexplained systemic disease. Seven patients had the Raynaud phenomenon. Spontaneous abortions were reported in 2 patients. Conclusion: Earlier reports that silicone breast implants and biopolymer fillers are safe, today are changing with the description of ASIA syndrome.


2021 ◽  
Vol 12 ◽  
Author(s):  
Delia Tulbă ◽  
Bogdan Ovidiu Popescu ◽  
Emilia Manole ◽  
Cristian Băicuș

Immune axonal neuropathies are a particular group of immune-mediated neuropathies that occasionally accompany systemic autoimmune rheumatic diseases such as connective tissue dissorders and primary systemic vasculitides. Apart from vasculitis of vasa nervorum, various other mechanisms are involved in their pathogenesis, with possible therapeutic implications. Immune axonal neuropathies have highly heterogeneous clinical presentation and course, ranging from mild chronic distal sensorimotor polyneuropathy to severe subacute mononeuritis multiplex with rapid progression and constitutional symptoms such as fever, malaise, weight loss and night sweats, underpinning a vasculitic process. Sensory neuronopathy (ganglionopathy), small fiber neuropathy (sensory and/or autonomic), axonal variants of Guillain-Barré syndrome and cranial neuropathies have also been reported. In contrast to demyelinating neuropathies, immune axonal neuropathies show absent or reduced nerve amplitudes with normal latencies and conduction velocities on nerve conduction studies. Diagnosis and initiation of treatment are often delayed, leading to accumulating disability. Considering the lack of validated diagnostic criteria and evidence-based treatment protocols for immune axonal neuropathies, this review offers a comprehensive perspective on etiopathogenesis, clinical and paraclinical findings as well as therapy guidance for assisting the clinician in approaching these patients. High quality clinical research is required in order to provide indications and follow up rules for treatment in immune axonal neuropathies related to systemic autoimmune rheumatic diseases.


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