Primary Sjögren syndrome‐related peripheral neuropathy: a systematic review and meta‐analysis

Background and purpose Primary Sjögren syndrome (pSS) is a chronic, systemic, autoimmune disorder characterized by lymphocytic infiltrates of the exocrine organs, leading to sicca symptoms and parotid enlargement. pSS has been linked to various neurological manifestations, including peripheral neuropathy (PN). We aimed to provide a comprehensive analysis of the currently available evidence regarding pSS-related PN. Methods A literature search in the PubMed database was performed, and 49 papers were eligible to be included in this systematic review and meta-analysis. Results The pooled prevalence of PN in pSS is estimated to be 15.0% (95% confidence interval = 10.7%–20.7%). The mean age of pSS patients at PN diagnosis is 59 years. Among the patients with pSS and PN, 83% are females. Neuropathic symptoms usually precede or lead to the pSS diagnosis at a 2:1 ratio in patients with pSS-related PN. The commonest type of pSS-related PN is distal axonal polyneuropathy (80% of patients with pSS-related PN), followed by sensory ganglionopathy. Peripheral and cranial mononeuropathies—particularly trigeminal—are also frequent. Risk factors for developing PN include increasing age and presence of vasculitis. Immune-mediated pathogenetic mechanisms are discussed. Glucocorticoids are the most commonly used treatment option for managing pSS-related PN, when associated with vasculitis, followed by the use of intravenous immunoglobulin. Conclusions PN is very common in pSS patients. Evidence on long-term prognosis of PN in pSS is limited, and further research is needed. Research into the use of immunosuppressive medication in nonvasculitic neuropathies in the context of pSS merits further consideration

Workup and Clinical Assessment for Allergen Immunotherapy Candidates

Allergen Immunotherapy (AIT) is a well-established, efficient, and safe way to treat respi-ratory and insect-venom allergies. After determining the diagnosis of the clinically relevant culprit allergen, AIT can be prescribed. However, not all patients are eligible for AIT, since some dis-eases/conditions represent contraindications to AIT use, as described in several guidelines. Allergists are often preoccupied on whether an extensive workup should be ordered in apparently healthy AIT candidates in order to detect contra-indicated diseases and conditions. These preoccupations often arise from clinical, ethical and legal issues. The aim of this article is to suggest an approach to the workup and assessment of the presence of any underlying diseases/conditions in patients with no case history before the start of AIT. Notably, there is a lack of published studies on the appropriate evaluation of AIT candidates, with no globally accepted guidelines. It appears that Allergists are mostly deciding based on their AIT training, as well as their clinical experience. Guidance is based mainly on experts’ opinions; the suggested preliminary workup can be divided into mandatory and optional testing. The evaluation for possible underlying neoplastic, autoimmune, and cardiovascular diseases, primary and acquired immunodeficiencies and pregnancy, might be helpful but only in subjects for whom the history and clinical examination raise suspicion of these conditions. A workup without any reasonable correlation with potential contraindications is useless. In conclusion, the evaluation of each individual candidate for possible medical conditions should be determined on a case-by-case basis. © 2022 by the authors. Licensee MDPI, Basel, Switzerland

Immunoglobulin Use for the Management of Painful Peripheral Neuropathy: A Systematic Review and Meta-Analysis

Background: Immunoglobulins (IG) are widely used for the treatment of a variety of immune-mediated diseases. The exact mechanism of action remains unknown, but IG modulate the expression and function of Fc receptors, interfere with complement activation and production of cytokines, neutralize pathogenic autoantibodies, and affect the activation and effector functions of B and T lymphocytes. Immunoglobulins are usually delivered intravenously, and are effective in ameliorating motor symptoms, and/or preventing disease progression in immune-mediated neuropathies, including Guillain–Barré syndrome and chronic inflammatory demyelinating polyneuropathy. Objective: The aim of this systematic review and meta-analysis was to study the potential of IG for the treatment of painful peripheral neuropathy (PPN). The outcome of interest was the percentage of patients with PPN who achieved pain relief following IG administration. Methods: We performed a systematic literature search on March 17, 2022, in the PubMed database without any publication date restrictions. We also looked for unpublished or ongoing trials in clinicaltrials.org. Pain reduction following IG treatment had to be within the aims (primary or secondary). Results: The aforementioned literature search strategy revealed five studies (two open-label, three randomized placebo-controlled) eligible to be included. The pooled estimate of the percentage of patients with PPN who received immunoglobulins and reported pain relief was found to be 65% (95% CI 58–71%). The likelihood of achieving pain relief with immunoglobulin treatment was 2.9 times higher (95% CI 1.6–5.2) compared to placebo (p = 0.0003). Conclusion: The use of IG for the treatment of pain due to peripheral neuropathy has a potential therapeutic benefit. Further studies across patients with different types of painful peripheral neuropathy are needed to better characterize this effect. Registration number on PROSPERO: CRD42022319614. © 2022, The Author(s)

Correction to: Primary Sjögren’s syndrome (pSS)-related cerebellar ataxia: a systematic review and meta-analysis (Acta Neurologica Belgica, (2021), 10.1007/s13760-021-01784-1)

The first name and family name of all authors have been incorrectly swapped in the original publication. The complete correct author names are given below. Andreas Liampas, Antonios Nteveros, Konstantinos Parperis, Mohammed Akil, Efthymios Dardiotis, Elizabeth Andreadou, Marios Hadjivassiliou, Panagiotis Zis. The original article has been corrected. © 2021, Belgian Neurological Society

Primary Sjögren’s syndrome (pSS)-related cerebellar ataxia: a systematic review and meta-analysis

Background: Primary Sjögren’s syndrome (pSS) is a chronic autoimmune disorder characterized by lymphocytic infiltrates of the exocrine glands, particularly the salivary and lacrimal glands, resulting in oral and ocular dryness. pSS has been linked to various neurological manifestations, including cerebellar dysfunction. We aimed to provide a comprehensive analysis of the currently available evidence regarding pSS-related cerebellar ataxia. Methods: A systematic literature search in the PubMed database was performed and 19 papers were eligible to be included in this paper. Results: The pooled prevalence of cerebellar ataxia in pSS is estimated to be 1.5% (95% CI 0.3–6.8%). pSS patients with cerebellar involvement have a female-to-male ratio of 6:1. Although most of the patients are adults in their fifth decade of life when diagnosed, cases of children with pSS and cerebellar involvement have been reported. Typical cerebellar ataxia related to pSS manifests with vermian dysfunction, namely gait ataxia and/or cerebellar speech. Cerebellar ataxia due to pSS may also mimic degenerative cerebellar ataxia, especially when the onset is progressive. Conclusions: The diagnostic approach to a patient with cerebellar ataxia of unknown etiology should include evaluation for an underlying pSS. A thorough history and clinical examination, antibody testing, brain MRI imaging and/or MRS of the cerebellum will assist in establishing the diagnosis. Setting up a joint neuro-rheumatology clinic can be valuable given that rheumatic and neurological diseases share comorbidities. © 2021, Belgian Neurological Society

Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis

Background and purpose: Primary Sjögren syndrome (pSS) is a chronic, systemic, autoimmune disorder characterized by lymphocytic infiltrates of the exocrine organs, leading to sicca symptoms and parotid enlargement. pSS has been linked to various neurological manifestations, including peripheral neuropathy (PN). We aimed to provide a comprehensive analysis of the currently available evidence regarding pSS-related PN. Methods: A literature search in the PubMed database was performed, and 49 papers were eligible to be included in this systematic review and meta-analysis. Results: The pooled prevalence of PN in pSS is estimated to be 15.0% (95% confidence interval = 10.7%–20.7%). The mean age of pSS patients at PN diagnosis is 59 years. Among the patients with pSS and PN, 83% are females. Neuropathic symptoms usually precede or lead to the pSS diagnosis at a 2:1 ratio in patients with pSS-related PN. The commonest type of pSS-related PN is distal axonal polyneuropathy (80% of patients with pSS-related PN), followed by sensory ganglionopathy. Peripheral and cranial mononeuropathies—particularly trigeminal—are also frequent. Risk factors for developing PN include increasing age and presence of vasculitis. Immune-mediated pathogenetic mechanisms are discussed. Glucocorticoids are the most commonly used treatment option for managing pSS-related PN, when associated with vasculitis, followed by the use of intravenous immunoglobulin. Conclusions: PN is very common in pSS patients. Evidence on long-term prognosis of PN in pSS is limited, and further research is needed. Research into the use of immunosuppressive medication in nonvasculitic neuropathies in the context of pSS merits further consideration. © 2022 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology

Acceptance and willingness to purchase a hypothetical COVID-19 vaccine in a region under Shariah law: A cross-sectional study in Aceh, Indonesia

Vaccines are urgently needed to control the coronavirus disease 2019 (COVID-19) pandemic. The aim of this study was to determine the acceptance of and willingness to purchase a hypothetical COVID-19 vaccine in the general population of Aceh, a holistic Shariah law implementation province in Indonesia. An online cross-sectional study was conducted using a quota sampling technique between 1 to 24 September 2021. To determine hypothetical vaccine acceptance, respondents were asked if they were willing to accept vaccines with combinations of either 50% or 95% effectiveness and either 5% or 20% risk of adverse effects. Willingness to purchase was assessed by asking whether the participants would pay for such vaccines at certain price points. Logistic regression analysis was used to assess the associated determinants. Out of 377 respondents included in the final analysis, 86.5% were willing to accept a COVID-19 vaccine with 95% effectiveness and 5% adverse effects. The acceptance rate dropped to 45.1% if the risk of adverse effects was 20%. Vaccines with 50% effectiveness and 5% adverse effects were acceptable to 42.2% but the acceptance went down to 17.2% if the risk of adverse effects increased to 20%. Multivariate analysis found that men were twice as likely to accept a vaccine with 95% effectiveness and 5% adverse effects compared to females (aOR: 2.01; 95% CI 1.05–3.86). We found that 156/377 (41.3%) of respondents were willing to purchase a COVID-19 vaccine and of these participants 71.1% were willing to pay between Indonesian Rupiah (IDR) 50,000–150,000 (US$ 3.33–10.00). In conclusion, the acceptance rate of a hypothetical COVID-19 vaccine varied based on effectiveness and the risk of adverse effects