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Medicine Dec 2023Immune system deregulation, including AAV, is a key event that may potentially evolve into ESRD. Abnormal activation of the cAP is also a cardinal feature of TMA,...
End stage renal disease in patient with microscopic polyangiitis and atypical hemolytic-uremic syndrome arose 3 weeks after the third dose of anti-SARS-CoV2 vaccine mRNA-1273: A case report with literature revision.
RATIONALE
Immune system deregulation, including AAV, is a key event that may potentially evolve into ESRD. Abnormal activation of the cAP is also a cardinal feature of TMA, particularly aHUS. The kidney is the most frequently involved organ, and renal-limited forms of TMA are often encountered in clinical practice. Isolated case reports described the occurrence of renal TMA in AAV patients. Some cases of both de novo and relapses of AAV and/or TMAs after anti-SARS-CoV2 vaccination have been reported. We reported, for the 1st time, a case of patients with new-onset MPA and aHUS occurring 3 weeks after the third dose of mRNA-1273 vaccine anti-SARS-CoV2.
PATIENT CONCERNS
We present a 67-year-old man, affected by arterial hypertension, reported, after mRNA-1273 vaccine anti-SARS-CoV2, anuria, fatigue, anorexia and nausea. Laboratory data revealed acute renal failure.
DIAGNOSIS
Positivity of MPO-ANCA was observed. 7 days after admission, we observed a worsening of anemia and thrombocytopenia with haptoglobin reduction, LDH increase and presence of schistocytes. Plasma levels of ADAMTS-13 were normal. A renal biopsy was performed, and findings were consistent with microscopic polyangiitis, with features of micro-thrombotic glomerulopathy. Genetic tests revealed absence of hybrid genes associated with the increased risk of aHUS.
INTERVENTIONS AND OUTCOMES
We started renal replacement treatment, including hemodialysis, and pulsed methylprednisolone, with no improvement of laboratory parameters. Then, plasma exchange was performed leading to partial haematological response. Only with Eculizumab, a human C5 inhibitor, we observed a normalization of haptoglobin levels and platelets' count. However, three months after discharge, the patient still required hemodialysis.
LESSONS
To our knowledge we observed the first case aHUS, without genetic predisposition, associated with MPA occurring after the third dose of anti-SARS-CoV2 vaccine. This case report highlights the potential link between anti-SARS-CoV2 vaccine as a trigger of MPA and aHUS. This systematic review offers additional perspectives. It is plausible to hypothesize that the vaccine was the trigger for the development of these 2 diseases.Solid evidence on the mechanisms of interaction between vaccine and immune system, the role of genetic predisposition, and other variables, will shed additional light on the controversial link between anti-SARS-CoV2 vaccine and autoimmunity.
Topics: Male; Humans; Aged; Atypical Hemolytic Uremic Syndrome; 2019-nCoV Vaccine mRNA-1273; Microscopic Polyangiitis; Haptoglobins; COVID-19; Kidney Failure, Chronic; Genetic Predisposition to Disease
PubMed: 38115241
DOI: 10.1097/MD.0000000000036560 -
Neurology(R) Neuroimmunology &... Jan 2024We characterize clinical and neuroimaging features of SARS-CoV-2-related acute necrotizing encephalopathy (ANE).
BACKGROUND AND OBJECTIVES
We characterize clinical and neuroimaging features of SARS-CoV-2-related acute necrotizing encephalopathy (ANE).
METHODS
Systematic review of English language publications in PubMed and reference lists between January 1, 2020, and June 30, 2023, in accordance with PRISMA guidelines. Patients with SARS-CoV-2 infection who fulfilled diagnostic criteria for sporadic and genetic ANE were included.
RESULTS
From 899 articles, 20 cases (17 single case reports and 3 additional cases) were curated for review (50% female; 8 were children). Associated COVID-19 illnesses were febrile upper respiratory tract infections in children while adults had pneumonia (45.6%) and myocarditis (8.2%). Children had early neurologic deterioration (median day 2 in children vs day 4 in adults), seizures (5 (62.5%) children vs 3 of 9 (33.3%) adults), and motor abnormalities (6 of 7 (85.7%) children vs 3 of 7 (42.9%) adults). Eight of 12 (66.7%) adults and 4 (50.0%) children had high-risk ANE scores. Five (62.5%) children and 12 (66.7%) adults had brain lesions bilaterally and symmetrically in the putamina, external capsules, insula cortex, or medial temporal lobes, in addition to typical thalamic lesions of ANE. Hypotension was only seen in adults (30%). Hematologic derangements were common: lymphopenia (66.7%), coagulopathy (60.0%), or elevated D-dimers (100%), C-reactive protein (91.7%), and ferritin (62.5%). A pathogenic heterozygous c/.1754 C>T variant in was present in 2 children: one known to have this before SARS-CoV-2 infection, and a patient tested because the SARS-CoV-2 infection was the second encephalopathic illness. Three other children with no prior encephalopathy or family history of encephalopathy were negative for this variant. Fifteen (75%) received immunotherapy (with IV methylprednisolone, immunoglobulins, tocilizumab, or plasma exchange): 6 (40.0%) with monotherapy and 9 (60.0%) had combination therapy. Deaths were in 8 of 17 with data (47.1%): a 2-month-old male infant and 7 adults (87.5%) of median age 56 years (33-70 years), 4 of whom did not receive immunotherapy.
DISCUSSION
Children and adults with SARS-CoV-2 ANE have similar clinical features and neuroimaging characteristics. Mortality is high, predominantly in patients not receiving immunotherapy and at the extremes of age.
Topics: Adult; Child; Child, Preschool; Female; Humans; Infant; Male; Middle Aged; Brain Diseases; COVID-19; Methylprednisolone; SARS-CoV-2; Seizures; Aged
PubMed: 38086061
DOI: 10.1212/NXI.0000000000200186 -
Journal of Clinical Medicine Nov 2023Percutaneous treatment for primary aneurysmal bone cysts (ABCs) has been widely accepted. The study aimed to evaluate the efficacy of various sclerotherapy agents on... (Review)
Review
BACKGROUND
Percutaneous treatment for primary aneurysmal bone cysts (ABCs) has been widely accepted. The study aimed to evaluate the efficacy of various sclerotherapy agents on patients with primary ABCs.
METHODS
A meta-analysis of relevant studies. A systematic search was conducted on five databases, resulting in the inclusion of 25 studies with different percutaneous agents.
RESULTS
A total of 729 patients with primary ABCs were included. Patients were administered with Ethibloc, doxycycline, embolization, alcohol, polidocanol, and calcitonin with methylprednisolone, respectively. Overall, 542 (74.3%) patients with ABCs had complete healing, 120 (16.4%) had partial healing, 44 (6%) had no-ossification or failure, and 26 (3.5%) had a recurrence. However, there was a total of 45 (6.1%) patients who had surgical curettage after sclerotherapy. Among the sclerotherapy agents, doxycycline showed highly effective results with minimal complications and recurrence, but it required multiple injections per patient. Ethibloc and embolization also proved to be highly effective with fewer injections required but had a higher rate of complications. Absolute alcohol, polidocanol, and calcitonin with methylprednisolone had similar efficacity and favorable success with fewer complications and fewer injections.
CONCLUSION
Percutaneous treatment showed promising results in treating primary ABCs. However, more robust research is needed to establish the best approach for sclerotherapy in clinical practice and to address the limitations of the current literature.
PubMed: 38068264
DOI: 10.3390/jcm12237213 -
Frontiers in Human Neuroscience 2023The number of reported cases of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis has gradually increased since its discovery in 2007, while there are no... (Review)
Review
BACKGROUND
The number of reported cases of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis has gradually increased since its discovery in 2007, while there are no uniform treatment guidelines.
OBJECTIVE
To summarize the clinical characteristics of patients with anti-NMDAR encephalitis and to analyze the factors affecting the disease prognosis.
METHODS
A systematic analysis of medical records was conducted, and PubMed, Embase, and Cochrane Library were searched from January 1, 2011, to December 31, 2021. Data were extracted, analyzed, and recorded in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines.
RESULTS
This study included 472 case reports. Most patients had prodromal symptoms of about 2 weeks, including psychiatric symptoms (53.2%), flu-like symptoms (51.5%), and seizures (23.9%), among others. Poor prognoses were associated with patients who had autonomic instability ( = 0.010), central hypoventilation ( = 0.014), and ICU support ( = 0.002). Patients with a higher age of onset were more likely to develop central hypoventilation (OR 1.024, CI 1.006-1.042, = 0.009), cognitive impairment (OR 1.023, CI 1.009-1.037, = 0.001), and memory impairment (OR 1.034, CI 1.017-1.050, < 0.001), whereas patients with a lower age were more likely to have seizures (OR 0.979, CI 0.965-0.993, = 0.003). In this study, 97.0% of patients received immunotherapy, with the most commonly used treatment regimen being intravenous methylprednisolone (IVGC) and intravenous immunoglobulin (IVIG). When compared with other treatment regimens, the IVGC+IVIG regimen ( < 0.001) resulted in better prognoses.
CONCLUSION
When encountering patients with fever, headache, and initial psychiatric symptoms of unknown etiology, clinicians should test their CSF for antibodies to distinguish autoimmune encephalitis. Patients with autonomic instability, central hypoventilation, and ICU support had poorer prognoses. Clinicians should be aware that older patients are more likely to develop central hypoventilation, cognitive impairment, and memory impairment, while younger patients are more likely to develop seizures. The IVGC+IVIG treatment regimen has better prognoses than others. This study includes case reports, which have obvious selection bias, and there are no unified standards to measure the severity of the disease. Therefore, in the future, larger samples and randomized controlled trials are needed to evaluate the efficacy of different treatment regimens.
PubMed: 38053649
DOI: 10.3389/fnhum.2023.1261638 -
Frontiers in Neurology 2023Relapsing polychondritis (RP) is a rare rheumatologic disorder that may affect the neurological system with various presentations. In this study, we present a case and...
BACKGROUND AND PURPOSE
Relapsing polychondritis (RP) is a rare rheumatologic disorder that may affect the neurological system with various presentations. In this study, we present a case and summarize the clinical characteristics of RP-associated meningoencephalitis.
CASE PRESENTATION
A 48-year-old man presented with first-ever seizures that were well controlled by valproate. Physical examination results were unremarkable, except for binaural deformation. The initial brain magnetic resonance imaging (MRI) without contrast and electroencephalogram (EEG) findings were normal. However, the patient subsequently developed recurrent fever, scleritis, headache, lethargy, and left arm paresis. Repeated brain MRI with contrast demonstrated increased enhancement of the pia mater and abnormal diffusion-weighted imaging (DWI) signals in the bilateral auricles. The cerebrospinal fluid (CSF) analysis showed 2 leukocytes/μL, 736.5 mg/L of protein, and no evidence of infectious disease or autoimmune encephalitis. Meningoencephalitis secondary to RP was considered. The patient's condition improved significantly and quickly with the administration of dexamethasone (10 mg per day). Oral methylprednisolone was continued, and the patient remained well without relapse during the 9-month follow-up period.
CONCLUSION
RP-associated meningoencephalitis is rare but fatal. Although symptoms vary, red or deformed ears remain the most common and suggestive features. Non-specific parenchymal changes and/or meningeal enhancement can be observed on brain MRI scans. CSF lymphocytic pleocytosis with mild protein elevation was observed in most patients.
PubMed: 38033767
DOI: 10.3389/fneur.2023.1265345 -
Seminars in Ophthalmology Jan 2024Optic neuritis may occur in a variety of conditions, including as a manifestation of multiple sclerosis. Despite significant research into the efficacy of... (Review)
Review
INTRODUCTION
Optic neuritis may occur in a variety of conditions, including as a manifestation of multiple sclerosis. Despite significant research into the efficacy of corticosteroids as a first-line treatment, the optimal route of administration has not been well defined. This review aims to explore the efficacy, adverse effects and economic implications of using oral versus intravenous methylprednisolone to treat acute optic neuritis.
METHODS
A systematic search of the databases PubMed/MEDLINE, Embase and CENTRAL was performed to July 2022, prior to data collection and risk of bias analysis in accordance with the PRISMA guidelines.
RESULTS
Six articles fulfilled the inclusion criteria. The results showed that in the treatment of acute optic neuritis, oral methylprednisolone has a non-inferior efficacy and adverse effect profile in comparison to intravenous methylprednisolone. In a cost analysis, oral methylprednisolone to be more cost-effective than intravenous methylprednisolone.
CONCLUSIONS
Oral methylprednisolone has comparable efficacy and adverse effect profiles to intravenous methylprednisolone for the treatment of optic neuritis. The analysis suggests oral administration is more cost-effective than intravenous administration; however, further analyses of the formal cost-benefit ratio are required.
Topics: Humans; Methylprednisolone; Prednisone; Glucocorticoids; Administration, Intravenous; Optic Neuritis; Administration, Oral
PubMed: 38013424
DOI: 10.1080/08820538.2023.2287100 -
European Archives of... Mar 2024Bell's palsy is a condition affecting cranial nerve VII that results in acute peripheral unilateral facial weakness or paralysis of unclear etiology. Corticosteroids are... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Bell's palsy is a condition affecting cranial nerve VII that results in acute peripheral unilateral facial weakness or paralysis of unclear etiology. Corticosteroids are the primary therapy choice, because they improve outcomes. According to a recent study, prednisolone effectively treats Bell's palsy in the short and long term. This study aimed to assess the effectiveness and safety of Single-Dose Intravenous Methylprednisolone to Oral Prednisolone in treating Bell's palsy patients.
METHODS
PRISMA statement guidelines were used to design and conduct this systemic review. MEDLINE, Cochrane Library, and EMBASE databases were used in our search. We conducted the database search in November 2022.
RESULTS
Thirty-three publications were reviewed as a result of the literature review. Three studies were included in the meta-analysis after applying our criteria. 317 Bell's palsy patients were included in our study. Regarding complete recovery to grade 1 in 1 month, IV methylprednisolone was higher than oral prednisolone; (log OR = 0.52, 95% CI [0.08, 0.97], P = 0.022). However, at 3 months, the two groups had no significant difference. Patients with grade 4 Bell's palsy were more likely to fully recover to grade 1 in 1 month with IV methylprednisolone than with oral prednisolone (log OR = 0.73, 95% CI [0.19, 1.26], P = 0.008), but not for patients with grade 3 or grade 2 Bell's palsy.
CONCLUSION
This study shows evidence that patients with Bell's palsy can fully recover to grade 1 in 1 month when IV methylprednisolone is used instead of oral prednisolone. At 3 months, however, there was no discernible difference between the two treatments. Within 3 days of the onset of symptoms, IV methylprednisolone treatment can be started, which may help patients recover fully to grade 1 in 1 month. However, administering IV methylprednisolone may not always have long-term advantages compared to oral prednisolone.
Topics: Humans; Bell Palsy; Randomized Controlled Trials as Topic; Glucocorticoids; Methylprednisolone; Facial Paralysis
PubMed: 37940744
DOI: 10.1007/s00405-023-08288-z -
Journal of Lower Genital Tract Disease Jan 2024Lichen sclerosus (LS) is a chronic, inflammatory process affecting predominantly anogenital skin, with extragenital involvement in up to 20% of cases. The mainstay of...
BACKGROUND
Lichen sclerosus (LS) is a chronic, inflammatory process affecting predominantly anogenital skin, with extragenital involvement in up to 20% of cases. The mainstay of therapy for anogenital LS is topical immunosuppression. However, in treatment-refractory cases, severe, or hypertrophic disease, systemic modalities may be used. Currently, there are no guidelines for systemic therapy in LS.
OBJECTIVE
This study aimed to provide a review of the current literature on use of systemic therapies for LS, including demographic and clinical features of LS, as well as reported outcomes.
METHODS
A primary literature search was conducted using the following databases: PubMed, Ovid, Scopus, and Web of Science, from the year the journal was published until June 2022.
RESULTS
Ultimately, 71 studies consisting of 392 patients were included. Of these, 65% (n = 254) had anogenital disease, 9% (n = 36) had extragenital disease, and 19% (n = 73) had both anogenital and extragenital disease, and in 7% (n = 29) of cases, location was not specified. The most frequent therapies, stratified by total cases, included oral retinoids (n = 227), methotrexate (n = 59), hydroxychloroquine (n = 36), and systemic steroids (prednisone, methylprednisolone, prednisolone, oral triamcinolone, and other systemic steroids) (n = 60). Overall, 76% (n = 194) of anogenital, 94% (n = 34) of extragenital, and 81% (n = 59) of patients with both anogenital and extragenital involvement were reported to have clinical or symptomatic improvement.
CONCLUSION
Overall, we found many therapies that have been used with reported success for extragenital and genital LS. However, future studies are needed to better define treatment outcomes and directly compare efficacy of different therapies for LS.
Topics: Humans; Lichen Sclerosus et Atrophicus; Methotrexate; Treatment Outcome; Skin; Steroids
PubMed: 37924260
DOI: 10.1097/LGT.0000000000000775 -
Frontiers in Public Health 2023New reports suggest that anti-inflammatory drugs are widely used to treat respiratory tract infections caused by SARS-CoV-2. Anti-inflammatory drugs were the most... (Meta-Analysis)
Meta-Analysis
BACKGROUND
New reports suggest that anti-inflammatory drugs are widely used to treat respiratory tract infections caused by SARS-CoV-2. Anti-inflammatory drugs were the most frequently used treatment for the COVID-19-related cytokine storm in China. However, the efficacy of anti-inflammatory drugs has yet to be systematically analyzed, and clinicians are often uncertain which class of anti-inflammatory drug is the most effective in treating patients with respiratory tract infections caused by SARS-CoV-2, especially those with severe disease.
METHODS
From 1 October 2022, relevant studies were searched in the PubMed, Embase, Medline, Cochrane Library, and Web of Science databases. A total of 16,268 publications were retrieved and collated according to inclusion and exclusion criteria, and sensitivity analyses were performed using STATA 14 software. Publication bias was assessed using funnel plots and Egger's test. Study quality was assessed using the PEDro scale, and the combined advantage ratio was expressed as a 95% confidence interval (CI). In total, 19 randomized controlled trials were included in the study. STATA 14 software was used for all random effects model analyses, and the results are expressed as relative risk ratios (RR) with 95% CI.
RESULTS
Quantitative analyses were performed on 14,514 patients from 19 relevant randomized controlled clinical trials. Pooled estimates (RR = 0.59, 95% CI 0.44-0.80) revealed that the use of anti-inflammatory drugs resulted in a significant reduction in mortality in patients with respiratory tract infection caused by SARS-CoV-2 compared with controls, and methylprednisolone (RR = 0.14, 95% CI 0.03-0.56) was more effective than other anti-inflammatory drugs. Anti-inflammatory drugs were effective in reducing mortality in critically ill patients (RR = 0.67, 95% CI 0.45-0.98) compared with non-critically ill patients (RR = 0.50, 95% CI 0.34-0.76); however, more clinical evidence is needed to confirm these findings.
CONCLUSION
The use of anti-inflammatory drugs in patients with respiratory infections caused by SARS-CoV-2 reduces patient mortality, especially in severe cases. In individual studies, methylprednisolone was more effective than other drugs.
Topics: Humans; SARS-CoV-2; COVID-19; Anti-Inflammatory Agents; Methylprednisolone; Respiratory Tract Infections
PubMed: 37920591
DOI: 10.3389/fpubh.2023.1198987 -
Revista Espanola de Cirugia Ortopedica... Oct 2023Systematic review and meta-analysis. (Review)
Review
STUDY DESIGN
Systematic review and meta-analysis.
OBJECTIVE
To compare early (<24h) versus late (>24h) spinal cord decompression on neurological recovery in patients with acute spinal cord injury.
METHODS
A systematic review was performed according to the PRISMA protocol to identify studies published up to December 2022. Prospective cohort studies and controlled trials comparing early versus delayed decompression on neurological recovery were included. Variables included number of patients, level of injury, treatment time, ASIA grade, neurological recovery, use of corticosteroids, and complications. For the meta-analysis, the «forest plot» graph was developed. The risk of bias of the included studies was assessed using the ROBINS-I22 and Rob223 tools.
RESULTS
Six of the seven studies selected for our review were included in the meta-analysis, with a total of 1188 patients (592 patients in the early decompression group and 596 in the delayed decompression group), the mean follow-up was 8 months, in 5 studies used methylprednisolone, the most reported complications were thromboembolic cardiopulmonary events. Five studies showed significant differences in favor of early decompression (risk difference 0.10, 95% confidence interval 0.07-0.14, heterogeneity 46%). The benefit was greatest in cervical and incomplete injuries.
CONCLUSION
There is scientific evidence to recommend early decompression in the first 24h after traumatic spinal cord injury, as it improves final neurological recovery, and it should be recommended whenever the patient and hospital conditions allow it to be safely done.
PubMed: 37805026
DOI: 10.1016/j.recot.2023.09.009