scholarly journals Comparison of Clinical, Immunological And Radiological Characteristics In Autoimmune GFAP Astrocytopathy, MOGAD And AQP4-Igg +NMOSD Mimicking Infectious Meningitis As The Initial Manifestation

Author(s):  
Jun Xiao ◽  
Shuo-Qi Zhang ◽  
Xin Chen ◽  
Yue Tang ◽  
Man Chen ◽  
...  

Abstract Objective: Several autoimmune CNS inflammatory diseases, including autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and aquaporin-4-immunoglobulin-G-positive neuromyelitis optica spectrum disorders (AQP4-IgG+ NMOSD) often presented initially with similar infectious meningitis-like symptoms. However, it was not easy to differentiate them at disease onset without antibody detection. The present study aimed to compare the clinical, immunological and radiological features among the three diseases. Methods: In our single-center cohorts, 9 A-GFAP-A, 17 MOGAD and 11 AQP4-IgG+ NMOSD patients mimicking infectious meningitis as initial symptoms were retrospectively included. The autoantibodies were detected with cell-based assays. The clinical, immunological and radiological characteristics of the three groups were summarized. Results: AQP4-IgG+ NMOSD patients were statistically more often in men (10, 90.9%, P=0.003). Tremor was predominated in A-GFAP-A (4, 44.4%) over MOGAD (1, 5.9%, P= 0.034) and never found in AQP4-IgG+NMOSD (0, P=0.026). The Modified Rankin Score (mRS) at the clinical nadir of diseases was lower in AQP4-IgG+NMOSD (2.2 [IQR, 1-3]) compared to A-GFAP-A (3.7 [IQR, 3-5], P=0.04). On CSF examination, white blood cell count (WBC) was higher in A-GFAP-A (median, 272×106/L [range, 0-1600]) compared to AQP4-IgG+NMOSD (median, 12×106/L [range, 0-48], P=0.049). Significant increase in CSF protein (1490.7±871.2 mg/L), lactic acid (3.43±0.81 mmol/L), IgG (130.9±60.4 mg/L), IgM (8.6±6.1mg/L) and IgA (23.0±11.4mg/L) levels in A-GFAP-A was found compared to MOGAD (CSF protein: 606.7±379.4 mg/L, P<0.001; lactic acid: 2.15 ± 0.62mmol/L, P<0.001; IgG: 77.9±71.3 mg/L, P=0.043; IgM, 2.7±2.9mg/L, P=0.002; IgA, 11.3±12.1mg/L, P=0.012) and AQP4-IgG+NMOSD (CSF protein: 441.8±178.0 mg/L, P<0.001; lactic acid: 2.40 ± 0.66 mmol/L, P=0.003; IgG, 53.2±30.3 mg/L, P=0.01; IgM, 2.1±3.9mg/L, P=0.003; IgA, 5.2±5.0mg/L, P=0.001). Over half of the A-GFAP-A patients (5/8, 62.5%) showed small (<2 cm), symmetrical lesions in ganglia and thalamus (5/8, 62.5%), but never in MOGAD (0%, P=0.001) and AQP4-IgG+NMOSD (0%, P=0.026). Diffuse meningeal enhancement was common in A-GFAP-A (8, 88.9%) compared to MOGAD (5, 29.4%, P=0.011) and AQP4-IgG+NMOSD (1/6, 16.7%, P=0.011). Acute disseminated encephalomyelitis (ADEM) -like lesions occurred frequently in MOGAD (6/16, 37.5%) but never in A-GFAP-A and AQP4-IgG+NMOSD (P=0.02). Conclusion: Our study demonstrated that several signs including the symptom of tremor, a more severe disease course, higher CSF immunological profiles and ganglia bilateral symmetrical lesions, diffuse meningeal enhancement were distinct features in A-GFAP-A, and ADEM-like lesions occurred only in MOGAD mimicking infectious meningitis as initial symptoms, providing possible clinical implications for patient differential diagnosis.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1309.1-1310
Author(s):  
S. Remuzgo Martinez ◽  
F. Genre ◽  
V. Pulito-Cueto ◽  
D. Prieto-Peña ◽  
B. Atienza-Mateo ◽  
...  

Background:Interferon signaling pathway plays a relevant role in autoimmunity. Genetic variants in theinterferon regulatory factor (IRF) 5gene, that encodes the major regulator of the type I interferon induction [1], have been related to the development of several inflammatory diseases [2].Objectives:To determine the influence ofIRF5on Immunoglobulin-A vasculitis (IgAV), an inflammatory vascular disease.Methods:ThreeIRF5polymorphisms (rs2004640, rs2070197 and rs10954213) representative of 3 different haplotype blocks were genotyped in 372 Caucasian patients with IgAV and 876 sex and ethnically matched healthy controls.Results:No statistically significant differences between patients with IgAV and controls were observed when eachIRF5polymorphism was analyzed independently. Similarly, no statistically significant differences between patients with IgAV and controls were found whenIRF5polymorphisms were evaluated combined conforming haplotypes. Additionally, there were no statistically significant differences in genotype, allele and haplotype frequencies ofIRF5when patients with IgAV were stratified according to the age at disease onset or to the presence/absence of gastrointestinal or renal manifestations.Conclusion:Our results do not support an influence ofIRF5on the pathogenesis of IgAV.References:[1]Nat Immunol 2011; 12: 231-8;[2]Arthritis Res Ther 2014; 16: R146.Acknowledgments:This study was supported by European Union FEDER funds and “Fondo de Investigaciones Sanitarias” (grant PI18/00042) from ‘Instituto de Salud Carlos III’ (ISCIII, Health Ministry, Spain). RL-M is a recipient of a Miguel Servet type I programme fellowship from the ISCIII, co-funded by the European Social Fund (ESF, `Investing in your future´) (grant CP16/00033). SR-M is supported by funds of the RETICS Program (RD16/0012/0009) (ISCIII, co-funded by the European Regional Development Fund (ERDF)). VP-C is supported by a pre-doctoral grant from IDIVAL (PREVAL 18/01). LL-G is supported by funds of PI18/00042 (ISCIII, co-funded by ERDF).Disclosure of Interests:Sara Remuzgo Martinez: None declared, Fernanda Genre: None declared, Verónica Pulito-Cueto: None declared, D. Prieto-Peña: None declared, Belén Atienza-Mateo: None declared, Belén Sevilla: None declared, Javier Llorca: None declared, Norberto Ortego: None declared, Leticia Lera-Gómez: None declared, Maite Leonardo: None declared, Ana Peñalba: None declared, María Jesús Cabero: None declared, Luis Martín-Penagos: None declared, Jose Alberto Miranda-Filloy: None declared, Antonio Navas Parejo: None declared, Javier Sanchez Perez: None declared, Maximiliano Aragües: None declared, Esteban Rubio: None declared, MANUEL LEON LUQUE: None declared, Juan María Blanco-Madrigal: None declared, E. Galindez: None declared, Javier Martin Ibanez: None declared, Santos Castañeda: None declared, Ricardo Blanco Grant/research support from: Abbvie, MSD and Roche, Consultant of: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen and MSD, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Lilly and MSD, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Raquel López-Mejías: None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 407.1-407
Author(s):  
M. G. Lazzaroni ◽  
S. Zingarelli ◽  
P. Airò ◽  
Y. Allanore ◽  
O. Distler

Background:Anti-PM/Scl antibodies positivity has been associated with frequent skeletal muscle involvement in patients with Systemic Sclerosis (SSc) in different studies, including the EUSTAR cohort (1). Moreover, although myositis has been previously associated with heart involvement in SSc patients (2), this issue has never been explored among anti-PM/Scl+ patients.Objectives:To evaluate the cardiac involvement in anti-PM/Scl patients with SSc in the large multicentre EUSTAR database, with focus on the subgroup of patients with muscle involvement.Methods:Patients from the EUSTAR database were included when the item anti-PM/Scl was fulfilled in at least one visit.Results:Anti-PM/Scl status was available in 7,353 SSc patients from EUSTAR database: 295 were anti-PM/Scl+. After exclusion of 151 patients with multiple autoantibody positivity, 144 anti-PM/Scl + patients were compared with 7,058 anti-PM/Scl- patients. Among them, 3,120 (44.2%) were positive for ACA, 2,361 (33.5%) for anti-Topo I and 274 (3.88%) for anti-RNAP3.Regarding the specific cardiac outcomes, in the anti-PM/Scl+ as compared to the anti-PM/Scl- group, a decreased rate of elevated sPAP at ECHO was recorded (12.8% vs 25.0%, p:0.001), while no differences were observed in the frequency of conduction blocks (26.2% vs 23.7%, p:0.526), abnormal diastolic function (33.9% vs 36.4%, p:0.582), pericardial effusion (10.2% vs 10.9%, p:1.000) and LVEF ≤50% (4.76% vs 6.11%, p:0.818). In multivariate analysis, adjusted for age at disease onset, sex, and disease duration, the negative association of anti-PM/Scl with elevated sPAP was not confirmed (p:0.061).When comparing anti-PM/Scl+ patients with (n=47) and without (n=87) CK elevation, the former group had a higher frequency of conduction blocks (43.2% vs 17.5%, p:0.005; OR 95% CI 3.47, 1.51-7.97) and left ventricular dysfunction, both diastolic (45.6% vs 27.2%, p:0.050; OR 95% CI 2.25, 1.05-4.81) and systolic (LVEF ≤50% 13.3% vs 0%, p:0.018; OR 95% CI 16.8, 0.87-324). Moreover, anti-PM/Scl+ patients with CK elevation had significantly increased rate of lung fibrosis on HRCT (p:0.045), intestinal symptoms (p:0.017), joint contractures (p:0.045) and tendon friction rubs (p:0.034).Conclusion:In the largest series of anti-PM/Scl positive SSc patients so far reported, muscle involvement in anti-PM/Scl+ patients (defined as increased serum CK) seems to represent a marker of a more severe disease phenotype, including a higher frequency of cardio-pulmonary involvement.References:[1]Lazzaroni MG, et al. Ann Rheum Dis 2018. 77 (2), 421-2.[2]Follansbee WP, et al. Am Heart J 1993. 125: 194-203.Acknowledgments:Authors would like to thank the patients’ association GILS (Gruppo Italiano Lotta Sclerodermia) for the grant that supported the project.Disclosure of Interests:Maria Grazia Lazzaroni: None declared, Stefania Zingarelli: None declared, Paolo Airò: None declared, Yannick Allanore Grant/research support from: BMS, Inventiva, Roche, Sanofi, Consultant of: Actelion, Bayer AG, BMS, BI, Oliver Distler Grant/research support from: Grants/Research support from Actelion, Bayer, Boehringer Ingelheim, Competitive Drug Development International Ltd. and Mitsubishi Tanabe; he also holds the issued Patent on mir-29 for the treatment of systemic sclerosis (US8247389, EP2331143)., Consultant of: Consultancy fees from Actelion, Acceleron Pharma, AnaMar, Bayer, Baecon Discovery, Blade Therapeutics, Boehringer, CSL Behring, Catenion, ChemomAb, Curzion Pharmaceuticals, Ergonex, Galapagos NV, GSK, Glenmark Pharmaceuticals, Inventiva, Italfarmaco, iQvia, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Roche, Sanofi and UCB, Speakers bureau: Speaker fees from Actelion, Bayer, Boehringer Ingelheim, Medscape, Pfizer and Roche


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Diana Prieto-Peña ◽  
Sara Remuzgo-Martínez ◽  
Fernanda Genre ◽  
Verónica Pulito-Cueto ◽  
Belén Atienza-Mateo ◽  
...  

AbstractCytokines signalling pathway genes are crucial factors of the genetic network underlying the pathogenesis of Immunoglobulin-A vasculitis (IgAV), an inflammatory vascular condition. An influence of the interleukin (IL)33- IL1 receptor like (IL1RL)1 signalling pathway on the increased risk of several immune-mediated diseases has been described. Accordingly, we assessed whether the IL33-IL1RL1 pathway represents a novel genetic risk factor for IgAV. Three tag polymorphisms within IL33 (rs3939286, rs7025417 and rs7044343) and three within IL1RL1 (rs2310173, rs13015714 and rs2058660), that also were previously associated with several inflammatory diseases, were genotyped in 380 Caucasian IgAV patients and 845 matched healthy controls. No genotypes or alleles differences were observed between IgAV patients and controls when IL33 and IL1RL1 variants were analysed independently. Likewise, no statistically significant differences were found in IL33 or IL1RL1 genotype and allele frequencies when IgAV patients were stratified according to the age at disease onset or to the presence/absence of gastrointestinal (GI) or renal manifestations. Similar results were disclosed when IL33 and IL1RL1 haplotypes were compared between IgAV patients and controls and between IgAV patients stratified according to the clinical characteristics mentioned above. Our results suggest that the IL33-IL1RL1 signalling pathway does not contribute to the genetic network underlying IgAV.


Foods ◽  
2018 ◽  
Vol 7 (12) ◽  
pp. 195 ◽  
Author(s):  
Victoria Bell ◽  
Jorge Ferrão ◽  
Lígia Pimentel ◽  
Manuela Pintado ◽  
Tito Fernandes

Changes in present-day society such as diets with more sugar, salt, and saturated fat, bad habits and unhealthy lifestyles contribute to the likelihood of the involvement of the microbiota in inflammatory diseases, which contribute to global epidemics of obesity, depression, and mental health concerns. The microbiota is presently one of the hottest areas of scientific and medical research, and exerts a marked influence on the host during homeostasis and disease. Fermented foods and beverages are generally defined as products made by microbial organisms and enzymatic conversions of major and minor food components. Further to the commonly-recognized effects of nutrition on the digestive health (e.g., dysbiosis) and well-being, there is now strong evidence for the impact of fermented foods and beverages (e.g., yoghurt, pickles, bread, kefir, beers, wines, mead), produced or preserved by the action of microorganisms, on general health, namely their significance on the gut microbiota balance and brain functionality. Fermented products require microorganisms, i.e., Saccharomyces yeasts and lactic acid bacteria, yielding alcohol and lactic acid. Ingestion of vibrant probiotics, especially those contained in fermented foods, is found to cause significant positive improvements in balancing intestinal permeability and barrier function. Our guts control and deal with every aspect of our health. How we digest our food and even the food sensitivities we have is linked with our mood, behavior, energy, weight, food cravings, hormone balance, immunity, and overall wellness. We highlight some impacts in this domain and debate calls for the convergence of interdisciplinary research fields from the United Nations’ initiative. Worldwide human and animal medicine are practiced separately; veterinary science and animal health are generally neither considered nor inserted within national or international Health discussions. The absence of a clear definition and subsequent vision for the future of One Health may act as a barrier to transdisciplinary collaboration. The point of this mini review is to highlight the role of fermented foods and beverages on gut microbiota and debate if the need for confluence of transdisciplinary fields of One Health is feasible and achievable, since they are managed by separate sectors with limited communication.


Plant Disease ◽  
2005 ◽  
Vol 89 (11) ◽  
pp. 1241-1241 ◽  
Author(s):  
A. Garibaldi ◽  
A. Minuto ◽  
M. L. Gullino

Several species of Diplotaxis (D. tenuifolia, D. erucoides, and D. muralis), known as wild or sand rocket, are widely cultivated in Italy. Rocket is used in Mediterranean cuisine as salad, a component of packaged salad products, and as a garnish for food. In winter 2003, a severe disease was observed on D. tenuifolia grown in unheated glasshouses on commercial farms near Albenga in northern Italy. Initial symptoms included stem necrosis at the soil level and darkening of leaves. As stem necrosis progressed, infected plants wilted and died. Wilt, characterized by the presence of soft and watery tissues, occurred within a few days on young plants. The disease was extremely severe in the presence of high relative humidity and mild temperature (15°C). Necrotic tissues became covered with white mycelium that produced dark sclerotia. Diseased stem tissue was disinfested for 1 min in 1% NaOCl and plated on potato dextrose agar (PDA) amended with 100 ppm streptomycin sulfate. Sclerotinia sclerotiorum (1) was consistently recovered from infected stem pieces. Sclerotia observed on infected plants measured 1.23 to 3.00 × 1.40 to 5.38 mm (average 2.10 × 2.85 mm). Sclerotia produced on PDA measured 1.00 to 4.28 × 1.00 to 6.01 mm (average 2.38 × 3.23 mm). Pathogenicity of three isolates obtained from infected plants was confirmed by inoculating 30-day-old plants of D. tenuifolia grown in 18-cm-diameter pots in a glasshouse. Inoculum, 2 g per pot of wheat kernels infested with mycelium and sclerotia of each isolate, was placed on the soil surface around the base of each plant. Three replicates of five pots each were used per isolate. Noninoculated plants served as controls. The inoculation trial was repeated once. All plants were kept at temperatures ranging between 10 and 26°C (average 15°C) with an average relative humidity of 80% and were watered as needed. Inoculated plants developed symptoms of leaf yellowing within 12 days, soon followed by the appearance of white mycelium and sclerotia, and eventually wilted. Control plants remained symptomless. S. sclerotiorum was reisolated from inoculated plants. To our knowledge, this is the first report of infection of D. tenuifolia by S. sclerotiorum in Italy as well as worldwide. The disease currently has been observed in the Liguria Region but not yet in other areas where sand rocket is cultivated. The economic importance of this disease for the crop can be considered medium at the moment, but is expected to increase in the future. Reference: (1) N. F. Buchwald. Den. Kgl. Veterin.er-og Landbohojskoles Aarsskrift, 75, 1949.


Author(s):  
Geetharani Gopalan ◽  
Sudha R. Gopinath ◽  
Kothandaramasamy R. ◽  
Sathesh Pandian

<p class="abstract"><strong>Background:</strong> Discoid lupus erythematosus (DLE) is the commonest form of cutaneous lupus erythematosus.The objective of our study is to analyze the clinical and epidemiological aspects of DLE.</p><p class="abstract"><strong>Methods:</strong> All clinically diagnosed cases of DLE attending the dermatology OPD from October 2010 to September 2012were included in the study. A detailed history, complete physical examination, biopsy for confirmation and other relevant investigations were done in all cases.<strong></strong></p><p class="abstract"><strong>Results:</strong> The incidence was 4.79 per 10000 cases (51 of 106368 dermatology patients) showing female to male ratio of 4.1:1. Localized type was more common than the disseminated type. Few lesions (less than five) in a localized area without head and neck involvement were also classified as localized type in this study. Mucosal, verrucous, tumid and lupus panniculitis were the variants of DLE encountered. The sites involved were face, scalp, trunk, upper and lower limb in descending order of frequency. Antinuclear antibody (ANA) was positive in 22 of 30 cases done (73%). The systemic involvement was seen in 15 patients all of whom were diagnosed as systemic lupus erythematosus (SLE). Squamous cell carcinoma was seen in 2 cases of disseminated DLE.</p><p class="abstract"><strong>Conclusions:</strong> Majority of patients had disease onset at 3<sup>rd</sup> to 5<sup>th</sup> decade showing female predominance. When compared to localized type, disseminated type was found more frequently in males. Early onset and severe disease was noted among offspring born to a patient suffering from disseminated DLE. Serious morbidity like lupus nephritis was observed only in 1 case. The occurrence of DLE over the herpes zoster scar was an interesting observation.</p>


2021 ◽  
pp. 16-19
Author(s):  
Shelesh Kumar Swami ◽  
Nitesh Kumar Chauhan ◽  
Shuchi Goyal ◽  
A.K. Verma ◽  
Shweta Biyani

Background & objectives: The 2019 novel coronavirus disease (COVID-19) pandemic is a big challenge for scientic and medical eld. Progression of severe disease is a difcult problem in treatment. Therefore, there is essential need to recognize severe forms of COVID-19 early in the disease course. Identication of effective biomarkers are able to classify patients based on severity. In this pilot study, we aimed to validate the association between immunologic biomarkers Interleukin-6 and ferritin with the severity of the COVID-19. Methods: A total of 1443 patients diagnosed with COVID-19 were enrolled including a severe group and a nonsevere group. Baseline clinical characteristics were collected. Serum interleukin-6 and ferritin were measured and the resultant data was statistically analyzed. Results: The most common initial symptoms were fever (68.81%) and cough (47.82%), followed by loss of appetite (9.28%), fatigue (17.46%) and breathlessness (15.52%). Level of IL-6 and Ferritin were signicantly higher in the severe patients (p<.05 for both) compared with nonsevere patients. Interpretation & conclusions: This pilot study conrmed that IL-6 and ferritin biomarkers are closely associated with the severity of COVID-19. Assessment of these biomarkers could be helpful to medical practitioners in starting treatment and close monitoring against COVID-19 infection which could improve prognosis and lower mortality


2020 ◽  
Vol 104 (8) ◽  
pp. 3331-3337 ◽  
Author(s):  
Jean Guy LeBlanc ◽  
Romina Levit ◽  
Graciela Savoy de Giori ◽  
Alejandra de Moreno de LeBlanc

Cephalalgia ◽  
1998 ◽  
Vol 18 (8) ◽  
pp. 583-584 ◽  
Author(s):  
C Stöllberger ◽  
J Finsterer ◽  
C Fousek ◽  
FR Waldenberger ◽  
H Haumer ◽  
...  

The most common initial symptom of aortic dissection is chest pain. Other initial symptoms include pain in the neck, throat, abdomen and lower back, syncope, paresis, and dyspnoea. Headache as the initial symptom of aortic dissection has not been described previously. A 61-year-old woman with a history of migraine and arterial hypertension developed continuous bifrontal headache. Two hours later, right-sided thoracic pain and a diastolic murmur were suggestive of aortic dissection that was confirmed by echocardiography and subsequent surgery. The dissection commenced in the ascending aorta and involved all cervical arteries until the base of the skull. Headache as the initial manifestation of aortic dissection was assumed due to either vessel distension or pericarotid plexus ischemia. Aortic dissection has to be considered as a rare differential diagnosis of frontal headache, especially in patients who develop aortic regurgitation or chest pain for the first time.


Medicina ◽  
2019 ◽  
Vol 55 (10) ◽  
pp. 647 ◽  
Author(s):  
Lee ◽  
Fu

Pulmonary embolism is a life-threatening disease. Its development is generally thought to be due to causes collectively known as the Virchow's triad. Chronic inflammations are associated with the activation of coagulation and increased risks of venous thromboembolic events. Asthma is one of the chronic inflammatory diseases associated with procoagulants and antifibrinolytic activities in the airways. Coagulation is activated in patients with asthma with the following steps of pathophysiology: Increased tissue factor expression in various cell types, decreased activity of the anticoagulant protein C system and inhibition of fibrinolysis through over-production of plasminogen activator inhibitor type 1 (PAI-1). Asthma is therefore likely a risk factor for pulmonary embolism, especially in those patients with severe disease conditions together with frequent exacerbation. Here we present a case of severe asthma associated with coagulopathy and complicated by massive pulmonary embolism, presented with typical S1Q3T3 on electrocardiography (ECG) and massive thrombosis on computed tomography angiography, successfully treated with directed catheter thrombolytic therapy.


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