Nefropatía por enfermedad relacionada a IGG4 / Nephropathy due to IGG4-related disease

IgG4-related disease (ER-IgG4) is a group of systemic fibro-inflammatory diseases, whose renal involvement is rare and difficult to diagnose. Diagnosis is usually made by serological and histological studies. Treatment is based on systemic corticosteroids. The renal prognosis is determined by the patient's comorbidities and the degree of fibrosis in the renal biopsy. We present the case of an elderly patient with exacerbated chronic kidney disease, whose study showed nephropathy associated with ER-IgG4.

La enfermedad relacionada a IgG4 (ER-IgG4) es un grupo de enfermedades fibro-inflamatorias sistémicas, cuya afectación renal es poco frecuente y de difícil diagnóstico. Habitualmente el diagnóstico se realiza mediante estudios serológicos e histológicos. El tratamiento se basa en corticoides sistémicos. El pronóstico renal está determinado por las comorbilidades del paciente y el grado de fibrosis en la biopsia renal. Se presenta el caso de un paciente adulto mayor con enfermedad renal crónica reagudizada, cuyo estudio demostró nefropatía asociada a ER-IgG4.

Expression of Gn protein of severe fever with thrombocytopenia syndrome virus and its immunogenicity / 中国生物制品学杂志

@#Objective To express the Gn protein of severe fever with thrombocytopenia syndrome virus(SFTSV) through adeno-associated virus 9(AAV9) expression system and evaluate its immunogenicity.Methods SFTSV Gn gene was inserted into viral vector pAAV-CMV-FH and the recombinant plasmid was transfected into HEK293T cells to obtain recombinant virus AAV9-Gn.The expression of Gn protein was determined by immunofluorescence and Western blot.Eighteen fernale BALB/c mice were randomly divided into three groups:Mock group(serum-free DMEM),AAV9-GFP group(1 × 10~(11) vg) and AAV9-Gn group(1 × 10~(11) vg),all of which were injected intramuscularly into the right hind limb at a dose of 100 μL per mouse.The body mass,diet,behavior and mental state of mice in each group were monitored continuously for 21 d,and the change rate of body mass was calculated;At 2,4,8 and 16 weeks after immunization,the levels of SFTSV neutralizing antibody in serum of mice in each group were detected by fluorescent reduction neutralization test(FRNT),and the levels of specific IgGl and IgG2a in serum of mice in AAV9-Gn group were detected by ELISA.Results After incubation with specific antibody,Vero cells transfected with AAV9-Gn showed specific green fluorescence under fluorescence microscope,and had specific binding to mouse anti-SFTSV Gn monoclonal antibody,and the specific binding bands were found at a relative molecular mass of about 61 000.The body mass of the three groups showed an increasing trend,there was no significant difference between the three groups(F=0.158—2.621,P> 0.05),and the diet,behavior and mental state were normal.At 2,4,8 and 16 weeks after immunization,the titer of SFTSV neutralizing antibody in serum of mice in AAV9-Gn group was significantly higher than that of Mock group and AAV9-GFP group(H=13.332—14.538,each P <0.001),and the titer peak appeared at 8 weeks;The level of specific IgG1 in serum of mice was significantly higher than that of IgG2a(F=4.373—12.975,each P <0.05) at different time points.Conclusion SFTSV Gn protein can be expressed correctly through AAV9 expression system,and has low toxicity to mice with good immunogenicity,which is expected to be a candidate component of SFTSV vaccine.

Immunogenicity evaluation of prototype and variant inactivated SARS-CoV-2 vaccines in rats / 中国生物制品学杂志

@#Objective To evaluate the immunogenicity of prototype strain,Beta strain,Gamma strain and Delta strain of SARS-CoV-2 inactivated vaccines in rats.Methods Five female Wistar rats were immunized with SARS-CoV-2 inactivated vaccines of prototype,Beta,Gamma and Delta strains through thigh muscle twice at an interval of 14 d,with an immunization dose of 3 μg virus protein/(0.5 mL per rat).Serum samples were collected and isolated by vein 14,28 and 42 d after the first immunization.The serum IgG antibody levels were detected by indirect ELISA,the titers of serum neutralizing antibody were measured by microneutralization,and the antigenic ratios of the serum neutralizing antibody titers were calculated to evaluate the antigenicity difference between different strains.Results at 14 d after the first immunization,IgG antibodies against four strains of virus were detected in all immunized serum samples.The levels of IgG antibodies increased by more than 10 times at 28 d compared with those at 14 d,and decreased slightly at 42 d.At 14 d after the first immunization,all the neutralizing antibodies against the four strains were positive in the serum of rats immunized with prototype strain or Delta strain vaccine;In the serum samples of rats immunized with Beta and Gamma strains,all the neutralizing antibodies against Beta and Gamma strains were positive,while some neutralizing antibodies against prototype or Delta strains were positive.At 28 d after the first immunization,the neutralizing antibodies in the immune serum of the four strains were positive,and the titers of neutralizing antibodies were significantly higher than those at 14 d;The neutralizing antibody titers were slightly lower at 42 d after the first immunization than 28 d.There was small difference in the antigenicity between Beta and Gamma,prototype and Gamma,but significant difference in the antigenicity between prototype and Beta strains.Conclusion The prototype strain,Beta strain,Gamma strain and Delta strain of SARS-CoV-2 inactivated vaccines can stimulate rats to produce neutralizing antibodies with high titer,while the immunogenicity has difference.

Allergenic response induced by Pterobothrium crassicolle (Cestoda: Trypanorhyncha) extracts in murine model / Resposta alergênica induzida por extratos de Pterobothrium crassicolle (Cestoda: Trypanorhyncha) em modelo murino

Abstract The aim of this study was to determine the allergenic activity of components present in crude extracts of Pterobothrium crassicolle plerocerci (CPE) and blastocysts (CBE) obtained from Micropogonias furnieri in a murine model. Two groups of seven animals each received 50 µg of CPE or CBE on days 1, 35 and 120. Serum samples were tested by ELISA and Immunoblotting. Specific IgG and IgE levels were detected by ELISA, showing specific humoral responses for the primary immunization for both immunoglobulins and continuously growing titers for IgE. Positive Passive Cutaneous Anaphylaxis tests in rats sensitized with anti-CBE sera and tested by CBE, showed biologically, the allergenic activity of the extracts. The CPE and CBE showed some different recognition regions but both experimental groups recognized all regions of the extracts when tested for cross reactions, showing that CPE and CBE could share antigenic recognition sites.

Resumo O objetivo deste estudo foi determinar a atividade alergênica de componentes presentes em extratos crus de plerocercos (CPE) e de blastocistos de Pterobothrium crassicolle (CBE), obtidos de Micropogonias furnieri, em modelo murino. Dois grupos de sete animais receberam cada um 50 µg de CPE ou CBE nos dias 1, 35 e 120. As amostras de soro foram testadas por ELISA e Imunoblot. Os níveis específicos de IgG e IgE foram detectados por ELISA, mostrando respostas humorais específicas para a imunização primária para ambas as imunoglobulinas e títulos crescentes de IgE. Testes positivos de Anafilaxia Cutânea Passiva em ratos sensibilizados com soros anti-CBE e testados por CBE, demonstraram biologicamente, a atividade alergênica dos extratos. O CPE e o CBE evidenciaram algumas regiões de reconhecimento diferentes, mas ambos os grupos experimentais reconheceram todas as regiões dos extratos, quando testados para reações cruzadas, mostrando que o CPE e o CBE poderiam compartilhar locais de reconhecimento antigênico.

Pseudotumor inflamatorioocular asociado a IgG4. Reporte de un caso / IgG4-Associated Ocular Inflammatory Pseudotumor. A Case Report

Resumen Introducción: El pseudotumor inflamatorio ocular asociado a IgG4 es un proceso inflamatorio no neoplásico y poco frecuente con una incidencia de 0.28-1.08 por cada 100,000 personas. El diagnóstico de esta patología es de exclusión debiendo cumplir criterios histopatológicos, agrandamiento de un órgano en específico y concentraciones serias de IgG4. El tratamiento tiene como objetivo evitar las complicaciones secundarias. Caso clínico: Mujer de 54 años de edad sin patologías previas referidas, acude refiriendo que 6 meses previos presenta de forma súbita y sin causa aparente prurito a nivel de globo ocular y párpado izquierdo, además de disminución de la agudeza visual y dolor retroocular, motivo por el que acude a valoración. Al examen físico presentó movimientos oculares normales, proptosis izquierda, dolor a la palpación, hiperemia subconjuntival, opacidad corneal, ausencia de reflejo rojo y más renitente a la presión el ojo izquierdo en relación con el contralateral. Tomografía cráneo-toraco-abdominal reporta a nivel ocular izquierdo imágenes sugestivas de melanoma coroideo sin hallazgos de actividad metastásica. Se realiza enucleación de ojo izquierdo. Se envía pieza a patología la cual reporta infiltrado difuso linfoplasmocítico positivas para IgG4. Conclusión: La enfermedad por IgG4 ocular es una patología muy infrecuente de clínica inespecífica y diagnóstico complejo. Sin embargo, un abordaje rápido y correcto es fundamental para evitar complicaciones.

Abstract Introduction: IgG4-associated ocular inflammatory pseudotumor is a rare, non-neoplastic inflammatory process with an incidence of 0.28-1.08 per 100,000 people. The diagnosis of this pathology is one of exclusion, having to meet histopathological criteria, enlargement of a specific organ, and serious concentrations of IgG4. Treatment aims to avoid secondary complications. Clinical case: A 54-year-old woman with no previous reported pathologies, reports that for the past 6 months she has itching at the level of the eyeball and the left eyelid, suddenly and without apparent cause, in addition to decreased visual acuity and retro-ocular pain; this is why she asked for the evaluation. The physical examination revealed normal eye movements, left proptosis, pain on palpation, subconjunctival hyperemia, corneal opacity, absence of red reflex, and the left eye being more resistant to pressure in relation to the contralateral eye. The cranio-thoraco-abdominal tomography reported images at the left ocular level that were suggestive of choroidal melanoma without findings of metastatic activity. Enucleation of the left eye was performed. The specimen was sent to pathology which reported diffuse lymphoplasmacytic infiltrate positive for IgG4. Conclusion: Ocular IgG4 disease is a very rare pathology with non-specific symptoms and complex diagnosis. However, a quick and correct approach is essential to avoid complications.

Immunoglobulin G4-Related Disease, A Diagnosis to Take into Account: About a Case

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated and multisystemic condition associated with developing fibroinflammatory lesions in any organ. The diagnosis is made based on the sum of clinical, serological, radiological, and histopathological criteria; however, this is often difficult due to its similarity to neoplasms, infections, or other immune-mediated diseases. Treatment is based on corticosteroids, in a possible combination with immunomodulators. The present case concerns a 59-year-old man with a history of jaundice syndrome and weight loss, admitted for suspected malignant neoplasia of the bile duct. Imaging revealed stricture with dilation of the intrahepatic bile ducts, prominent pancreas, pancreatic duct stricture, and nodular renal lesions. Due to the history of left submandibulectomy two years before the current disease and histology compatible with Küttner's tumor, plus the high IgG4 serum values, the diagnosis of IgG4-RD was established. He started treatment with corticosteroids and was asymptomatic during follow-up.

La enfermedad relacionada con inmunoglobulina G4 (ER-IgG4) es una condición inmunomediada y multisistémica asociada al desarrollo de lesiones fibroinflamatorias en cualquier órgano. El diagnóstico se realiza bajo la suma de criterios clínicos, serológicos, radiológicos e histopatológicos; sin embargo, este suele ser difícil debido a su similitud con neoplasias, infecciones u otras enfermedades inmunomediadas. El tratamiento se basa en corticosteroides, en una posible combinación con inmunomoduladores. El presente caso trata de un varón de 59 años con historia de síndrome ictérico y baja de peso, admitido por sospecha de neoplasia maligna de la vía biliar. Las imágenes revelaron estenosis con dilatación de las vías biliares intrahepáticas, páncreas prominente, estenosis del conducto pancreático y lesiones nodulares renales. Debido al antecedente de submandibulectomía izquierda 2 años antes de la enfermedad actual e histología compatible con tumor de Küttner, más los valores elevados de IgG4 sérico, se estableció el diagnóstico de ER-IgG4. Inició tratamiento con corticosteroides y cursó asintomático durante el seguimiento.

Compromiso oftálmico en enfermedad relacionada con IgG4 / Ophthalmic involvement in IgG4-related disease

Resumen Introducción: la enfermedad oftálmica relacionada con IgG4 (EOR-IgG4) presenta una frecuencia del 11-59%. Pocos estudios describen las disparidades con los pacientes con ER-IgG4 extraoftálmica (NO EOR-IgG4). Objetivos: describir las características clínicas, imagenológicas, anatomopatológicas, resultados de laboratorio y tratamiento de la EOR-IgG4, y compararlas con las de los pacientes NO EOR-IgG4. Materiales y métodos: se realizó un estudio descriptivo sobre una cohorte de 54 pacientes con ER-IgG4. Se reclutaron 16 pacientes con EOR-IgG4 y 38 con NO EOR-IgG4. Se compararon ambos grupos. Resultados: la EOR-IgG4 predominó en mujeres. El 75% presentó afectación oftálmica bilateral. El antecedente de asma se asoció al grupo NO EOR-IgG4 (p=0,018). Los pacientes con EOR-IgG4 presentaron niveles séricos menores de IgE e IgG total, y la glándula lagrimal fue la estructura más afectada. Predominó el infiltrado linfoplasmocitario y eosinofílico, siendo la fibrosis estoriforme más frecuente que la no estoriforme en el grupo EOR-IgG4. Conclusiones: si bien los resultados fueron similares a lo reportado previamente, en discordancia con otras series, encontramos asociación negativa entre el asma y los niveles de IgG total sérica en los pacientes EOR-IgG4.

Abstract Introduction: IgG4-related ophthalmic disease (IgG4-ROD) presents a frequency of 11-58.8%. Few studies describe the disparities with patients with extraophthalmic IgG4-related disease (NOT IgG4-ROD). Objectives: describe the clinical, imaging, pathological characteristics, laboratory results, and treatment of IgG4-ROD characteristics; and compare them with those of the NOT IgG4-ROD patients. Materials and methods: a descriptive study was carried out on a cohort of 54 patients with ER-IgG4. 16 patients with IgG4-ROD and 38 with NOT IgG4-ROD were recruited. The data was analyzed with the SPSS Statistics 19 software. Results: IgG4-ROD predominated in women. 75% presented bilateral ophthalmic involvement. A history of asthma was associated with the NOT IgG4-ROD group (p=0,018). Patients with IgG4-ROD presented lower serum levels of IgE and total IgG, and the lacrimal gland was the most affected structure. Lymphoplasmacytic and eosinophilic infiltrates predominated, with storiform fibrosis being more frequent than non-storiform in the IgG4-ROD group. Conclusions: although the results were similar to those previously reported, in disagreement with other series, we found a negative association between asthma and serum total IgG levels with EOR-IgG4 patients.

Changes in the levels of immunological markers after treatment in patients with allergic rhinitis

Introduction: Monitoring changes in the levels of immune markers is of great significance in evaluating the effectiveness of treatment in patients with allergic rhinitis. Objectives: Determine the change in the concentration of immune markers after treatment in patients with allergic rhinitis caused by cotton dust. Methods: A descriptive, single-group, comparative before and after intervention study on 52 patients with allergic rhinitis caused by cotton dust. Comparison of immunological markers results before and after 36 months of treatment. Results: Total IgE concentration after treatment decreased, the median decreased from 1227.756 U/mL to 676.805 UI/mL. Serum levels of IgG, IgG4, and IgG1 in patients after treatment increased compared to before (p< 0.001). The cytokines also changed in the direction of no longer responding toward allergy. Median IL-17 decreased from 1.752 mg/dL to 0.417 mg/dL. Conclusion: In patients with allergic rhinitis after specific sublingual desensitization treatment, IgE levels and cytokines such as IL-6 and IL-17 are significantly reduced and IgG, IgG4 and IgG1 levels are increased after treatment.

Introducción: El monitoreo de los cambios en los niveles de marcadores inmunes es de gran importancia para evaluar la efectividad del tratamiento en pacientes con rinitis alérgica. Objetivos: Determinar el cambio en la concentración de marcadores inmunes después del tratamiento, en pacientes con rinitis alérgica causada por polvo de algodón. Métodos: Estudio descriptivo, monogrupo, comparativo antes y después de la intervención, en 52 pacientes con rinitis alérgica por polvo de algodón. Se compararon resultados de marcadores inmunológicos antes y después de 36 meses de tratamiento. Resultados: La concentración de IgE total después del tratamiento disminuyó, la mediana disminuyó de 1227,756 U/mL a 676,805 UI/mL. Los niveles séricos de IgG, IgG4 e IgG1 en pacientes, después del tratamiento, aumentaron (p< 0,001). Las citocinas también cambiaron en dirección a ausencia de respuesta a la alergia. La mediana de IL-17 disminuyó de 1,752 mg/dL a 0,417 mg/dL. Conclusión: En pacientes con rinitis alérgica, después del tratamiento específico de desensibilización sublingual, los niveles de IgE y citocinas como IL-6 e IL-17 se reducen significativamente y los niveles de IgG, IgG4 e IgG1 aumentan.

Trends of humoral immune responses to heterologous antigenic exposure due to vaccination & omicron SARS-CoV-2 infection: Implications for boosting

Background & objectives: Vaccination and natural infection can both augment the immune responses against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), but how omicron infection has affected the vaccine-induced and hybrid immunity is not well studied in Indian population. The present study was aimed to assess the durability and change in responses of humoral immunity with age, prior natural infection, vaccine type and duration with a minimum gap of six months post-two doses with either ChAdOx1 nCov-19 or BBV152 prior- and post-emergence of the omicron variant. Methods: A total of 1300 participants were included in this observational study between November 2021 and May 2022. Participants had completed at least six months after vaccination (2 doses) with either ChAdOx1 nCoV-19 or an inactivated whole virus vaccine BBV152. They were grouped according to their age (? or ?60 yr) and prior exposure of SARS-CoV-2 infection. Five hundred and sixteen of these participants were followed up after emergence of the Omicron variant. The main outcome was durability and augmentation of the humoral immune response as determined by anti-receptor-binding domain (RBD) immunoglobulin G (IgG) concentrations, anti-nucleocapsid antibodies and anti-omicron RBD antibodies. Live virus neutralization assay was conducted for neutralizing antibodies against four variants – ancestral, delta and omicron and omicron sublineage BA.5. Results: Before the omicron surge, serum anti-RBD IgG antibodies were detected in 87 per cent participants after a median gap of eight months from the second vaccine dose, with a median titre of 114 [interquartile range (IQR) 32, 302] BAU/ml. The levels increased to 594 (252, 1230) BAU/ml post- omicron surge (P<0.001) with 97 per cent participants having detectable antibodies, although only 40 had symptomatic infection during the omicron surge irrespective of vaccine type and previous history of infection. Those with prior natural infection and vaccination had higher anti-RBD IgG titre at baseline, which increased further [352 (IQR 131, 869) to 816 (IQR 383, 2001) BAU/ml] (P<0.001). The antibody levels remained elevated after a mean time gap of 10 months, although there was a decline of 41 per cent. The geometric mean titre was 452.54, 172.80, 83.1 and 76.99 against the ancestral, delta, omicron and omicron BA.5 variants in the live virus neutralization assay. Interpretation & conclusions: Anti-RBD IgG antibodies were detected in 85 per cent of participants after a median gap of eight months following the second vaccine dose. Omicron infection probably resulted in a substantial proportion of asymptomatic infection in the first four months in our study population and boosted the vaccine-induced humoral immune response, which declined but still remained durable over 10 months

Hypophysitis an uncommon manifestation of IgG4-related disease: Case report

IgG4-related disease is a recently described disease that can involve various organs and systems. Single organ involvement is the exception to the rule, it is generally a multi-system entity. We present a 36-year-old woman, with no previous pathological history or autoimmune disease, with headache caused by cystic macroadenoma. A transsphenoidal resection was performed and pathology documented areas of fibrosis with a predominantly plasmolymphocytic infíltrate and positive IgG4 staining in more than 20 cells per high-power field, meeting diagnostic criteria for IgG4-related sclerosing disease. Involvement of other organs was ruled out, and the patient improved clinically after management.

La enfermedad relacionada con IgG4 es una entidad recientemente descrita, capaz de involucrar diversos órganos y sistemas. El compromiso de órganos aislados es la excepción a la regla, dado que generalmente se trata de una entidad multisistémica. Se presenta el caso de una mujer de 36 años, sin antecedentes patológicos previos, en quien como causa de cefalea se documenta un macroadenoma quístico llevado a resección transesfenoidal, cuyo resultado de patología documenta zonas de fibrosis con infiltrado de predominio plasmo-linfocitario y la tinción para IgG4 positiva en más de 20 células por campo de alto poder, lo que configura criterios diagnósticos para enfermedad esclerosante relacionada con IgG4; se descartó compromiso de otros órganos y hubo mejoría clínica posterior al manejo.

Una inusual causa de estrechez de la vía aérea laringotraqueal / An unusual cause of laryngotracheal airway narrowing

La enfermedad relacionada con inmunoglobulina (Ig) G4 es una enfermedad de reciente conocimiento que puede comprometer cualquier órgano teniendo preferencias por ciertas regiones del cuerpo, donde la región de cabeza y cuello es uno de sus principales puntos afectados, pudiendo comprometer tanto la órbita, glándulas salivales, glándulas lagrimales, glándula tiroides, cavidades paranasales, hueso temporal, faringe y laringe. Este último órgano es infrecuentemente comprometido, solo existiendo 12 casos registrados en la literatura antes de la publicación de este escrito. Presentamos un caso de una mujer de 49 años con historia de disnea frente a esfuerzo, diagnosticándose una estenosis subglótica la cual fue manejada quirúrgicamente con una reconstrucción laringotraqueal. En el estudio histopatológico se evidenció histología compatible con enfermedad relacionada con IgG4, por lo que se inició tratamiento médico con corticotera- pia oral por un lapso de 2 meses en conjunto con inmunología. Paciente luego de 4 años de seguimiento, no ha presentado recaídas, manteniendo un lumen subglótico adecuado.

Immunoglobulin (Ig) G4-related disease is a medical condition of recent knowledge that can compromise any organ, having preferences for certain regions of the body, where the head and neck region is one of the main affected points, being able to affect orbit, salivary glands, lacrimal glands, thyroid gland, paranasal cavities, temporal bone, pharynx and larynx. The latter is infrequently compromised, with only 12 cases registered in the literature before the publication of this writing. We present a case of a 49-year-old woman with a history of exertional dyspnea, diagnosed with a sub- glottic stenosis which was managed surgically with laryngotracheal reconstruction. The histopathological study revealed histology compatible with IgG4-related disease, so medical treatment with oral corticosteroid therapy was started for a period of 2 months in conjunction with immunology. After 4 years of follow-up, the patient has not presented relapses, maintaining an adequate subglottic lumen.

Enfermedad relacionada con IgG4, enfermedad de Erdheim Chester y compromiso aórtico y/o sus ramas. Diagnóstico diferencial con otras vasculitis de grandes vasos / IgG4 related disease, Erdheim Chester disease and aortic involvement and/or its branches. Differential diagnosis with other large vessel vasculitis

La afección cardiovascular en entidades como Erdheim-Chester (EEC), una rara histiocitosis de células no Langerhans, y la enfermedad relacionada con IgG4 (ER-IgG4), una afección fibrinoinflamatoria inmunomediada, es muy variada y habitualmente asintomática hasta su progresión a daños irreversibles cuando no es sospechada. Ante la dificultad de realizar biopsias por el sitio anatómico, es fundamental valerse de características clínicas, demográficas o imagenológicas que puedan diferenciarlas de otras entidades, como las arteritis de grandes vasos. La población masculina, los mayores de 60 años con compromiso a nivel de la aorta abdominal infrarrenal o aneurismas a nivel de la aorta ascendente con o sin compromiso de otros órganos, son orientativos de ER-IgG4. En la EEC es característico el tejido blando concéntrico que recubre la aorta (aorta recubierta) y sobre todo ante la presencia de fibrosis retroperitoneal, compromiso de huesos largos, hidronefrosis, lesión renal aguda posrrenal e hipertensión arterial, existencia de tejido fibrótico perirrenal, engrosamiento de la fascia renal y tejido adiposo perirrenal (signo del riñón peludo).

Cardiovascular disease in entities such as Erdheim-Chester (ECD), a rare non-Langerhans cell histiocytosis, and IgG4-related disease (ER-IgG4), an immunemediated fibrinoinflammatory disease, is highly varied and usually asymptomatic until it progresses to irreversible damage if they are not taken into account. Given the difficulty of performing biopsies by the anatomical site, it is essential to use clinical, demographic or imaging characteristics that can differentiate them from other entities such as large vessel arteritis. Male population, over 60 years of age with involvement of the infrarenal abdominal aorta or aneurysms at the level of the ascending aorta with or without involvement of other organs, are indicative of ER-IgG4. In ECD, the concentric soft tissue covering the aorta (covered aorta) is characteristic, and especially in the presence of retroperitoneal fibrosis, involvement of long bones, hydronephrosis, post-renal acute kidney injury and arterial hypertension, the presence of perirenal fibrotic tissue, thickening of the renal fascia, perirenal adipose tissue (hairy kidney sign).

Exoftalmos bilateral por compromiso orbitario debido a enfermedad relacionada con IgG4 / Bilateral exophthalmos due to orbital involvement due to IgG4-related disease

La enfermedad relacionada con IgG4 (ER-IgG4) se caracteriza por el compromiso fibrótico e infiltrado linfoplasmocitario con predominio de células IgG4 en uno o varios órganos. El fenotipo limitado a la cabeza y el cuello abarca el compromiso de las glándulas lagrimales y salivares, enfermedad orbitaria, paquimeningitis, hipofisitis, tiroiditis, mastoiditis y compromiso de senos paranasales. Las manifestaciones orbitarias pueden incluir proptosis uni o bilateral, miopatía cicatrizal de los músculos extraoculares, inflamación orbitaria y dolor que pueden simular a la oftalmopatía de Graves. La enfermedad oftálmica relacionada con IgG4 puede presentarse con compromiso de músculos extraoculares y de grasa orbitaria, y usualmente se confunde con afección orbitaria por enfermedad de Graves. Se presenta el caso de un varón de 58 años, con historia de exoftalmos bilateral de más de 15 años de evolución, en quien se arriba al diagnóstico de ER-IgG4 y cuya resonancia magnética de órbitas evidencia un patrón característico de compromiso de músculos extraoculares.

IgG4-related disease (IgG4-ER) is characterized by fibrotic involvement and lymphoplasmacytic infiltrate with IgG4 cells predominance in one or several tissues. The head and neck phenotype includes lacrimal and salivary gland involvement, orbital disease, pachymeningitis, hypophysitis, thyroiditis, mastoiditis, and sinus involvement. Orbital manifestations may include unilateral or bilateral proptosis, extraocular muscles scarring, orbital inflammation, and pain that may mimic Graves' ophthalmopathy. IgG4-related ophthalmic disease may present with extraocular muscles and orbital fat involvement and is often confused with orbital Graves' disease. We present the case of a 58-year-old man, with a history of bilateral exophthalmos of more than 15 years, in whom the diagnosis of IgG4-ER was reached and whose orbits magnetic resonance showed a characteristic pattern of extraocular muscle involvement.

Serological analysis of varicella-zoster virus IgG antibody in healthy people aged 1～30 years in Liaoning Province / 中国生物制品学杂志

@#Abstract：Objective To carry out serological analysis of varicella⁃zoster virus（VZV）IgG antibody level in healthy people aged 1 ~ 30 years in Liaoning Province. Methods In October 2020，3～5 mL venous blood samples were collected from 617 healthy people aged 1～30 years selected from six counties and districts in Shenyang，Fuxin and Dandong of Liaoning Province by stratified random sampling method，of which serum samples were collected and determined for VZV IgG antibody level by ELISA. The positive rate of serum antibody and geometric mean concentration（GMC）of antibody were calculated and compared. Results Among 617 serum samples，302 samples were positive for VZV IgG antibody，the positive rate was 48. 947%，and the GMC was 112. 772 mIU／mL. The positive rate of VZV IgG antibody was 29. 670%～75. 789% and the GMC was 45. 508～366. 559 mIU／mL in healthy people of various ages. Both of the antibody positive rate（χ2 = 67. 104， P < 0. 001）and GMC（F = 20. 685，P < 0. 001）showed significant differences. The positive rates of VZV IgG antibody in male and female were 44. 817% and 53. 633% respectively，which showed significant difference（χ2 = 4. 779，P = 0. 029）， while the GMCs were 96. 983 and 133. 829 mIU／mL respectively（t = -1. 958，P = 0. 051）with no significant difference. The positive rates of VZV IgG antibody of healthy people in Shenyang，Fuxin and Dandong of Liaoning Province were 55. 224%，40. 201% and 51. 152% respectively with significant differences（χ2 = 9. 683，P = 0. 008），of which the positive rate of FuxinwassignificantlylowerthanthoseofShenyangandDandong（χ2 =9. 046and5. 013，P =0. 003and0. 025，respectively）； While the GMCs were 133. 523，85. 953 and 123. 713 mIU／mL respectively with no significant difference（F = 0. 514， P = 0. 598）. Among 617 serum samples，54 sampleswere suspicious，which remained within the criticalrange afterre⁃examina⁃ tion，while the gap between positive rate and the total percentage of positive and suspicious results gradually decreased with the increase of age，indicating that the immunity to varicella gradually increased with the increase of age. Conclusion The VZV⁃IgG antibody level of healthy people aged 1～30 years in Liaoning Province increased gradually with age，while the overall level was low. To control the spread of varicella virus，it is recommended to increase varicella vaccine coverage in vulnerable areas and susceptible population to build VZV immune barrier.

Detection of helicobacter pylori infection by helicobacter pylori IgG serology test in pediatric patients at the Philippine General Hospital

Objective@#To determine the validity of serum H. pylori IgG in the detection of H. pylori-associated gastroduodenitis in patients with gastrointestinal symptoms. @*Methods@#Cross-sectional study which included consecutive patients 1-18 years old with upper gastrointestinal symptoms who underwent esophagogastroduodenoscopy. H. pylori infection was diagnosed by positive tests for both rapid urease test (RUT) and Giemsa stain of gastric biopsies. H. pylori IgG (ELISA) serology was also performed.@*Results@#Twenty-five patients [Mean (SD) age: 12 (4.5) years, 68% females] were included. Majority presented with epigastric pain (64%) and had endoscopic gastritis (84%). Four patients had ulcers (1 antral, 3 duodenal). Giemsa stain was positive in 16 (64%) patients and RUT in one. Prevalence of H. pylori infection was 4%. Serum H. pylori IgG test was positive in two; borderline in three with a 100% sensitivity, 80% specificity, and a positive and negative likelihood ratio of 10.9 and 0.6.@*Conclusion@#The present study showed a low prevalence of H. pylori infection, thus, the validity of the H. pylori serology could not be adequately evaluated. We presently could not recommend the serum IgG in the detection of H. pylori gastroduodenitis in our setting.

Results of serum antibody detection from patients with hemorrhagic fever with renal syndrome in Heilongjiang, 2019-2021 / 中国热带医学

@#Abstract: Objective　To detect the antibody levels of hantavirus in serum samples from patients suspected with hemorrhagic fever with renal syndrome (HFRS) in Heilongjiang Province from 2019 to 2021, and to provide scientific basis for the prevention and control of disease. Methods　Enzyme-linked immunosorbent assays (ELISA) were used to detect the IgM antibodies to hantavirus in serum samples collected from suspected patients with HFRS in the acute-phase, and IgM and IgG antibody in convalescent-phase serum samples. The positive rate of IgM antibody in acute-phase serum samples of patients in different years was analyzed with χ2 test by SPSS 19.0, and the data were sorted out and analyzed about patients' gender, occupation, age, date of onset and interval from onset to initial diagnosis by EpiData 3.1, Excel 2003 software. Results　A total of 351 acute-phase serum samples and 208 convalescent-phase serum samples were detected in patients suspected with HFRS, respectively. There were 317 positive IgM antibodies of serum samples in the acute stage, with the positive rate of 90.31%. There was no significant difference in the positive rate of IgM antibodies in the acute stage between different years (χ2=0.895, P=0.639). T The IgM antibodies and IgG antibodies were positive in 32 (15.39%) and 28 (13.46%) of the convalescent-phase serum samples, respectively. Moreover, 148 patients (71.15%) were double-positive for IgM and IgG antibodies at the convalescent stage. The ratio of male to female patients was 4.56∶1, for which male patients were much more than female patients. Occupation was dominated by farmers (253 cases, 79.81%), followed by workers (19 cases, 5.99%) and the unemployed (17 cases, 5.36%), respectively. The age of patients ranged from 10 to 88 years old, with a median age of 49 years old. Most of the patients were in the age group from 30 years old to 60 years old (209 cases, 65.93%), among which the age group from 40 years old to 50 years old (86 cases, 27.13%) had the highest proportion, and the age group from 60 years old to 90 years old had a proportion of 20.18% (19 cases). May and November were the peak periods of HFRS in Heilongjiang Province. The median interval between onset and initial diagnosis was 4 days. Conclusions　There is a gap of about 10% between the clinical diagnosis of HFRS cases and the confirmed cases detected by laboratory in Heilongjiang Province from 2019 to 2021. The virus-specific detection results are important for confirming the diagnosis of local patients with HFRS.

Control of lupus activity during pregnancy via the engagement of IgG sialylation: novel crosstalk between IgG sialylation and pDC functions / 医学前沿

Immunoglobulin (IgG) glycosylation affects the effector functions of IgG in a myriad of biological processes and has been closely associated with numerous autoimmune diseases, including systemic lupus erythematosus (SLE), thus underlining the pathogenic role of glycosylation aberration in autoimmunity. This study aims to explore the relationship between IgG sialylation patterns and lupus pregnancy. Relative to that in serum samples from the control cohort, IgG sialylation level was aberrantly downregulated in serum samples from the SLE cohort at four stages (from preconception to the third trimester of pregnancy) and was significantly associated with lupus activity and fetal loss during lupus pregnancy. The type I interferon signature of pregnant patients with SLE was negatively correlated with the level of IgG sialylation. The lack of sialylation dampened the ability of IgG to suppress the functions of plasmacytoid dendritic cells (pDCs). RNA-seq analysis further revealed that the expression of genes associated with the spleen tyrosine kinase (SYK) signaling pathway significantly differed between IgG- and deSia-IgG-treated pDCs. This finding was confirmed by the attenuation of the ability to phosphorylate SYK and BLNK in deSia-IgG. Finally, the coculture of pDCs isolated from pregnant patients with SLE with IgG/deSia-IgG demonstrated the sialylation-dependent anti-inflammatory function of IgG. Our findings suggested that IgG influences lupus activity through regulating pDCs function via the modulation of the SYK pathway in a sialic acid-dependent manner.

The serum levels of FCGBP, SAA1, CXCL10 in children with mycoplasma pneumoniae pneumonia and their relationship with prognosis / 中国医师杂志

Objective:To investigate the serum levels and clinical significance of Fc fragment of the IgG-binding protein (FCGBP), serum amyloid protein A1 (SAA1), and CXC chemokine ligand 10 (CXCL10) in children with mycoplasma pneumoniae pneumonia (MPP) and their relationship with prognosis.Methods:A prospective study was conducted on 122 children with MPP admitted to the department of pediatrics of the 970th Hospital of the Joint Logistics Support Force of the Chinese People′s Liberation Army from January 2019 to December 2021. According to the severity and prognosis of MPP, they were divided into mild and severe groups, good prognosis group, and poor prognosis group. Forty healthy children who underwent physical examination during the same period were set as the control group. Enzyme-linked immunosorbent assay (ELISA) was used to detect the serum levels of FCGBP, SAA1, and CXCL10 in each subject, and to compare the differences in serum levels of FCGBP, SAA1, and CXCL10 among different groups. Multivariate logistic regression analysis was used to investigate the influencing factors of poor prognosis in MPP patients. The diagnostic value of individual and combined detection of serum procalcitonin (PCT), FCGBP, SAA1, and CXCL10 for poor prognosis in MPP children by analyzing the receiver operating characteristic (ROC) curve.Results:The levels of serum FCGBP [(115.68±10.57)ng/ml, (78.41±6.73)ng/ml, (12.55±3.25)ng/ml], SAA1 [(34.18±3.72)mg/L, (25.54±2.63)mg/L, (6.74±0.82)mg/L], and CXCL10 [(714.26±55.64)ng/L, (353.74±42.67)ng/L, (106.25±12.92)ng/L] in the severe MPP group were significant higher than those in the mild MPP group and the control group, with statistical significance (all P<0.05). The white blood cell (WBC), neutrophil percentage, C reactive protein (CRP), erythrocyte sedimentation rate (ESR), PCT, lactate dehydrogenase (LDH), D-dimer (D-D), FCGBP, SAA1, CXCL10 of the children in the poor prognosis group were significantly higher than those in the good prognosis group, and the differences were statistically significant (all P<0.05). Multivariate logistic regression analysis showed that increased PCT ( OR=1.603, 95% CI: 1.190-2.160), FCGBP ( OR=1.757, 95% CI: 1.115-2.770), SAA1 ( OR=1.900, 95% CI: 1.327-2.720) and CXCL10 ( OR=1.704, 95% CI: 1.212-2.397) were independent risk factors for poor prognosis of MPP children (all P<0.05). The combined detection of serum PCT, FCGBP, SAA1, and CXCL10 had a significantly higher diagnostic value for the risk of poor prognosis in children with MPP than a single indicator. Conclusions:The elevated levels of serum FCGBP, SAA1, and CXCL10 in children with MPP are associated with the severity of MPP and are independent risk factors for poor prognosis in MPP patients.

Determination of the ELISA kits for screening convalescence plasma with high potency of SARS-CoV-2 IgG / 中国输血杂志

【Objective】 To determine the ELISA kit for screening convalescence plasma with high potency of SARS-CoV-2 IgG by comparing and analyzing the plasma detection results of convalescent plasma collected in different periods via ELISA kits from two manufacturers and the results of mixed plasma with different potency via pseudovirus neutralization experiments. 【Methods】 Two ELISA kits from different manufacturers(named A, B) were used to detect the plasma of 269 convalescent patients collected from Feb.2020~Jan.2022. The correlation and concordance rate of the two results were analyzed to determine the kit preliminarily. According to the titers of diluted series of standard of the preliminary selected kit, 5 mixed plasma samples (G4-G128) with different potency were prepared. The correlation of ELISA IgG results of product A/B, as well as the pseudovirus neutralization test of the original strain, Omicron mutant BA.1 and BA.2 strains were analyzed. Combined with the outside-well dilution mode of the strongly positive samples, the kit for high potency of SARS-CoV-2 IgG screening was determined. 【Results】 When the internal control reference B2 was used as the standard, the detection sensitivity of product A and B was 1∶32 vs 1∶8; the detection sensitivity of product A was 4 times that of product B. The correlation Pearson r between the results given by two kits was 0.944 1(P<0.000 1). Product B with low sensitivity was primarily selected as an alternative kit. The ELISA IgG results of samples from mixed plasma showed that the order of correlation r between product A and B was 0.988. The correlation r between product A and neutralization antibody potency of the three viruses was original strain (0.978)>BA.2(0.970)>BA.1(0.799); the order of correlation r between ELISA IgG results of product B and neutralization antibody potency of the three viruses was original strain(0.994)>BA.2(0.968)>BA.1(0.804). If twice-diluted B2 was taken as the excellent standard, 55.4% of product B met the criterion, while 47.2% of product A met.For positive plasma with high IgG potency, the product B kit required a lower dilution of the sample, which was more convenient to operate. 【Conclusion】 Both of the ELISA IgG kit from product A and B can be used to screen IgG antibodies of SARS-CoV-2, while product B is more suitable for screening positive plasma with high IgG potency.

Effect of IgG dimer concentration on the binding ability of IgG Fc fragment in IVIG to THP-1 cell surface receptors / 中国输血杂志

【Objective】 To investigate the effect of immunoglobulin G (IgG) dimer concentration of intravenous immunoglobulin (IVIG) on the binding ability of IgG Fc fragment to THP-1 cell surface receptors. 【Methods】 Firstly, protein purification and high performance liquid chromatography (HPLC) were used to prepare different concentrations of IgG dimers. After that, IgG dimer was added to IVIG to prepare IVIG containing different concentrations of IgG dimer. Finally, based on the method established in our laboratory, we analyzed the effect of IgG dimer concentration in IVIG on the binding ability of IgG Fc fragment to THP-1 cell surface receptors. 【Results】 When the concentration of IgG dimer in IVIG was 1.11%-10.30%, its binding ability to Fc receptors on the surface of THP-1 cell was 97.67%-135.33%, and this binding ability was positively correlated with the concentration of IgG dimer. When the IgG dimer concentration exceeded 13.22%, the binding ability had no correlation with the IgG dimer concentration. 【Conclusion】 A certain concentration of IgG dimer can promote the binding ability of the IgG Fc fragment in IVIG to receptors on the surface of THP-1 cells, which needs further verification from animal experiments and clinical data.