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Summary: Background. Papular Urticaria (PU) is a cutaneous hypersensitivity disorder triggered by hematophagous arthropod bites. Despite being a common condition, especially in tropical environments, many knowledge gaps are observed for this disease. The main objective of this study was to investigate the patterns of humoral immune response to mosquito antigens in children with PU and establish a correlation between this response and the severity of clinical symptoms. Methods. An analytical cross-sectional observational study was carried out. Clinical and sociodemographic data and children's blood samples were collected to measure the specific antibodies from: 1. A. aegypti salivary gland antigens; 2. A. aegypti whole body antigens (both produced in the laboratory of the Center for Health Sciences at the Federal University of Rio de Janeiro). A PU severity score based on clinical data is proposed to correlate disease severity with antibody reactivity signatures. Results. According to the clinical data, 58.9% of children received high severity scores. A significant statistical correlation was found between patients with high PU severity score and the development of symptoms before the age of two (p = 0.0326) and high IgG4 anti-salivary gland antigens concentration (p less than 0.05). Conclusion. It is suggested that PU severity in children is associated with a high concentration of IgG4 anti-salivary gland antigens from Aedes aegypti. Further studies are recommended to deepen the understanding of the mechanisms involved.

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PURPOSE: This study aimed to characterize the histopathological immunohistochemical features of chronic sclerosing sialadenitis, emphasizing the IgG4-related disease. METHODS: Seventeen cases of chronic sclerosing sialoadenitis were examined for histopathological aspects, (inflammation, fibrosis, glandular parenchyma, and lymphoid follicles) and immunohistochemistry (BCL2, CD3, CD20, CD34, CD163, p63, cyclin D1, mast cell, SMA, S100A4, IgG, and IgG4) which were scored. IgG4-related disease features were investigated. Demographic and clinical data were also collected. RESULTS: Males predominated (10:7), with an average lesion size of 3.9 cm. Common histopathological findings included reduced acinar parenchyma, lymphoid follicle formation, and ductular proliferation. CD3-positive T lymphocytes and CD34- and SMA-positive stromal fibroblasts were abundant. Nine cases (53%) showed sialoliths and three cases met the criteria for IgG4-related disease. CONCLUSION: CSS of the submandibular gland represents a reactive pattern rather than IgG4-RD as only 3 cases seemed to be related to IgG4-RD. The immunohistochemical profile revealed an abundant population of CD3-positive T lymphocytes, as opposed to regulatory proteins such as cyclin D1, demonstrating that populations of CD34- and SMA-positive stromal fibroblasts contribute to the fibrosis characteristic of CSS. In addition, our results provide a comprehensive insight into the study of CSS and its relationship with IgG4-RD.

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Immunoglobulin G4-related disease is a systemic immune-mediated disease with insidious evolution characterized by fibroinflammatory lesions over virtually any organ system. Despite the remarkable progression of knowledge, its etiology remains undefined. Due to its relapse-remitting pattern, it could accumulate irreversible damage, increasing comorbidities and mortality. This paper emphasizes key concepts for diagnosing and treating patients with this condition.

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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.

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Humoral response to SARS-CoV-2 has been studied, predominantly the classical IgG and its subclasses. Although IgE antibodies are typically specifc to allergens or parasites, a few reports describe their production in response to SARS-CoV-2 and other viruses. Here, we investigated IgE specifc to receptor binding domain (RBD) of SARS-CoV-2 in a Brazilian cohort following natural infection and vaccination. Samples from 59 volunteers were assessed after infection (COVID-19), primary immunization with vectored (ChAdOx1) or inactivated (CoronaVac) vaccines, and booster immunization with mRNA (BNT162b2) vaccine. Natural COVID-19 induced IgE, but vaccination increased its levels. Subjects vaccinated with two doses of ChAdOx1 exhibited a more robust response than those immunized with two doses of CoronaVac; however, after boosting with BNT162b2, all groups presented similar IgE levels. IgE showed intermediate-to-high avidity, especially after the booster vaccine. We also found IgG4 antibodies, mainly after the booster, and they moderately correlated with IgE. ELISA results were confrmed by control assays, using IgG depletion by protein G and lack of reactivity with heterologous antigen. In our cohort, no clinical data could be associated with the IgE response. We advocate for further research on IgE and its role in viral immunity, extending beyond allergies and parasitic infections. (AU)

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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.

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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.

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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.

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SARS-CoV-2, the virus that causes the COVID-19 disease, has been shown to cause immune suppression in certain individuals. This can manifest as a reduced ability of the host's immune system to effectively control the infection. Studies have reported that patients with COVID-19 can exhibit a decline in white blood cell counts, including natural killer cells and T cells, which are integral components of the immune system's response to viral pathogens. These cells play critical roles in the immune response to viral infections, and their depletion can make it harder for the body to mount an effective defense against the virus. Additionally, the virus can also directly infect immune cells, further compromising their ability to function. Some individuals with severe COVID-19 pneumonia may develop a "cytokine storm", an overactive immune response that may result in tissue damage and organ malfunction. The underlying mechanisms of immune suppression in SARS-CoV-2 are not entirely understood at this time, and research is being conducted to gain a more comprehensive understanding. Research has shown that severe SARS-CoV-2 infection promotes the synthesis of IgG4 antibodies. In this study, we propose the hypothesis that IgG4 antibodies produced by B cells in response to infection by SARS-CoV-2 generate immunological tolerance, which prevents its elimination and leads to persistent and chronic infection. In summary, we believe that this constitutes another immune evasion mechanism that bears striking similarities to that developed by cancer cells to evade immune surveillance.

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Intestinal schistosomiasis is a chronic and debilitating disease that affects public health systems worldwide. Control interventions to reduce morbidity primarily involve the diagnosis and treatment of infected individuals. However, the recommended Kato-Katz (KK) parasitological method shows low sensitivity in individuals with low parasite loads and is not useful for monitoring elimination of parasite transmission at later stages. In the current study, we evaluated the accuracy of serum reactivity levels of different immunoglobulin isotypes in an enzyme-linked immunosorbent assay (ELISA), utilizing Schistosoma mansoni crude extracts, with the aim to improve the diagnosis of infected individuals with low parasite loads. The serum reactivity of IgM and IgG subclass antibodies (IgG1, IgG3, and IgG4) against soluble adult worm and egg antigen preparations was evaluated in residents from a schistosomiasis-endemic area in northern Minas Gerais, Brazil. The parasitological status of the study population was determined through fecal examination with multiple parasitological tests to create a consolidated reference standard (CRS) plus a fecal DNA detection test (q-PCR). Twelve months after praziquantel treatment, a second serum sample was obtained from the population for reexamination. A two-graph receiver operating characteristic curve (TG-ROC) analysis was performed using the serum reactivity of non-infected endemic controls and egg-positive individuals, and the cut-off value was established based on the intersection point of the sensibility and specificity curves in TG-ROC analyses. The diagnostic accuracy of each serological test was evaluated in relation to the parasitological CRS and to the combination of CRS plus qPCR results. The data revealed that serum reactivity of IgM and IgG3 against S. mansoni antigens did not allow identification of infected individuals from the endemic area. In contrast, serum IgG1 and IgG4-reactivity against schistosome antigens could distinguish between infected and non-infected individuals, with AUC values ranging between 0.728-0.925. The reactivity of IgG4 anti-soluble egg antigen - SEA (sensitivity 79 %, specificity 69 %, kappa = 0.49) had the best diagnostic accuracy, showing positive reactivity in more than 75 % of the infected individuals who eliminated less than 12 eggs per gram of feces. Moreover, serum IgG4 reactivity against SEA and against soluble worm antigen preparation (SWAP) was significantly reduced in the serum of infected individuals after 12 months of confirmed parasitological cure and in the absence of re-infection. These results reinforce that the described IgG4 anti-SEA ELISA assay is a sensitive alternative for the diagnosis of active intestinal schistosomiasis in individuals from endemic areas, including in those with a very low parasite load.

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INTRODUCTION: Hypertrophic pachymeningitis (HP) is a clinico-radiological entity characterized by a thickening of the dura mater that may be focal or diffuse and manifested by a variety of neurological syndromes. Aetiologically, it is classified as infectious, neoplastic, autoimmune, and idiopathic. Many of these formerly idiopathic cases have been shown to fall into the spectrum of IgG4-related disease. OBJECTIVE: To describe the case of a patient attended for neurological involvement due to hypertrophic pachymeningitis with initial diagnosis of inflammatory myofibroblastic tumour and final diagnosis of IgG4-related disease. CASE: A 25-year-old woman with neurological symptoms of 3 years' evolution characterized initially by right hypoacusis, evolving with headache and diplopia. Magnetic resonance imaging (MRI) of the encephalon showed pachymeningeal thickening with involvement of vasculo-nervous structures in the tip of the cerebellum, cavernous sinus, ragged foramen, and optic chiasm. The patient presented for consultation with the result of an incisional biopsy that reported a proliferative lesion combining fibrous elements of fascicular or swirling arrangement with collagenized streaks with dense, lymphoplasmacytic infiltrate and some macrophages, with negative staining for ALK 1, with a diagnosis of inflammatory myofibroblastic tumour. Due to suspicion of IgG4-related disease (IgG4-RD) the biopsy was sent for review and pertinent complementary studies were requested. BIOPSY REVIEW: Non storiform fibrosis, predominantly lymphoplasmacytic infiltrate, histiocytes, and polymorphonuclear infiltrate in sectors, without granulomas or atypia. Staining for germs negative. Immunohistochemistry with 50-60 IgG4+/HPF cells and range of 15%-20%, CD68+ in histiocytes, CD1a-, S100-. The patient presented deterioration of visual acuity due to ophthalmic nerve involvement, so glucocorticoid treatment was started in pulses and rituximab with regression of symptoms and imaging improvement of the lesions. CONCLUSION: HP is a clinical imaging syndrome with variable symptoms and aetiologies that poses a diagnostic challenge. In this case the initial diagnosis was inflammatory myofibroblastic tumour, which is a neoplasm of variable behaviour, locally aggressive, and can metastasize; it is one of the main differential diagnoses of IgG4-related disease because they share anatomopathological features, including storiform fibrosis. IgG4-RD is an immune-mediated condition that can have single or multiple involvement. Its diagnosis is complex when it presents with single organ involvement or in non-typical organs (CNS, meninges) in which data are scarce, as in the case of our patient with single organ involvement of the CNS. Although there are classification criteria to guide non-specialists in the diagnosis, the sum of the clinical picture, imaging, laboratory, pathological anatomy, and immunohistochemistry will always be evaluated together for a definitive diagnosis.

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Isolated external iliac artery aneurysm is a rare occurrence. These aneurysms have varied presentations depending on size and proximity. Both open surgical and endovascular modalities can be used for treatment depending upon presentation, aneurysmal anatomy, and patient condition. Preservation of at least one internal iliac artery is important to prevent post-repair hypogastric ischemia. There are no previous reports of IgG4-related disease (IgG4-RD) as etiology of these aneurysms. A 32-year-old male patient presented with a left lower abdominal lump and was found to have a left external iliac artery aneurysm on computed tomography angiography. The patient underwent iliofemoral bypass with an 8 mm polyester graft. Histopathological examination of the aneurysm wall suggested IgG4-RD. The patient fulfilled the 2020 Revised Comprehensive Diagnostic Criteria for IgG4-RD. An 18-Fluorodeoxyglucose-Positron Emission Tomography scan performed in the postoperative period showed no active disease, hence medical therapy was not instituted. The patient is doing well at 1 year.

O aneurisma isolado da artéria ilíaca externa é uma ocorrência rara. Esses aneurismas têm apresentações variadas, dependendo do tamanho e da proximidade. Ambas as modalidades cirúrgicas aberta e endovascular podem ser usadas para o tratamento, dependendo da apresentação, anatomia do aneurisma e condição do paciente. A preservação de pelo menos uma artéria ilíaca interna é importante para prevenir isquemia hipogástrica pós-reparação. A doença relacionada à imunoglobulina G4 (IgG4-RD) nunca havia sido encontrada como etiologia desse aneurisma. Um paciente do sexo masculino de 32 anos que apresentava um nódulo no abdome inferior esquerdo foi diagnosticado com aneurisma da artéria ilíaca externa esquerda na angiotomografia computadorizada. O paciente foi submetido a bypass iliofemoral com enxerto de poliéster de 8 mm. O exame histopatológico da parede do aneurisma era indicativo de IgG4-RD. O paciente cumpriu os Critérios Abrangentes Revisados ​​para IgG4-RD de 2020. A tomografia por emissão de pósitrons com 18-fluorodesoxiglicose no pós-operatório não evidenciou doença ativa, não sendo instituída terapia medicamentosa. Após seguimento de 1 ano, o paciente está bem.

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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.

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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.

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We aim to assess and compare a cytokine and chemokine profile in tears from patients with IgG4-related disease (IgG4-RD) and Sjögren's syndrome (SS), and to see if this profile could aid in differentiating these two diseases. We included 10 patients with IgG4-RD who met the Comprehensive Diagnostic Criteria for IgG4-RD and 17 patients who met the AECG criteria for primary SS. The Schirmer-I test was carried out using two standardized sterile tear strips, which were then immediately frozen at - 86 °C until assayed. The tears were extracted from the strips after they had been defrosted using a buffer containing 0.5 M NaCl and 0.5% Tween-20. The amounts (pg/ml) of the following cytokines and chemokines were then measured using luminometry: IFN-γ, TNF-α, G-CSF, IL-1-α, IL-1ß, IL-4, IL-7, IL-12p40, IL-12p70, IL-13, IL-17A, CCL2, CCL3, CCL4, CCL11, and CXCL10. In the IgG4-RD group, seven patients had lacrimal gland involvement, five had dry eye symptoms, and six had a positive Schirmer-I test. In the SS group, 16 (94.1%) had dry eyes and all had a positive Schirmer-I test. We were able to differentiate between both diseases using levels of IL-7, IL-1α, and IL-1ß; in particular, the IL-7/IL-1α and IL-7/IL-1ß ratios had the best discriminatory potential, with cut-off values of 0.32 (AUC: 0.93, sensitivity: 94%, specificity: 80%, p = 0.0003) and 12.55 (AUC: 0.96, sensitivity: 94%, specificity: 90%, p = 0.0001), respectively. Our results suggest that IL-7, IL-1α, and IL-1ß tear levels could help differentiate IgG4-RD from SS. Key Points • The lacrimal gland is frequently involved in IgG4-RD and SS. This characteristic makes both diseases mimics of one another. • Patients with IgG4-RD and SS have different profiles of tear cytokines and chemokines. • Tear IL-7, IL-1α, and IL-1ß levels may serve as helpful biomarkers in separating IgG4-RD from SS.

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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).

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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.

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Introducción: la enfermedad renal relacionada con IgG4 (ERR-IgG4) presenta una prevalencia del 8 al 24%, es más frecuente en el sexo masculino (73-87%), con una edad media de 65 años. Objetivos: determinar los hallazgos del compromiso renal y su frecuencia en pacientes con enfermedad relacionada con IgG4 (ER-IgG4); describir y comparar las características demográficas, clínicas, datos de laboratorio y tratamiento en pacientes con ER-IgG4 con afectación renal versus sin afectación renal. Materiales y métodos: estudio observacional retrospectivo de pacientes con compromiso renal (ERR-IgG4) en una cohorte de pacientes con ER-IgG4. Los datos se recolectaron mediante revisión de historias clínicas. Se describen parámetros clínicos basales, hallazgos histológicos y radiológicos, y tratamientos. Resultados: de 44 pacientes con ER-IgG4, 6 (13,6%) tenían ERR-IgG4 con una edad de 50,8±17,1 años y 4 eran mujeres. Todos presentaron compromiso en otros órganos. Los hallazgos radiológicos más comunes fueron las lesiones de baja densidad en la tomografía. A 3 pacientes se les realizó biopsia renal, siendo el principal diagnóstico histopatológico la nefritis tubulointersticial por IgG4. Todos los pacientes recibieron tratamiento con glucocorticoides e inmunosupresores como manejo inicial. Los pacientes con enfermedad renal, a diferencia de aquellos sin compromiso renal, mostraron mayor número de órganos afectados y niveles séricos de IgG2, IgG3, y nivel más bajo de C3. Conclusiones: en general, las características de los pacientes con ER-IgG4 y compromiso renal fueron similares a los trabajos publicados.

Abstract Introduction: IgG4-related Kidney desease (IgG4-RKD) has a prevalence of 8 to 24%, more frequent in males (73-87%), with a mean age of 65 years. Objectives: to determinate the kidney involvement finding and their frequency in patients with IgG4-related disease (IgG4-RD). To describe and compare demographic, clinical characteristics, laboratory data and treatment among patients with IgG4-RD who have kidney involvement versus patients without kidney involvement. Materials and methods: we conducted a retrospective observational study of patients with IgG4-related kidney (IgG4-RKD) in a IgG4-RD cohort. Data were collected through review of medical records. We describe clinical parameters at baseline, histological and radiological findings and treatment. Results: of 44 patients with IgG4-RD, 6 (13.6%) had IgG4-RKD. Patients were aged 50,8±17,1 years, and 4 were female. All of them had involvement the other organs systems. The most common radiological finding was low-density lesions on enhanced CT. Three patients had a renal biopsy, the main histopathological diagnosis being IgG4 tubulointerstitial nephritis. All patients received glucocorticoids and immunosupresor as initial management. Patients with kidney disease, as opposed to patients without kidney involvement had a higher number of involved organs, and IgG2, IgG3 serum levels and lower C3 level. Conclusions: in general, the characteristics of patients with ER-IgG4 and kidney involvement were similar to published works.

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Abstract Isolated external iliac artery aneurysm is a rare occurrence. These aneurysms have varied presentations depending on size and proximity. Both open surgical and endovascular modalities can be used for treatment depending upon presentation, aneurysmal anatomy, and patient condition. Preservation of at least one internal iliac artery is important to prevent post-repair hypogastric ischemia. There are no previous reports of IgG4-related disease (IgG4-RD) as etiology of these aneurysms. A 32-year-old male patient presented with a left lower abdominal lump and was found to have a left external iliac artery aneurysm on computed tomography angiography. The patient underwent iliofemoral bypass with an 8 mm polyester graft. Histopathological examination of the aneurysm wall suggested IgG4-RD. The patient fulfilled the 2020 Revised Comprehensive Diagnostic Criteria for IgG4-RD. An 18-Fluorodeoxyglucose-Positron Emission Tomography scan performed in the postoperative period showed no active disease, hence medical therapy was not instituted. The patient is doing well at 1 year.

Resumo O aneurisma isolado da artéria ilíaca externa é uma ocorrência rara. Esses aneurismas têm apresentações variadas, dependendo do tamanho e da proximidade. Ambas as modalidades cirúrgicas aberta e endovascular podem ser usadas para o tratamento, dependendo da apresentação, anatomia do aneurisma e condição do paciente. A preservação de pelo menos uma artéria ilíaca interna é importante para prevenir isquemia hipogástrica pós-reparação. A doença relacionada à imunoglobulina G4 (IgG4-RD) nunca havia sido encontrada como etiologia desse aneurisma. Um paciente do sexo masculino de 32 anos que apresentava um nódulo no abdome inferior esquerdo foi diagnosticado com aneurisma da artéria ilíaca externa esquerda na angiotomografia computadorizada. O paciente foi submetido a bypass iliofemoral com enxerto de poliéster de 8 mm. O exame histopatológico da parede do aneurisma era indicativo de IgG4-RD. O paciente cumpriu os Critérios Abrangentes Revisados ​​para IgG4-RD de 2020. A tomografia por emissão de pósitrons com 18-fluorodesoxiglicose no pós-operatório não evidenciou doença ativa, não sendo instituída terapia medicamentosa. Após seguimento de 1 ano, o paciente está bem.