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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.
ABSTRACT
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.
ABSTRACT
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.
Subject(s)
Immunoglobulin G4-Related Disease , Orbital Neoplasms , Eye DiseasesABSTRACT
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.
Subject(s)
Humans , Female , Adult , Brain Diseases , Immunoglobulin G , Proteins , Hypophysitis , Amino Acids, Peptides, and Proteins , Nervous System DiseasesABSTRACT
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.
ABSTRACT
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).
ABSTRACT
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.
ABSTRACT
Lolium multiflorum grass is the major pollen allergen source in the southern region of Brazil, but most of its allergens remain poorly characterized. The aim of this study was to investigate antibody reactivity to L. multiflorum crude and carboxymethyl-ligand extracts in allergic patients and healthy individuals. Ion exchange carboxymethyl (CM) chromatography (CM-Sepharose) was used to isolate proteins (S2) from L. multiflorum crude extract (S1), which were assessed by SDS-PAGE. S1- and S2-specific IgE and IgG4 levels were measured by ELISA using sera from 55 atopic and 16 non-atopic subjects. Reactive polypeptide bands in S1 and S2 were detected by immunoblotting, and the most prominent bands in S2 were analyzed by mass spectrometry (MS-MS). Similar IgE and IgG4 levels were observed to both S1 (IgE median absorbance: 1.22; IgG4 median absorbance: 0.68) and S2 (IgE median absorbance: 1.26; IgG4 median absorbance: 0.85) in atopic subjects. S1 and S2 had positive correlations for IgE and IgG4 (IgE: r=0.9567; IgG4: r=0.9229; P<0.0001) levels. Homology between S1 and S2 was confirmed by IgE (84%) and IgG4 (83%) inhibition. Immunoblotting revealed that the 29-32 kDa band was recognized by 100% of atopic subjects in both S1 and S2. MS-MS analysis identified similarity profile to groups 1 and 5 grass allergens. This study revealed that carboxymethyl-ligand fraction played an important role for pollen allergy diagnosis by containing clinically relevant allergens and constituted a promising candidate for allergen-specific immunotherapy.
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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.
ABSTRACT
@#Almost a year after the worldwide appearance of the coronavirus (SARS-CoV-2), several novel vaccines of diverse platforms have been successfully developed and administered. Two mRNA vaccines represented a new type of vaccine that comprised of synthetic mRNA molecules containing the code sequence necessary to build the SARS-CoV-2 spike protein. These mRNA vaccines almost single handedly carried the brunt of the US COVID-19 immunization strategy during the past three years. The known and potential benefits of COVID-19 vaccination outweigh the risks and adverse complications. The ongoing COVID-19 pandemic has stimulated unprecedented research on aspects of the vaccines’ ability to reduce the risk of severe infection and death. Likewise, basic immunological studies are pivotal to unraveling the potential and long-term effects of the vaccines as well as to be able to make adjustments to new vaccine development. As the circulating virus strain continues to evolve, updated vaccines will be critical to protecting the population, particularly the elderly and immune compromised.
Subject(s)
COVID-19 , Vaccination , Post-Acute COVID-19 Syndrome , MyocarditisABSTRACT
A case of IgG4-related hypertrophic pachymeningitis was reported. The patient was an elderly female, with the course of disease more than 8 years. Clinical manifestations included recurrent headache, vision and hearing loss, exophthalmos and thyroid dysfunction. Finally, she was diagnosed as IgG4-related disease and IgG4-related hypertrophic pachymeningitis by PET-CT and dural biopsy. After treatment with methylprednisolone and mycophenolate mofetil, the patient′s clinical symptoms improved.
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IgG4-related sclerosing mesenteritis is a rare disease of mesentery of an unknown etiology which shows a constellation of histopathologic findings of lymphoplasmacytic inflammation with IgG4-positive plasma cells and marked fibrosis. This chronic inflammatory condition of mesentery forming an abdominal cocoon has never been described before to the best of our knowledge. Here, we report a patient with a history of subacute small bowel obstruction who was found to have an intra-abdominal encapsulating mass in the right iliac fossa and was finally diagnosed as IgG4-related sclerosing encapsulating peritonitis (abdominal cocoon) based on peroperative findings, histology and immunohistochemistry.
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The case is presented on a 52-year-old male patient, who was seen in the Rheumatology department. He had painless lymph nodes in the cervical, axillary, supraclavicular, and neck region. He also had a fever, and parotid and submaxillary gland enlargement. Complementary studies were performed, showing normocytic-normochromic anemia, thrombocytopenia and eosinophilia, impaired renal function with hypoalbuminaemia and hematuria, ANA 1/5120, Sm+, ACL+. Biopsies were also performed on the compromised tissues, reaching the diagnosis of Rosai-Dorfman Disease and IgG4-related Disease. Differential diagnoses of cervical, axillary and inguinal lymph nodes, with fever, renal and hematological compromise are discussed.
Se describe el caso de un paciente varón de 52 años que consulta al servicio de reumatología por presentar adenopatías indoloras en las regiones cervical, axilar, supraclaviculares y en la nuca, así como fiebre, aumento de tamaño de parótidas y submaxilares. Se realizan estudios complementarios que arrojan como resultado anemia normocítica-normocrómica, trombocitopenia y eosinofilia, alteración de la función renal con hipoalbuminemia y hematuria, FAN 1/5.120, Sm+, ACL+ y biopsia de los tejidos comprometidos, por lo que se arriba al diagnóstico de enfermedad de Rosai-Dorfman y enfermedad relacionada con IgG4. Se discuten diagnósticos diferenciales de adenopatías cervicales, axilares e inguinales, fiebre, compromiso renal y hematológico.
Subject(s)
Humans , Male , Middle Aged , Hemic and Lymphatic Diseases , Autoimmune Diseases , Histiocytosis , Histiocytosis, Sinus , Immunoglobulin G4-Related Disease , Immune System Diseases , Lymphatic DiseasesABSTRACT
Introducción: la enfermedad relacionada con IgG4 (ER-IgG4) es una afección inmunomediada, asociada con lesiones fibroinflamatorias que pueden ocurrir en casi cualquier órgano. Ante su sospecha clínica es fundamental realizar el examen histopatológico para excluir malignidad y otras enfermedades. El objetivo principal de este estudio fue describir los hallazgos histológicos de la biopsia de glándulas salivales labiales (GSL) en pacientes con diagnóstico de ER-IgG4 y, posteriormente, comparar estos hallazgos con la histología e inmunohistoquímica de la glándula salival mayor (GSM). Materiales y métodos: estudio retrospectivo, observacional y descriptivo sobre una cohorte de 40 pacientes con diagnóstico de ER-IgG4. Se incluyeron 26 pacientes con diagnóstico de ER-IgG4 que presentaron biopsias de GSL. Resultados: el 73% de la muestra tuvo un infiltrado linfoplasmocitario y el 11%, fibrosis con patrón no estoriforme. En ningún caso se halló arteritis obliterativa ni ninguna forma de flebitis. Al realizar la comparación con los hallazgos de las biopsias de GSM, no hubo significancia estadística. El 65% de los pacientes manifestó compromiso extraglandular y fueron los sistemas pancreato hepato biliar y las adenopatías los más afectados. Conclusiones: la biopsia incisional de glándula submandibular sería más útil y apropiada que la biopsia de GSL para un diagnóstico definitivo de ER-IgG4.
Introduction: IgG4-related disease (IgG4-RD) is an immune-mediated condition associated with fibroinflammatory lesions that can occur in almost any anatomical site. Histopathological examination is essential when suspected to diagnose it to exclude malignancy and other diseases. The objective of this study was to describe the histological findings of the minor salivary gland biopsy (GSL) in patients diagnosed with IgG4-RD, and subsequently to correlate these findings with the histology and immunohistochemistry of the major salivary gland (GSM). Materials and methods: retrospective, observational and descriptive study on a cohort of 40 patients diagnosed with RD-IgG4. Twenty-six patients diagnosed with IgG4-RD who presented SLG biopsies were included. Results: the 73% of the samples presented a lymphoplasmacytic infiltrate and 11% of them fibrosis with a non-storiform pattern. In no case was obliterative arteritis or any form of phlebitis found. When comparing the findings of the GSM biopsies, there was no statistical significance. Sixty-five percent of the patients presented extraglandular involvement; the pancreato-hepato-biliary system and the adenopathies were the most affected. Conclusions: incisional biopsy of the submandibular gland would be more useful and appropriate than LSG for a definitive diagnosis of IgG4-RD.
Subject(s)
Immunoglobulin G , Salivary Glands , BiopsyABSTRACT
Las enfermedades relacionadas con IgG4 (ER-IgG4) son entidades fibroinflamatorias e inmunomediadas, caracterizadas por la afección multiorgánica, con la formación de pseudotumores que provocan lesión tisular y daño orgánico subsecuente. Se describe el caso de un paciente de 43 años que presentó sialoadenitis esclerosante y cumplió todos los criterios diagnósticos de enfermedad relacionada con IgG4.
IgG4-related diseases (IgG4-RD) are fibroinflammatory immune-mediated entities characterized by multiorgan involvement with the development of pseudotumors that cause tissue injury and subsequent organ damage. We describe the case of a 43-year-old man who presented sclerosing sialadenitis and fulfilled the diagnostic criteria for IgG4-related disease.
Subject(s)
Immunoglobulin G4-Related Disease , SialadenitisABSTRACT
Abstract Some patients diagnosed with idiopathic retroperitoneal fibrosis could be reclassified as IgG4-related disease (IgG4-RD). Classification criteria have not been uniform and prevalence of IgG4-related retroperitoneal fibrosis (IgG4-RPF) is unknown in our region. We aimed to describe IgG4-RPF frequency relying on criteria published recently and comparing clinical, histopathologic and radiologic features with non-IgG4-RPF. From January, 2005 to December, 2020, nineteen adults with histopathologic diagnosis of idiopathic retroperitoneal fibrosis were included in a dynamic retrospective cohort at Hospital Italiano de Buenos Aires. Pathology slides were reviewed and immunohistochemistry was performed and assessed for each case. We used classification criteria described in 2019 American College of Rheumatology/European League Against Rheumatism to identify IgG4-RD cases. Ten of 19 patients met criteria for IgG4-RPF. Median age was similar in two subsets (61 versus 55, p = 0.2) and both had male predominance. Three out of 10 patients (p = 0.2) featured other manifestations of IgG4-RD in the IgG4-RPF group, and periaortic fibrosis was the most significant finding in images (p = 0.01). Corticosteroids were mostly used as therapy, followed by azathioprine and rituximab. Most patients did not receive specific treatment. IgG4-RPF patients had dense lymphocytic infiltrate and 8 out of 10 showed storiform fibrosis (p = 0.02). IgG4+ cells/hpf and IgG4/IgG ratio were significantly higher (p = 0.01). Over half of the patients in our cohort met the criteria of IgG4-RPF. New criteria may harmonize the identification of IgG4-RD. As IgG4-RD may be reversible at initial stages, these findings may lead to early recognition, treatment and integral follow-up.
Resumen Muchos pacientes con diagnóstico de fibrosis retroperitoneal idiopática (FRI) pueden ser reclasificados como enfermedad relacionada con IgG4 (ER- IgG4). Los criterios diagnósticos no han sido uniformes y la frecuencia de fibrosis retroperitoneal relacionada con IgG4 en nuestra región es desconocida. El objetivo fue describir la frecuencia de ER-IgG4 en pacientes clasificados como FRI y comparar características clínicas, histopatológicas y de laboratorio con aquellos que no reunían criterios de la enfermedad. Se incluyeron 19 adultos en un estudio de cohorte retrospectiva dinámica con diagnóstico anatomopatológico de FRI, en el Hospital Italiano de Buenos Aires, desde enero de 2005 hasta diciembre de 2020. Se revisaron las biopsias y se realizó inmun ohistoquímica en cada una. Se consideró caso al paciente que reunía los criterios de la American College of Rheumatology/European League Against Rheumatism 2019. Diez pacientes reunieron criterios de ER-IgG4. La mediana de edad fue similar en ambos grupos (61 vs. 55, p = 0.2) y en ambos hubo predominio masculino. Tres de 10 pacientes (p = 0.2) tuvieron otras manifestaciones de ER-IgG4 y la fibrosis periaórtica fue el hallazgo más significativo en los estudios por imágenes (p = 0.01). Los corticoides fueron las drogas más utilizadas seguidos por azatioprina y rituximab, pero la mayoría no reci bió tratamiento específico. Todos los pacientes con fibrosis retroperitoneal relacionada con IgG4 presentaron infiltrado linfocitario denso y 8/10 fibrosis estoriforme (p = 0.01), así como las células IgG4+/hpf y ratio IgG4/ IgG fueron significativamente mayores (p = 0.01). Más de la mitad de los pacientes con FRI cumplieron criterios de ER-IgG4. Los nuevos criterios diagnósticos podrían contribuir a homogeneizar la identificación de ER-IgG4. Dado que esta enfermedad puede ser reversible en estadios tempranos, estos resultados promueven aumentar el conocimiento de la entidad para tratamiento precoz y seguimiento integral.
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Objective:By retrospectively analyzing 6 cases of IgG4-related membranous nephropathy (IgG4-MN), combined with literature review, to explore the clinical and renal pathological characteristics of the disease, and improve clinicians' understanding of the disease.Methods:The data of six patients with biopsy-proven IgG4-MN in the nephrology center of our hospital during April 2017 to January 2021 were collected. At the same time, we reviewed the literature systematically and summarized the clinicopathological characteristics.Results:Six male patients with the age ranged fom 55 to 75 years old were described. Urine protein level was (3.1±2.1) g/24 h, 3 cases (50%) showed nephrotic syndrome and 4 cases (67%) had elevated serum creatinine. The median creatinine level was (103±24) μmol/L. Six cases (100%) had elevated serum immunoglobulin (Ig)E level, and 4 cases (67%) had elevated IgG4. M-type phospholipase A2 receptor (PLA2R) was positive in 1 case (17%) and tubulointerstitial nephritis (TIN) was present in 6 cases. The review of the literature suggested that a total of 49 cases with IgG4-MN were reported, including 6 cases in this report. There were 40 males (40/46, 87%), with a age range of (61±12) years old, 32 cases (32/49, 65%) showed nephrotic syndrome range proteinuria, and the proportion of serum IgG and IgG4 increase was 61%(20/33) and 88% (36/41), respectively, 13 cases (13/15, 87%) had elevated serum IgE level, 47% (14/30) had low-complement C3 and 44%(12/27) had low-complement C4 level. The main organs involved were pancreas (15/37) and lymph nodes (16/37). Renal pathology showed TIN in 74%(36/49). Electron dense deposition was mainly subepithelial deposits. 7%(2/28) were positive for anti-PLA2R antibody in serum, 17%(3/18) were positive for PLA2R in kidney tissue, 6%(1/18) were suspected positive for PLA2R in kidney tissue, and 8%(1/12) were dual positive in blood and kidney tissue.Conclusion:IgG4-MN usually presents with nephrotic range proteinuria or nephrotic syndrome in middle-aged and elderly patients. Most of them are complicated with TIN and other organ involvement. A certain proportion of patients are PLA2R positive in IgG4-MN, and whether it is primary or secondary MN needs further study.
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Objective To evaluate the diagnostic efficiency of four anti-cysticercus IgG, IgG4 or IgM antibody test kits (enzyme-linked immunosorbent assay, ELISA) by different manufacturers, so as to provide insights into the epidemiological investigation and clinical detection of cysticercosis. Methods Forty serum samples from cerebral cysticercosis patients, 100 serum samples from healthy volunteers, 30 serum samples from paragonimiasis skrjabini patients, 17 serum samples from cystic echinococcosis and 19 serum samples from subcutaneous or cerebral sparganosis patients were collected and detected using anti-cysticercus IgG, IgG4 or IgM antibody test kits (brand A) and the anti-cysticercus IgG antibody test kit (brand B). The sensitivity, specificity and false negative rate of the four kits for detection of cysticercosis were estimated. Results The anti-cysticercus IgG, IgG4 or IgM antibody test kits (brand A) showed 95.00% (38/40), 87.50% (35/40), 7.50% (3/40) sensitivities and 98.00% (98/100), 100.00% (100/100) and 100.00% (100/100) for detection of cysticercosis, while the anti-cysticercus IgG antibody test kit (brand B) presented a 75.00% (30/40) sensitivity and 100.00% (100/100) specificity for detection of cysticercosis. The sensitivity for detection of cysticercosis was significantly higher by the anti-cysticercus IgG antibody test kit (brand A) than by the anti-cysticercus IgG antibody test kit (brand B) (χ2 = 6.28, P < 0.05); however, no significant difference was seen in the specificity by two kits (χ2 = 2.01, P > 0.05). The four ELISA kits showed overall false positive rates of 37.88% (25/66), 22.73% (15/66), 62.12% (41/66) and 15.15% (10/66) for detection of paragonimiasis, echinococcosis and sparganosis (χ2 = 37.61, P < 0.05), and the anti-cysticercus IgG antibody test kit (brand A) presented the highest overall false positive rate for detection of paragonimiasis, echinococcosis and sparganosis (χ2 = 7.56, P’ < 0.008), while a higher overall false positive rate was seen for detection of paragonimiasis, echinococcosis and sparganosis by the anti-cysticercus IgG antibody test kit (brand A) than by the anti-cysticercus IgG antibody test kit (brand B) (χ2 = 8.75, P’ < 0.008). The four ELISA kits showed false positive rates of 40.00% (12/30), 16.67% (5/30), 76.67% (23/30) and 13.33% (4/30) for detection of paragonimiasis (χ2 = 32.88, P < 0.05) and 21.05% (4/19), 26.32% (5/19), 73.68% (14/19) and 15.79% (3/19) for detection of sparganosis (χ2 = 19.97, P < 0.05), and the highest false positive rates were found by the anti-cysticercus IgM antibody test kit (brand A) for detection of paragonimiasis and sparganosis (all P’ < 0.008). However, the four ELISA kits showed comparable false positive rates of 52.94% (9/17), 29.41% (5/17), 23.53% (4/17) and 17.65% (3/17) for detection of echinococcosis (χ2 = 8.24, P > 0.05). In addition, the anti-cysticercus IgM anti-body test kit (brand A) showed false positive rates of 76.67% (23/30), 23.53% (4/17) and 73.68% (14/19) for detection of paragonimiasis, echinococcosis and sparganosis (χ2 = 14.537, P < 0.05), with the lowest false positive rate seen for detection of echinococcosis (χ2 = 14.537, P’ < 0.014), while no significant differences were seen in the false positive rate for detection of paragonimiasis, echinococcosis and sparganosis by other three ELISA kits (all P > 0.05). Conclusions The four anti-cysticercus IgG, IgG4 or IgM antibody test kits exhibit various efficiencies for serodiagnosis of cysticercosis. The anti-cysticercus IgG antibody test kit (brand A) has a high sensitivity for serodiagnosis of cysticercosis; however, it still needs to solve the problems of cross-reaction with other parasitic diseases and stability.
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Objective@# To summarize the clinical manifestations of IgG4-related diseases in the head and neck, explore treatment methods.@*Methods @#The clinical data of 21 patients diagnosed with IgG4-related diseases were retrospectively analyzed. The clinical data and the results of glucocorticoid and immunosuppressive therapy were studied retrospectively.@* Results@#All patients had swollen sclerotic masses, and CT showed irregular high-density masses with uniform enhancement in the enlarged glands. Some patients had mucosal thickening and mass-like changes in theoral cavity, nose, sinuses, throat and other tissues, and most of the patients had cervical lymphadenopathy and elevated serum IgG4 levels (≥ 1.35 g/L). Histopathological examination of affected exosine glands and affected mucosa and lymph nodes in all patients showed infiltration of lymphocytes, plasma cells and IgG4+ plasma cells. In 21 patients, the mass in the affected glands and mucosa (including head, neck and other tissues) disappeared, and the clinical symptoms were relieved after the application of glucocorticoids. However, with a reduction in glucocorticoids, the mass recurred or even worsened.@*Conclusion @#For patients with a single mass in the submandibular gland, parotid gland and other salivary glands, as well as lymph node enlargement, CT is the first choice to identify the nature of gland neoplasms. Combined with pathological examination, related auxiliary examination and peripheral blood examination are also needed to obtain a definitive diagnosis. Glucocorticoid therapy is used to achieve a good prognosis, and long-term follow-up and timely adjustment of medication regimens are required.
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In 2015, the first Chinese consensus on the diagnosis and management of primary sclerosing cholangitis was issued. In the past years, more data have emerged from the literature. Herein, the Autoimmune Liver Disease Group of the Chinese Society of Hepatology organized an expert group to review the evidence and updated the recommendations to formulate the guidelines. There are 21 recommendations on PSC clinical practice. To facilitate the differentiation between PSC and IgG4-related sclerosing cholangitis, 10 recommendations on IgG4-SC are also attached. These guidelines aim to provide a working reference for the management of PSC and IgG4-SC.