Positive association of serum IgG4 levels with modified marsh classification system in children with gluten enteropathy: a single centre experience

Background: Celiac disease or gluten enteropathy is an immune-mediated systemic disorder elicited by gluten and related prolamines in genetically susceptible individuals and is characterized by the presence of gluten enteropathy, celiac disease-specific antibodies, HLA-DQ2/DQ8 haplotypes. IgG4-related diseases is an increasingly recognized immune-mediated condition in autoimmune disorders such as primary sclerosing cholangitis, autoimmune hepatitis, and autoimmune thyroiditis, characterized by tissue fibrosclerosis and infiltration by IgG4-positive plasma cells and increased serum IgG4 concentrations.Methods: A total of 33 children with newly diagnosed celiac disease and 31 control subjects were included in this study. All suspected celiac disease children underwent duodenal biopsy and were diagnosed based on Marsh grading. Serum IgG4 level estimations were performed using an enzyme-linked immune sorbet assay method with a cut-off of 135 mg/dl for diagnosis.Results: A significant positive association between serum IgG4 levels and Marsh classification was found, the higher the levels of IgG4 in serum, the higher the grade on Marsh staging. Mean serum IgG4 levels in Marsh 3a was 126.32 mg/dl, in 3b was 171.35 mg/dl and in 3c was 209.24 mg/dl (p value=0.004)Conclusions: With increasing serum IgG4 levels, increased severity of damage was seen on biopsy specimens based on higher Marsh grade. To the best of found knowledge, this is the first study to establish the relation between IgG4 and mucosal damage in children with celiac disease.

Chinese guideline for diagnosis and treatment of autoimmune pancreatitis （Shanghai， 2023） / 临床肝胆病杂志

Autoimmune pancreatitis （AIP） is an immune⁃mediated， special type of chronic pancreatitis， which can involve multiple organs. The clinical manifestation of AIP is complex and varied， making the diagnosis and treatment challenging. With reference to the latest guidelines and studies from both domestic and international sources， this guideline comprises 20 recommendations regarding the diagnosis， treatment， follow⁃up， and prognosis of AIP. The aim of this guideline is to promote the care capability and improve the outcome of patients with AIP in China.

Clinical features and lymphocyte subtypes in patients with IgG 4-related diseases / 中华内科杂志

Objective:To deepen understanding of IgG 4-related diseases (RDs), we analyzed the associated lymphocyte subtypes, and explored the pathogenesis and potential immunotherapeutic targets. Methods:Eighty-six patients with IgG 4-RDs were enrolled, and their clinical characteristics, peripheral lymphocyte subtypes, and disease course were analyzed. Results:The mean age of the participants was 36-87(62±11) years; 51 were male (59.3%) and 35 were women (40.7%); and 34.9% had a history of allergy. Follow-up lasted 4.8 (0.4, 14.1) months. The most common symptoms were abdominal pain, and submandibular gland and lacrimal gland swelling (each 20.9%). Sixty-five (75.6%) participants had multiple organ involvement, and the most frequently affected organs were the pancreas (52.3%), submandibular gland (51.2%), and lacrimal gland (34.9%). A high eosinophil count; high IgE, IgG, IgG 1, and IgG 4 concentrations; and low complement C3 and C4 concentrations were present in 18.8% (16/85), 30.0% (24/80), 72.9% (62/85), 58.3% (28/48), 89.5% (77/86), 61.2% (52/85), and 50.0% (42/84), respectively, of the participants. In addition, 64.7% (55/85) were positive for autoantibodies, and the most frequent was anti-nuclear antibody (63.5%). The proportion of CD4 +T lymphocytes increased in 25.7% (9/35) of the participants, which was accompanied by an increase in the ratio of CD4 +/CD8 +T lymphocytes (22.9%, 8/35). Importantly, most participants (90.0%, 18/20) had a high proportion of regulatory T (Treg) cells. High interleukin (IL)-2, IL-6, and IL-10 concentrations were present in 50.0% (11/22), 33.3% (10/30), and 16.7% (5/30), respectively, of the participants. Substantial lymphoplasmacytic infiltration, fibrosis, IgG 4-positive plasma cell infiltration, and lymphoid follicle hyperplasia or ectopic formation were present in 79.2% (42/53), 67.9%(36/53), 35.8%(19/53) and 30.2% (16/53), respectively, of the participants. Fifty-three participants with detailed pathologic data were also further evaluated, of whom 24.5% (13/53), 3.8% (2/53), and 67.9% (36/53) had definite, probable, and possible diagnoses; and 3.8% (2/53) could not be diagnosed. Compared with baseline, the percentage of eosinophils and the IgE, IgG, and IgG 4 concentrations decreased significantly; and the complement C3 and C4 concentrations had increased significantly after 6 months of treatment (all P<0.05). The IgG 4 concentration after 6 months of treatment negatively correlated with that of C4, and positively correlated with the baseline concentration of IgE and the IgG 4/IgG ratio. Conclusion:IgG 4-RDs are a group of diseases characterized by male predisposition; multiple organ involvement; a high eosinophil count; high IgE, IgG, IgG 1, and IgG 4 concentrations; and a low C3 concentration. Peripheral CD4 +T cells and Treg cells are also more abundant. The diseases can be controlled with glucocorticoids and immunosuppressive drugs in the majority of instances. The IgG 4 concentration after 6 months of treatment negatively correlates with the baseline complement C4 concentration and positively correlates with the IgE concentration and IgG 4/IgG ratio, which suggests that IgG 4/IgG, IgE, and complement should be closely monitored to evaluate disease activity and the efficacy of treatment in such patients.

Clinical and endoscopic ultrasound features of IgG4-related autoimmune pancreatitis / 中华消化内镜杂志

Objective:To analyze the differences in clinical and endoscopic ultrasonography (EUS) findings between diffuse and focal IgG4-related autoimmune pancreatitis (IgG4-AIP).Methods:Data of patients diagnosed as having IgG4-AIP who underwent EUS at Chinese PLA General Hospital from September 2011 to April 2022 were retrospectively collected. General clinical data, EUS features, and postoperative pathology were analyzed for characteristic differences.Results:A total of 40 patients were included in the study, 60.03±10.87 years old, a higher proportion of males (85.0%, 34/40). All patients underwent EUS, and 28 underwent EUS-guided fine-needle aspiration. Among the 40 patients, 29 (72.5%) had diffuse type and 11 (27.5%) had focal type. Abdominal pain [65.5% (19/29) VS 18.2% (2/11), χ2=5.393, P=0.020] and thickening of the bile duct wall [51.7% (15/29) VS 9.1% (1/11), χ2=4.394, P=0.036] were more common in the diffuse type, while main pancreatic duct dilation [45.5% (5/11) VS 10.3% (3/29), χ2=4.146, P=0.042] was more common in the focal type, with the lesion most commonly located in the pancreatic head (90.9%, 10/11). There was no significant difference in the presence of chronic pancreatitis parenchymal changes between the two groups [34.5% (10/29) VS 27.3% (3/11), χ2=0.003, P=0.955]. Conclusion:There are certain differences in abdominal pain and biliary and pancreatic duct lesions between diffuse and focal AIP. The high expression of chronic pancreatitis characteristics is not observed in either group, which provides clues for the classification of AIP in clinical practice.

Clinicopathologic characteristics of Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma:report of nine cases / 临床与实验病理学杂志

Purpose To explore the clinicopathologic fea-tures of Epstein-Barr virus-positive inflammatory follicular den-dritic cell sarcoma(EBV+IFDCS).Methods The clinico-pathologic features of 9 cases of EBV+IFDCS were retrospective-ly analyzed and followed up.Results The age of 9 patients with EBV+IFDCS ranged from 22 to 78 years(mean 44.7 years).7 cases occurred in the liver and 2 in the spleen.Fi-brinoid degeneration and hyaline degeneration in the vessel walls(6/9),eosinophilic infiltration(3/9),and epithelioid granulo-mas(2/9)were seen in some cases.The tumor cells expressed CD21(7/9),CD23(8/9)and CD35(9/9),partially ex-pressed SMA(6/9)and D2-40(1/9).It was noteworthy that 2 cases from the spleen accompanied by high expression of IgG4 plasma cells(80-135/10 HPF),and in the liver(0-36/10 HPF).All cases were followed up for 3-84 months,with 6 pa-tients disease-free,2 patients underwent metastasis,1 patient lost of follow-up.Conclusion EBV+IFDCS is a rare low-grade malignant tumor.EBER in situ hybridization and immunohisto-chemical detection play important roles in the diagnosis and dif-ferential diagnosis of EBV+IFDCS.Surgical resection is the main therapeutic intervention for EBV+IFDCS,and patients re-quire long-term post-surgical follow-up.

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.

Isolated IgG4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma: a case report of a challenging disease

IgG4-related disease (IgG4-RD) has recently attracted attention worldwide; however, its diagnosis still remains challenging. It is an immune-mediated multisystem disease affecting a wide range of organs in the human body. Even though it's uncommon, IgG4-sclerosing cholangitis, which is a biliary manifestation of IgG4-RD, can happen without pancreatic involvement. Here, we report a case of a patient with imaging features typical of hilar cholangiocarcinoma, which eventually turned out to be IgG4 sclerosing cholangitis.

Hypertrophic pachymeningitis: a rare manifestation of IgG4 related disease

Hypertrophic pachymeningitis (HP) is a rare form of diffuse inflammatory disease that causes thickening of the dura mater. It can involve the cranial or the spinal dura or both. An increasingly well-known symptom of IgG4-related illness, a fibroinflammatory syndrome that may affect almost any organ, is IgG4-related hypertrophic pachymeningitis (IgG4-RHP). It is estimated that IgG4-RHP may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic. Contrast magnetic resonance imaging (MRI) shows pachymeningeal enhancement. Serum IgG4 levels may be elevated but are normal in most patients. However, most patients have elevated cerebrospinal fluid (CSF) IgG4 index. Hence, CSF IgG4 index could serve as a less invasive diagnostic marker of IgG4-RHP. Confirmation of diagnosis is by meningeal biopsy that shows swirling “storiform” fibrosis with lymphocytic infiltrates, obliterate phlebitis and IgG4 positive plasma cells. This case highlights the diagnostic dilemma of IgG4-RHP as gold standard of diagnosis is meningeal biopsy which has many of its own limitations. CSF IgG4 index could be an alternate option for meningeal biopsy when the procedure is contraindicated or uninformative.

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.

IgG4-related disease with renal pelvic involvement suspiciously diagnosed as renal pelvis cancer: a case report / 中华泌尿外科杂志

Here we report a case of IgG4-related disease (IgG4-RD) involving the renal pelvis. A left renal pelvic mass was found on a patient during medical examination. The imaging examinations of CT urography, MRI and PET-CT all suggested renal pelvic cancer with multiple lymph node metastasis. The CT-guided biopsy of left renal pelvis was performed. Combining the pathological results and the serum IgG4 level, the IgG4-RD was finally clinically diagnosed. After oral methylprednisolone treatment, the volume of renal pelvic lesion reduced and the level of IgG4 decreased. IgG4-RD is a newly recognized fibro-inflammatory condition which may affect multiple organs. It is easy to be misdiagnosed as renal pelvis cancer when the renal pelvis is involved. Combing the tendency of multiple organ involvement, the serological test, the imaging features and the pathological characteristics, the correct diagnosis can be made. This disease responds well to glucocorticoid treatment, but the relapse is common.

Recommendations for the diagnosis and treatment of IgG 4-related disease in China / 中华内科杂志

IgG 4-related disease (IgG 4-RD) is an immune-mediated fibroinflammatory condition characterized by tumefactive lesions in multi-organs. It is a novel entity presented by variable manifestations. In recent years, there has been progress toward recognizing IgG 4-RD. However, the diagnosis and treatment of IgG 4-RD still present challenges due to insufficient experience. To address this, the Chinese Rheumatology Association has developed standardized guidelines for the diagnosis and treatment of IgG 4-RD based on domestic and international experience. These guidelines aim to enhance the understanding and management of IgG 4-RD, ultimately improving the prognosis for patients with IgG 4-RD.

IgG4-related hypertrophic pachymeningitis: a case report / 中华神经科杂志

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.

Medical visit status and clinical features in patients with IgG4 related disease / 北京大学学报(医学版)

OBJECTIVE@#To understand the medical treatment and clinical characteristics of patients with IgG4-related disease (IgG4-RD) with complex clinical manifestations and easy to be misdiagnosed and missed, and to improve the recognition of this disease among doctors from relevant medical departments.@*METHODS@#A retrospective analysis was conducted on the medical records of patients diagnosed with IgG4-RD who were hospitalized and discharged from Peking University Third Hospital from January 1, 2012 to December 31, 2022. The patient' s medical visit status, clinical manifestations, laboratory examinations, diagnosis, and treatment information were summarized.@*RESULTS@#A total of 116 patients diagnosed with IgG4-RD were included in this study, with a male to female ratio of 2. 52∶ 1 and an average age of (61.83±10.80) years. The departments for initial visits were gastroenterology, general surgery, and ophthalmology. While the departments responsible for definitive diagnosis were gastroenterology, rheumatology and immunology, and respiratory medicine. Twenty-one patients (18. 10%) required consultation and treatment from three or more departments before receiving a definitive diagnosis. The median time from symptom onset to the initial clinic visit was 2 (1, 7) months, and the median time from symptom onset to diagnosis was 1 (1, 12) month. Twenty-four patients (20.69%) underwent surgical resection of the affected sites before diagnosis. According to the classification criteria of IgG4-RD, sixty-eight (58.62%) cases were diagnosed definitively, eight (6.9%) cases were likely to be diagnosed, and 40 (34.48%) cases were suspected to be diagnosed. In the 68 definitively diagnosed patients, the most commonly affected organs were submandibular gland, the pancreas, biliary tract, parotid in sequence. The median serum IgG4 (IgG4, immunoglobulin G4) level was 6.16 (3. 61, 12. 30) g/L. Fifty-seven patients (83.82%) were treated with glucocorticoids, and 14 patients (20.59%) were treated with immunosuppressants. The use of immunosuppressants was mainly in the rheumatology and immunology department (78. 57%).@*CONCLUSION@#IgG4-RD is more common in elderly males, with submandibular gland, the pancreas, biliary tract, and parotid being most commonly affected. The distribution of initial visit departments in patients is wide. The proportion of definitive diagnosis based on pathology is relatively low. In terms of treatment, the main approach is steroid treatment, while the use of immunosuppres-sants is not widespread.

A case of duodenal ulcer as prominent manifestation of IgG4-related disease / 北京大学学报(医学版)

A case of IgG4-related disease presented with a duodenal ulcer to improve the understan-ding of IgG4-related diseases was reported. A 70-year-old male presented with cutaneous pruritus and abdominal pain for four years and blackened stools for two months. Four years ago, the patient went to hospital for cutaneous pruritus and abdominal pain. Serum IgG4 was 3.09 g/L (reference value 0-1.35 g/L), alanine aminotransferase 554 U/L (reference value 9-40 U/L), aspartate aminotransferase 288 U/L (reference value 5-40 U/L), total bilirubin 54.16 μmol/L (reference value 2-21 μmol/L), and direct bilirubin 29.64 μmol/L (reference value 1.7-8.1 μmol/L) were all elevated. The abdominal CT scan and magnetic resonance cholangiopancreatography indicated pancreatic swelling, common bile duct stenosis, and secondary obstructive dilation of the biliary system. The patient was diagnosed with IgG4-related disease and treated with prednisone at 40 mg daily. As jaundice and abdominal pain improved, prednisone was gradually reduced to medication discontinuation. Two months ago, the patient developed melena, whose blood routine test showed severe anemia, and gastrointestinal bleeding was diagnosed. The patient came to the emergency department of Beijing Hospital with no improvement after treatment in other hospitals. Gastroscopy revealed a 1.5 cm firm duodenal bulb ulcer. After treatment with omeprazole, the fecal occult blood was still positive. The PET-CT examination was performed, and it revealed no abnormality in the metabolic activity of the duodenal wall, and no neoplastic lesions were found. IgG4-related disease was considered, and the patient was admitted to the Department of Rheumatology and Immunology of Beijing Hospital for further diagnosis and treatment. The patient had a right submandibular gland mass resection history and diabetes mellitus. After the patient was admitted to the hospital, the blood test was reevaluated. The serum IgG4 was elevated at 5.44 g/L (reference value 0.03-2.01 g/L). Enhanced CT of the abdomen showed that the pancreas was mild swelling and was abnormally strengthened, with intrahepatic and extrahepatic bile duct dilation and soft tissue around the superior mesenteric vessels. We pathologically reevaluated and stained biopsy specimens of duodenal bulbs for IgG and IgG4. Immunohistochemical staining revealed remarkable infiltration of IgG4-positive plasma cells into duodenal tissue, the number of IgG4-positive cells was 20-30 cells per high-powered field, and the ratio of IgG4/IgG-positive plasma cells was more than 40%. The patient was treated with intravenous methylprednisolone at 40 mg daily dosage and cyclophosphamide, and then the duodenal ulcer was healed. IgG4 related disease is an immune-medicated rare disease characterized by chronic inflammation and fibrosis. It is a systemic disease that affects nearly every anatomic site of the body, usually involving multiple organs and diverse clinical manifestations. The digestive system manifestations of IgG4-related disease are mostly acute pancreatitis and cholangitis and rarely manifest as gastrointestinal ulcers. This case confirms that IgG4-related disease can present as a duodenal ulcer and is one of the rare causes of duodenal ulcers.

Isolated external iliac artery aneurysm: a rare case presentation of IgG4-related disease / Aneurisma isolado de artéria ilíaca externa - uma apresentação de caso raro de doença relacionada à IgG4

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.

IgG4 related sclerosing encapsulating peritonitis with cocoon formation: An unusual and undescribed presentation

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.

Hallazgos histológicos de glándulas salivales mayores y menores en pacientes con enfermedad relacionada a IgG4 / Histological findings of major and minor salivary glands in patients with IgG4 related disease

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.

Sialoadenitis esclerosante asociada a enfermedad por IgG4: reporte de un caso / Sclerosing sialadenitis associated with IgG4 disease: case report

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.

Clinical analysis of 21 cases of IgG4-related diseases / 口腔疾病防治

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.

Studies on newly recognized chronic sialadenitis / 北京大学学报(医学版)

Some kinds of chronic sialadenitis were recognized during the recent years. They have specific pathogenesis, clinical and histopathologic appearances, and require specific treatment. IgG4-related sialadenitis (IgG4-RS) is one of the immune-mediated diseases, characterized by tumefactive lesions. The incidence of IgG4-RS obviously increased during the past 30 years. The study on the potential relationship between occupational exposure to chemical substances and the incidence of IgG4-RS showed that subjects with occupational exposure to agents known to cause IgG4-RD had an increased risk for IgG4-RS. Surgical excision of involved SMG could not control the disease progression, which is not recommended for treatment of IgG4-RS. The combination of glucocorticoid and steroid-sparing agents is effective for treating IgG4-RS, and restores salivary gland function. Radioiodine induced sialadenitis (RAIS) is one of the common complications of postoperative adjuvant treatment of differentiated thyroid cancer by 131I. The incidence of the disease is related to radiation dosage. Clinically, the patients suffered from swelling and tenderness in the buccal or submandibular regions, especially during the mealtime. Imaging appearances are similar to those of chronic obstructive sialadenitis. Conservative managements, such as gland massage, sialagogues, are the mainstream methods in the treatment of RAIS. Sialendoscopy is feasible for RAIS, but not as effective as conventional obstructive sialadenitis (COS). Therefore the prevention of RAIS is crucial. Eosinophilic sialodochitis (ES) is a new type of chronic inflammatory disease of the salivary gland related to allergy. It has characteristics of swelling of multiple major salivary glands, strip-like gelatinous plugs discharged from the duct orifice of the gland, elevated level of serum IgE and eosinophils in peripheral blood, infiltration of eosinophils and IgE positive plasma cells in the tissues, allergic history, increased expression of allergy-related cytokines, such as IL-4, IL-5, IL-13, and eotaxin, which suggest allergic reactions as a potential pathogenesis of the disease. The clinical, laboratory, histological, and immunohistochemical characteristics of ES are significantly different from conventional obstructive sialadenitis (COS). Therefore, it is suitable to separate ES from COS. Conservative managements, such as self-maintenance therapy and anti- allergic modality are the choices of treatment for ES. Based on the results of our comprehensive studies a new classification of chronic sialadenitis is suggested.