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IgG4-related hepatobiliary and pancreatic diseases are a part of the IgG4-related disease multiorgan fibroinflammatory disorder, including IgG4-related autoimmune pancreatitis, IgG4-related sclerosing cholangitis, and IgG4-related hepatic involvement. The main pathological features include IgG4 + plasma cell/lymphocyte infiltration, storiform fibrosis, obliterative phlebitis, and eosinophil infiltration. The diagnosis of this disease is often based on the comprehensive diagnostic criteria for IgG4-related diseases and organ-specific diagnostic criteria. However, it is difficult to differentiate IgG4-related hepatobiliary and pancreatic diseases from neoplastic diseases, and novel diagnostic biomarkers are expected to improve the sensitivity and specificity of diagnosis. To date, glucocorticoids remain the first-line drug for this disease, and biological agents, especially anti-CD20 monoclonal antibody, may be an alternative therapy for patients with corticosteroid contraindication/intolerance or recurrent/refractory disease.
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IgG4-related diseases have a low incidence and are easily misdiagnosed as tumors in clinical treatments. A 26-year-old male patient was admitted to the hospital because of a left adrenal tumor found in health examination for more than 5 months. The tumor in the left adrenal region could be seen from abdominal CT, and the retroperitoneal laparoscopic resection of the left adrenal tumor was performed. Postoperative pathology was consistent with IgG4-related diseases, and serum IgG4 was abnormally high. After 2 months’ follow-up, serum IgG4 returned to normal, and no special discomfort.
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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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Mucor infection is rarely reported in non-immunocompromised population, especially in isolated gastrointestinal tracts. IgG4-related diseases (IgG4-RD) have been recognized in recent years, but secondary causes of IgG4 elevation should be differentiated. We reported a young man with duodenal mass and ulcer and high serum IgG4 level. Histological biopsy of the mass revealed positive mucor mycelium and infiltration of IgG4 positive plasma cells. Serum IgG4 decreased to normal range after surgical resection and systemic antifungal treatment. This case suggests that isolated mucor mycosis infection can develop in the digestive tract and mimics as IgG4-related disease.
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Objective@#To investigate the clinical significance and pathological features of lymphocytes and plasma cells infiltration and related ectopic lymphoid-like structures in IgG4-related tubulointerstitial nephritis (IgG4-TIN).@*Methods@#Complete data was collected from 24 patients with IgG4-TIN confirmed by pathology in the Peking University First Hospital. The renal specimens were examined by routine light microscopy, immunofluorescence and electron microscopy examination. In addition, immunohistochemistry was used to detect the distribution of CD20+ B lymphocytes, CD3+ T lymphocytes and CD138+ plasma cells.@*Results@#A total of 24 patients were enrolled in the study, including 21 males (87.5%), 3 females (12.5%). The age was (58.0±10.8) years (38-75 years). Pathology analysis showed ectopic lymphoid-like structures were located in 16 (66.7%) cases and Russell bodies were detected in infiltrative plasma cells of 19(79.2%) cases with IgG4-TIN. Compared with cases without Russell body formation, cases with Russell body formation in renal interstitial plasma cells were more prone to show ectopic germinal center-like structure formation (P=0.001), tubular basement membrane (TBM) electron dense deposits (P=0.040) and reduced blood C3 levels (P=0.028).@*Conclusions@#Abnormal tubulointerstitial infiltration of ectopic lymphoid-like structures and plasma cells with prominent Russell body exist in IgG4-TIN patients, which suggests the persistent activation of lymphocytes and plasma cells in renal interstitium may contribute to the pathogenesis of IgG4-TIN.
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Objective To investigate the clinical significance and pathological features of lymphocytes and plasma cells infiltration and related ectopic lymphoid-like structures in IgG4-related tubulointerstitial nephritis (IgG4-TIN). Methods Complete data was collected from 24 patients with IgG4-TIN confirmed by pathology in the Peking University First Hospital. The renal specimens were examined by routine light microscopy, immunofluorescence and electron microscopy examination. In addition, immunohistochemistry was used to detect the distribution of CD20+ B lymphocytes, CD3+ T lymphocytes and CD138+ plasma cells. Results A total of 24 patients were enrolled in the study, including 21 males (87.5％), 3 females (12.5％). The age was (58.0 ± 10.8) years (38-75 years). Pathology analysis showed ectopic lymphoid-like structures were located in 16 (66.7％) cases and Russell bodies were detected in infiltrative plasma cells of 19(79.2％) cases with IgG4-TIN. Compared with cases without Russell body formation, cases with Russell body formation in renal interstitial plasma cells were more prone to show ectopic germinal center-like structure formation (P=0.001), tubular basement membrane (TBM) electron dense deposits (P=0.040) and reduced blood C3 levels (P=0.028). Conclusions Abnormal tubulointerstitial infiltration of ectopic lymphoid-like structures and plasma cells with prominent Russell body exist in IgG4-TIN patients, which suggests the persistent activation of lymphocytes and plasma cells in renal interstitium may contribute to the pathogenesis of IgG4-TIN.
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Objective To analyze the clinicopathological features and prognosis of immunoglobulin G (IgG)4-related diseases with interstitial nephritis. Methods Forty cases of IgG4-related diseases diagnosed by pathology in our hospital from 2014 to 2018 were collected and their clinicopathological features were analyzed. Four patients with IgG4-related disease with interstitial nephritis were analyzed, including clinical laboratory tests and histopathological features, and immunohistochemical analysis of the type and proportion of renal interstitial infiltrating cells. At the same time, the treatment and prognosis of the patients were analyzed. Results Among 40 cases of IgG4-related diseases, 11 cases had parotid submandibular gland involvement (accounted for 28％), labial gland involvement (7 cases, 18％) and lymph node enlargement (6 cases, 15％). Patients with kidney involvement (4 cases, 10％) all presented with IgG4-related tubulo-interstitial nephritis (IgG4-TIN). All of them were elderly males, and 2 had glomerulopathy. One of them had anti-neutrophil cytoplasmic antibodies (ANCA)-related vasculitis renal damage. The number of CD4-positive cells in renal interstitium was more than CD8-positive cells. Another case complicated with IgA nephropathy. Renal dysfunction occurred in all 4 cases, and serum IgG4 level ranged from 4.65 g/L to 23.8 g/L. All 4 patients received glucocorticosteroid and symptomatic treatment, and the prognosis was good. Conclusion IgG4-related diseases may involve mul-tiple organs, renal dysfunction may occur when the kidney is involved. Interstitial nephritis is the major clinical manifestation. Glomerular lesions may accelerate the progress of IgG4-TIN. Corticosteroid therapy is effective.
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La hipofisitis linfoplasmocitaria con expresión de inmunoglobulina G4 (IgG4) es una entidad de reciente conocimiento. Pertenece al grupo de enfermedades relacionadas a IgG4 (IgG4-RD, del inglés: IgG4-related disease), donde uno o varios órganos pueden estar comprometidos, con síntomas compresivos u obstructivos, o disfuncionalidad por infiltración celular. La hipófisis puede estar afectada en forma aislada. Clínicamente, se presentan con diabetes insípida, hipopituitarismo y/o síntomas de masa ocupante selar, siendo los principales diagnósticos diferenciales los adenomas selares no secretantes, y otros tipos de hipofisitis. Para arribar al diagnóstico de este tipo patología es necesaria la presencia de una imagen de agrandamiento selar o engrosamiento del tallo pituitario en la resonancia magnética nuclear, una histopatología característica con inmunomarcación positiva para IgG4 en más de 10 células plasmáticas por campo de gran aumento y la presencia de IgG4 sérica elevada. Tienen una excelente respuesta a glucocorticoides, por lo que una sospecha diagnóstica oportuna evitaría una cirugía innecesaria en la mayoría de los pacientes con esta entidad.
Immunoglobulin G4 (IgG4)-related lymphoplasmacytic hypophysitis is a recently known entity. It belongs to the IgG4-related diseases (IgG4-RD), in which one or more organs may be involved, with compressive or obstructive symptoms, or dysfunctionality due to cellular infiltration. The pituitary gland can be isolatedly affected. Clinically, lymphoplasmacytic hypophysitis presents with diabetes insipidus, hypopituitarism and/or symptoms of an occupying sellar mass, being the non-secreting sellar adenomas and other types of hypophysitis the main differential diagnosis. In order to reach the diagnosis, the presence of pituitary enlargement or pituitary stalk thickening on an MRI scan, a distinctive histopathology with positive for IgG4 immunostaining in more than 10 plasma cells per high-powerfield, and elevated serum IgG4 levels, confirms this type of hypophysitis. As this entity has an excellent response to glucocorticoids, the diagnosis suspicion may avoid an unnecessary surgery in most patients.
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Humans , Male , Female , Immunoglobulin G/adverse effects , Immunoglobulin G/immunology , Hypophysitis/diagnosis , Immunoglobulin G/analysis , Diagnosis, Differential , Hypophysitis/classification , Hypophysitis/epidemiologyABSTRACT
A 39-year-old female with central diabetes insipidus complained of polydipsia and polyuria and was found to be accompanied by lung lesions. The diagnosis of IgG4-related disease was confirmed by laboratory and pathological results. It should be alert to consider the possibility of IgG4-related disease in a patient with central diabetes insipidus coexisting with the signs of multisystem lesions such as lung disease.
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Purpose To study the clinicopathological features and immunohistochemical characteristics of IgG4-related disease. Meth-ods The microscopic characteristics and immunohistochemical staining (EnVision) of IgG, IgG4, CD138 and CD34 have been per-formed on 12 cases of IgG4-related disease. Results IgG4-related disease were characterized by diffuse fibrosis, accompanied with in-filtrating of dense lymphocytes and plasma cells surrounding neurovascular and occlusive phlebitis. Immunohistochemical staining re-sults showed the ratio of IgG4+/IgG+ cells were over 40%. Conclusions IgG4-related disease is absent of characteristic clinical and radiographic features and is easily misdiagnosed as tumor. Preoperative serum IgG4 detection could be used as the prior examina-tion for the suspected cases.
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Objective To investigate the clinical significance of IgG subclasses in patients with primary Sj(o)gren's syndrome (pSS) patients.Methods The serum was collected from 101 pSS patients and 40 healthy subjects.The content of IgG subclasses was determined by immuno-scatter turbidmetry.The difference of IgG subclasses distribution between pSS patients and healthy subjects was analyzed.Com-bined with the clinical information of the SS patients,the difference of IgG subclasses distribution in SS patients with different characteristics was analyzed.For group comparisons,measurement data were compared using unpaired Student's t test.Results The average levels of serum IgG1-4 in 40 healthy controls were (8085±1566) mg/L,(5406±2151) mg/L,(395±233) mg/L and (566±420) mg/L.The percentage of IgG1-4 were (56.8±8.2)％,(36.6±7.8)％,(2.7±1.5)％ and (3.9±2.8)％.The average levels of serum IgG1-4 in 101 pSS patients were (14 094±5534) mg/L,(3912±1750) mg/L,(565±565) mg/L and (404±504) mg/L,The percentage of IgG1-4 were(73.0±10.9)％,(21.7±9.8)％,(3.1±2.0)％ and (2.2±2.4)％.In pSS patients,the levels of IgG1,IgG3 and IgG1/IgG were significantly higher,while the IgG2,IgG2/IgG and IgG4/IgG levels were significantly lower,compared with those of the healthy subjects (P＜0.05).There was no significant difference in the IgG subclasses distribution between the pSS patients whose had systemic involvement and the pSS patients who did not have systemic involvement(P＞0.05).The IgG subclasses distribution was abnormal in SS patients who IgG was normal,compared with the healthy subjects.Conclusion A characteristic IgG subclasses distribution abnormality exists in pSS patients,and the production of anti-SSA (Sj(o)gren's syndrome A) antibody is associated with the increase of IgG1.However,no relationship has been found between the systemic involvement of pSS patients and the abnormal distribution of IgG subclasses.
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Objective Mikulicz's disease (MD) was considered to be a subtype of Sj(o)gren's syndrome (SS) due to the clinical and histological similarities between them.Evidence had shown that there were differences between MD and typical SS.The purpose of this study was to investigate the correlation between MD and SS,by means of analyzing the expression of IgG4 in salivary glands and the clinical characteristics of patients who were previously considered as SS.Methods The paraffin sections of salivary glands from SS patients were stained with monoclonal antibodies to IgG4 and CD38.Patients were divided into two groups based on the pathological results.Analysis of the symptoms,the signs and the laboratory results were carried out in these patients.The difference in laboratory parameters and histopathological gradings in the two groups was analyzed.Normal and abnormal distributed data comparison was conducted using random independent samples t-test and rank sum test respectively.Two-sample rates were compared with Chi-square test.Results Based on immunohistochemistry of IgG4 distribution,the 58 patients with SS were divided into two groups:IgG4 related (9 cases) and non-IgG4 related (49 cases).Histopat-hologically,IgG4 related cases showed IgG4+ plasma cells/IgG+ plasma cells infiltration and there were more IgG4 related monoclonal antibody expressed when compared to IgG4 unrelated cases.In addition,there were also significant differences in clinical features between the two groups.IgG4 related disease was associated with male gender,higher level of plateletconnt,lymphocytes [(2.4±0.8)×109/L vs (1.4±0.7) ×109/L] count and CRP [(52±60) mg/L vs (15±17) mg/L] levels and lower titer of IgM [(1.2±0.7) g/L vs (1.8±0.8) g/L],antinuclear antibody (56％ vs 87％) and anti-SSB antibodies (13％ vs 54％) (P＜0.05),when compared with IgG4-unrelated cases.There was no significant difference in other indicators (P＞0.05).Conclusion The present study has demonstrated that some of the MD patients misdiagnosed as SS.Some of the laboratory tests such as the level of platelet and lymphocyte count,serum level of CRP,IgM,antinuclear antibody,anti-SSB antibodies,the serum levels of IgG4 and the histopathological presentations in the salivary gland are different between these two disorders.Because of good response to steroid in MD,so laboratory tests and pathological examinations for IgG4 can help to avoid misdiagnosis.