ObjectiveTo explore clinical manifestation, diagnosis and treatment of IgG4 sclerosing cholangitis developed postoperative gastroduodenal hemorrhage, so as to improve awareness and treatment of this disease. MethodThe clinical data of a case of IgG4 sclerosing cholangitis misdiagnosed as the hilar cholangiocarcinoma which developed postoperative gastrointestinal hemorrhage in this hospital were analyzed retrospectively. ResultsThis patient was misdiagnosed as the hilar cholangiocarcinoma and accepted the radical resection, while the postoperative pathology proved to be the IgG4 sclerosing cholangitis. One month later, the patient developed the acute gastrointestinal hemorrhage and it was resolved by using the endovascular embolization. ConclusionsPreoperative distinguishing IgG4 sclerosing cholangitis from hilar cholangiocarcinoma can avoid an unnecessary surgery. Endovascular intervention is both a useful measure of diagnosis and treatment for gastroduodenal pseudoaneurysm. Attention should be paid to arterial protection during process of arterial osteogenesis in hepatobiliary operation.
Objective To elucidate current research status of immunoglobulin G4 (IgG4) and cancer immunity. Method The relevant literatures of relationship between IgG4 and cancer immunity were collected and reviewed. Results The IgG4 high-level and the intratumoral infiltration of the IgG4-positive plasma cells were the predictors for a worse prognosis in the cancer patients. The relationship between the serum IgG4 level and the prognosis in the cancer patients was unclear. The IgG4 related immunity might contribute to the tumor-associated escape from the immune surveillance. Conclusions Recent studies implicate that IgG4 might play a role in tumor progression. Specific mechanisms for IgG4 in tumor immune microenvironment need to be further explored. Dissecting relationship between IgG4 and cancer immunity might provide a novel idea for cancer therapy.
ObjectiveTo summarize the clinical characteristic of IgG4 related lung disease. MethodsThe clinical manifestation,laboratory examination,imaging,diagnosis and treatment data of 2 patients with IgG4 related lung disease admitted in the PLA General Hospital from January 2000 to January 2014 were collected and analyzed retrospectively.Related literatures were also reviewed. ResultsThe serum IgG level of IgG4 related lung disease might be normal and the levels of IgG1,2,3 and 4 might increase.Some autoantibodies were positive.IgG4 related lung disease could be easily misdiagnosed as Sjogren's syndrome associated with interstitial pneumonia when the lacrimal gland and salivary gland were involved,and the chest CT occasionally showed multiple vesicles.The immunochemical staining of lung tissues revealed the increase of IgG4/IgG and the number of IgG4 positive lymphatic plasma cells >10/HP.The combination of clinical manifestation,laboratory examination,imaging and pathology results was more conducive for the diagnosis of IgG4 related lung disease.After the treatment of glucocorticoid,the clinical symptoms were relieved and the serum IgG4 level obviously decreased. ConclusionIgG4 related lung disease is rare in clinic,which can involve the lung alone or multiple organs.The increase of IgG4/IgG indicated by the immunochemical staining of lung tissues is the gold standard for diagnosis.Glucocorticoid has good treatment effect in IgG4 related lung disease.
Objective To summarize the relationship between IgG4 and IgG4 related thyroid diseases. Methods Domestic and international publications involving the pathological features of IgG4-related thyroid diseases and relationship with IgG4 were retrieved and reviewed. Results IgG4-related disease was a newly recognized class of chronic and systemic lymphocytes disease， which may be solitary or involving multiple body organs， as well as thyroid. The expression of IgG4 was found in leisons of Hashimoto thyroiditis， Riedel thyroiditis， and papillary thyroid carcinoma. Conclusions IgG4-related thyroid disease is a new concept of thyroiditis. The knowledge of this new disease will provide appropriate treatment for patients with thyroiditis.
ObjectiveTo analyze the R0 resection rate and survival time of pancreatic cancer with serum IgG4 elevated, and to discuss whether serum IgG4 can distinguish autoimmune pancreatitis from pancreatic cancer.MethodsThe retrospective cohort study was adopted. The clinical data of 146 patients with pancreatic cancer confirmed by histology in Affiliated Hospital of Qingdao University from January 2016 to December 2019 were analyzed retrospectively. According to the level of serum IgG4, they were divided into normal IgG4 group (<1.35 g/L, n=124) and IgG4 elevated group (≥1.35 g/L, n=22). The tumor R0 resection rate, survival time and whether complicated with AIP of the two groups were compared.ResultsOne hundred and one patients (81.5%) with normal serum IgG4 underwent radical surgery, while only 13 patients (59.1%) with elevated serum IgG4 underwent radical surgery, the difference was significant (P=0.019). The median survival time of patients with normal serum IgG4 was 18.7 months, while patients with elevated serum IgG4 was 8.1 months, there was no significant difference between the two groups (P=0.121). Subgroup analysis showed that the median survival time of patients with pancreatic ductal adenocarcinoma in the normal IgG4 group was 17.5 months, while the IgG4 elevated group was 6.8 months, the difference was significant (P=0.016). Only 1 case of pancreatic cancer with AIP in the 2 groups.ConclusionsSerum IgG4 ≥1.35 g/L indicates low radical resection rate in pancreatic cancer and poor prognosis in pancreatic ductal adenocarcinoma. Serum IgG4 can only be used as an auxiliary index to distinguish pancreatic cancer from autoimmune pancreatitis.
IgG4-related disease (IgG4-RD) is a chronic, immune-mediated inflammatory and fibrotic condition that can affect virtually any organ system. While some patients experience involvement of only a single organ, isolated IgG4-RD of the mediastinum is even rarer. This article reports a case of a 48-year-old male patient with isolated IgG4-RD of the middle mediastinum. After a biopsy failed to establish a definitive diagnosis, the patient underwent a right thoracotomy-assisted mediastinal tumor resection via video-assisted thoracoscopic surgery. The tumor was completely resected during the procedure, which lasted 130 minutes, with an estimated blood loss of approximately 50 mL. The patient had a favorable postoperative course and satisfactory clinical outcome. No glucocorticoid or other medical treatment was administered postoperatively, and there was no recurrence during a 6-month follow-up period.
Objective To identify potential clinical phenotypes in immunoglobulin G4-related disease (IgG4-RD) and to characterize differences in immune and metabolic profiles across distinct phenotypic subgroups. Methods A total of 125 patients diagnosed with IgG4-RD at West China Hospital, Sichuan University between January 2020 and December 2024 were retrospectively selected, and data regarding 12 organs (including the prostate) were collected. The prostate data were utilized solely for descriptive statistical purposes. Latent class analysis (LCA) was conducted using 11 organ variables, excluding the prostate, to perform a data-driven unsupervised classification of multiple organ involvement patterns. The optimal model was selected by comprehensively considering model fit indices, class size distribution, and clinical interpretability. Serum levels of immunoglobulin (Ig)G4, IgE, and uric acid were compared across different phenotypes. Results Among 125 patients with IgG4-RD, 89 were male (71.2%) and 36 were female (28.8%). Involvement of the pancreas was observed in 25 cases (20.0%), lymph nodes in 23 cases (18.4%), biliary system in 18 cases (14.4%), lungs in 16 cases (12.8%), lacrimal glands in 13 cases (10.4%), salivary glands and retroperitoneal fibrosis in 11 cases each (8.8%), eyes and pituitary glands in 7 cases each (5.6%), kidneys and aorta in 6 cases each (4.8%). LCA supported a three-class solution as optimal. Three distinct clinical phenotypes were identified: Class 1 (pulmonary-involvement type, n=26), characterized by frequent lung and lymph node involvement; Class 2 (mild systemic involvement type, n=89), showing limited multi-organ engagement; and Class 3 (glandular-lymphatic type, n=10), defined by predominant involvement of salivary glands, lacrimal glands, and lymph nodes, features resembling Mikulicz’s disease. Immunological and metabolic analysis revealed that the serum IgG4 level in Group 3 was higher than that in Group 1 and Group 2, but there was no statistically significant difference between the three groups (P>0.05). Spearman’s rank correlation analysis indicated a positive correlation between uric acid and IgG4 levels (r=0.2454, P=0.0067) as well as between IgE and IgG4 levels (r=0.4169, P<0.001). Conclusions Three distinct intrinsic clinical phenotypes of IgG4-RD were identified by LCA. Each phenotype exhibits characteristic patterns of organ involvement, along with specific immune and metabolic profiles. This suggests that interactions between metabolic and immune pathways may contribute to phenotypic differentiation and disease progression.