Immunoglobulin G4–related lacrimal sac dacryocystocele: A novel case report.
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SUBMITTER: Au SCL
PROVIDER: S-EPMC7334390 | biostudies-literature | 2020 Sep
REPOSITORIES: biostudies-literature
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Project description:BackgroundImmunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition with high serum IgG4 levels affecting various organs, such as the pancreas, lacrimal and salivary glands, thyroid, kidney, and lung. Typical cardiovascular manifestations of IgG4-RD include periaortitis, coronary arteritis, and pericarditis. However, reports of IgG4-RD associated with coronary arteritis are rare. Here, we report a case of IgG4-related masses surrounding the coronary arteries.Case summaryA 59-year-old man was referred to our hospital because of mediastinal masses detected by computed tomography (CT). Coronary CT angiography revealed masses surrounding the right coronary artery and the left anterior descending coronary artery. An elevated serum level of IgG4 and histological findings led to the diagnosis of IgG4-related coronary arteritis with mass formation. Coronary angiography showed numerous feeding arteries to the masses, which were demonstrated as multiple microchannels in the intravascular ultrasound (IVUS) images.DiscussionIgG4-RD involving the cardiovascular system has been reported. However, coronary artery disease associated with IgG4-RD is very rare, and the mechanism of mass formation in IgG4-related coronary arteritis is unclear. In our case, within the cardiovascular system, IgG4-RD was limited to the coronary arteries, suggesting that the affected coronary arteries may provide the necessary blood supply to the mass, thus, aiding its growth. These findings were supported by the images from coronary angiography and IVUS.
| S-EPMC8186918 | biostudies-literature
Project description:Immunoglobulin G4-related disease (IgG4-RD) is a heterogeneous autoimmune fibrosing disorder that presents common pathologic features but with unclear etiology. We report a rare case of IgG4-RD accompanied by primary myelofibrosis that eventually transformed into acute myeloid leukemia. A 50-year-old woman suffered from progressive lacrimal and parotid gland enlargement, diaphoresis, and rapid weight loss. Important clinical findings included remarkable leukocytosis, hyperglobulinemia, and splenomegaly. IgG4-RD was confirmed by salivary gland biopsy. Meanwhile, myelofibrosis was diagnosed according to histopathological findings of bone marrow and genetic mutation test of peripheral blood. The patient was on corticosteroid treatment. However, she developed into acute myeloid leukemia (AML) in the 8th month of follow-up. Our case suggested that myeloproliferative neoplasm (MPN) may co-occur with IgG4-RD. Bone morrow aspiration and genetic tests are helpful for throughout evaluation. An active search for hematological malignancies is warranted at diagnosis and during follow-up for patients who present with unexplained leukocytosis, pancytopenia, splenomegaly, or weight loss.
| S-EPMC8129158 | biostudies-literature
Project description:BackgroundImmunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory systemic disease of unknown etiology that can affect one or multiple organs. The disease can mimic many infectious and inflammatory diseases, mainly causing organ enlargement or hyperplasia. Its diagnosis primarily relies on clinical, serologic, and histological features (lymphoplasmacytic infiltrates, storiform fibrosis, and obliterative phlebitis of IgG4 + plasma cells). Here, we report a rare case of IgG4-related periodontitis, and review the relevant literatures.Case presentationA 38-year-old Chinese man visited the Department of Periodontics with gingival enlargement, loose teeth, and tooth loss. The patient had very poor oral hygiene and a large amount of calculus. Gingivae were edematous with deep periodontal pockets and attachment loss. Panoramic radiography showed alveolar bone loss. Serologic examination showed that IgG was 23.70 g/L and IgG4 concentration was 2.800 g/L. There was significant lymphoplasmacytic infiltration, a storiform pattern of fibrosis, and mitotic figures with hematoxylin and eosin staining; immunohistochemical staining showed 10 scattered IgG4-positive plasma cells in a high-power field. The patient was diagnosed as IgG4-related periodontitis. He received a course of corticosteroids with periodontal therapy, and the enlargement was significantly improved without recurrence.ConclusionIgG4-RD in the oral and maxillofacial region mainly involves salivary glands, but this rare case was characterized by enlarged gingivae. The differential diagnosis of IgG4-RD should be based on the clinical features and serologic (IgG4) and histopathological examinations. Corticosteroid therapy is effective for most IgG4-RD patients. Taken together, we hope this case report and the literature review can help dentists to improve their understanding of the IgG4-RD.
| S-EPMC8161922 | biostudies-literature
Project description:The authors report a case of immunoglobulin G4-related disease (IgG4-RD) presenting as a paraspinal, epidural mass. This disease encompasses a host of autoimmune conditions that were previously thought to be separate entities. It is characterized by fibrosis, mediated by the aberrant proliferation and tissue invasion of IgG4-positive plasma cells, which can occur in any organ. As with other autoimmune conditions, it tends to be responsive to steroids and other immunosuppressants. It can rarely present as a tumefactive lesion of the central nervous system, creating the potential for misdiagnosis (given its similar radiological appearance to malignancy) and mistreatment. In 2015, a panel of experts convened to set forth guidelines for the diagnosis and treatment of IgG4-RD. In the case presented here, the patient initially presented with pain and weakness in the left upper extremity. Initial neuroimages revealed a contrast-enhancing mass extending from C-4 to T-1, invading the epidural spinal canal, encasing the exiting nerve roots, infiltrating the paraspinal musculature, and surrounding the left vertebral artery. A PET scan confirmed the mass was hypermetabolic, but results of fine-needle aspiration and CT-guided biopsy were inconclusive. Open biopsy yielded fibrotic tissue that met the pathological criteria for IgG4-RD: lymphoplasmacytic infiltrate, fibrosis in a storiform pattern, and obliterative phlebitis. The patient was treated with 2 doses of 4 mg of dexamethasone (Decadron) and then 50 mg of prednisone per day. Within 2 weeks, the mass was radiologically shown to have drastically decreased in size. The prednisone dose was decreased to 40 mg per day, and 100 mg of azathioprine per day was added. The patient continued to improve and the mass continued to decrease over the next 6 months. Currently, she has been weaned from all steroids and will be maintained on a daily dose of 100 mg of azathioprine.
| S-EPMC5548420 | biostudies-other
Project description:BackgroundConstrictive pericarditis remains a problematic diagnosis and a thorough investigation is critical. Among possible aetiologies, immunoglobulin-G4 (IgG4)-related pericardial disease is an unusual cause of pericardial constriction. We report a challenging diagnostic case of pericardial constriction due to IgG4-related disease.Case presentationA 68-year old male with a history of inferior myocardial infarction with right ventricle (RV) involvement was thrice-hospitalized due to marked ascites and peripheral oedema. Systemic congestion was initially attributed to RV dysfunction due to previous infarction. Yet, at the final admission, a re-assessment echocardiogram followed by cardiac computed tomography, magnetic resonance and right heart catheterization raised a possible diagnosis of constrictive pericarditis with a finding of abnormal pulmonary venous return. Patient therefore underwent pericardiectomy and surgical correction of pulmonary venous return. Pericardium histology revealed an IgG4-related pericardial constriction. Patient was later discharged on corticosteroids with marked symptomatic improvement.ConclusionIgG4-related disease remains a rare cause of pericardium constriction while also presenting a challenging diagnosis in everyday clinical practice. This case exemplifies the difficulties faced by clinicians when reviewing a possible case of constrictive pericarditis, while highlighting the importance of a multimodality assessment.
| S-EPMC8815176 | biostudies-literature
Project description:BackgroundImmunoglobulin G4-related disease (IgG4-RD) is an autoimmune condition associated with high serum IgG4 levels which was first reported as autoimmune pancreatitis in 2001. Since then, many additional organs, such as bile duct, salivary gland, retroperitoneal organs, and liver, have been reported with high serum IgG4 levels in cases of IgG4-RD. However, evidence of the relationship between IgG4-RD and coronary artery disease (CAD) has been scare. Here, we report a case of CAD concomitant with IgG4-RD.Case summaryA 74-year-old man was referred to our hospital with a chief complaint of chest pain and was admitted. The patient was found to have had a myocardial infarction with ST-segment elevation and underwent an emergent percutaneous coronary intervention. Owing to a rapidly increased blood glucose level, computed tomography was performed and showed autoimmune pancreatitis. An elevated serum level of IgG4 led to a diagnosis of IgG4-RD involving acute coronary syndrome (ACS).DiscussionCardiac involvement in IgG4-RD has been reported; however, cases of ACS concomitant with IgG4-RD are rare. Our report suggests that CAD, specifically ACS, can coexist in patients with IgG4-RD. Therefore, cases of concomitant CAD and IgG4-RD should be accurately diagnosed and evidence should be collected to elucidate the mechanism and characteristics of this condition.
| S-EPMC6439393 | biostudies-literature
Project description:Immunoglobulin G4-related disease (IgG4-RD) has recently been recognized as a systemic fibroinflammatory disease characterized by IgG4-positive lymphoplasmacytic infiltration. Despite the increased awareness of this disease category, cardiovascular involvement has rarely been reported. An 83-year-old man was admitted because of a moderate amount of pericardial effusion concomitant with bilateral pleural effusion. Despite aggressive heart failure therapy, pericardial effusion increased. He underwent pericardial drainage, and heart failure symptoms significantly improved. After only 1 month, right-sided heart failure with re-accumulation of pericardial fluid recurred, although the results of cardiac catheter examination were not indicative of constrictive pericarditis. Despite the aggressive medication, he developed weight gain and pleural effusion 6 months after pericardial drainage. Based on multimodal hemodynamic and morphological evaluations, he was finally diagnosed with effusive constrictive pericarditis and underwent total pericardiectomy. Based on histopathological findings, the final diagnosis was established as IgG4-RD associated constrictive pericarditis. We report an extremely rare case of IgG4-RD associated pericardial involvement, in which rapid transition from cardiac tamponade to effusive constrictive pericarditis was confirmed. Diagnostic work-up for constrictive pericarditis requires a high index of suspicion. Increased recognition of IgG4-RD associated pericardial involvement could potentially mitigate morbidity and might contribute to improved patient outcomes. <Learning objective: Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by chronic IgG4-positive lymphocyte infiltration that causes fibrosclerotic changes in various tissues and organs. Detailed serial multimodal imaging and physical examination contribute to the recognition of latent constrictive physiology and clinical decision-making. Increased recognition of IgG4-RD associated pericardial involvement could lead to early diagnosis and might contribute to improved patient outcomes.>.
| S-EPMC8258268 | biostudies-literature
Project description:Immunoglobulin G4-related disease (IgG4-RD), which affects many organ systems, has been recognized as a distinct clinical entity in human medicine for just over a decade but has not been previously identified in dogs. In humans, IgG4-RD is characterized by diffuse IgG4-positive lymphoplasmacytic infiltrates that commonly lead to increased serum concentrations of IgG4 and IgE, peripheral eosinophilia, tumorous swellings that often include the parotid salivary glands, obliterative phlebitis, and extensive fibrosis. Herein we describe the diagnosis, clinical progression, and successful treatment of IgG4-RD in an 8-year-old female spayed Husky mixed breed dog. Immunoglobulin G4-related disease should be considered as a differential diagnosis for dogs with vague clinical signs, lymphoplasmacytic swellings, restricted polyclonal gammopathy, eosinophilia or some combination of these findings.
| S-EPMC6872619 | biostudies-literature
Project description:(IgG4-RD) is an immune-mediated fibrotic disorder characterized by severe resolution of inflammation and dysregulation of wound healing. IgG4-RD has been considered a unique disease since 2003, and significant progress has been achieved in the understanding of its essential features. The central role of B cells in IgG4-RD has been demonstrated by the robust clinical responsiveness of IgG4-RD to B cell depletion and the identification of multiple self-antigens that promote B cell expansion. Studies have increasingly revealed critical roles of these B cells and T cells in the pathogenesis of IgG4-RD, and we and other authors further identified CD4+ cytotoxic T lymphocytes as the main tissue-infiltrating CD4+ T cell subset in IgG4-RD tissues. Additionally, T follicular helper cell subsets that play a role in IgG4 isotype switching have been identified. In this review, we discuss research on IgG4-RD and the roles of B cell and T cell subsets, as well as the functions of CD4+ cytotoxic T cells in IgG4-RD pathogenesis. We highlight our findings from ongoing research using single-cell analysis of infiltrating CD4+ cytotoxic T cells, CD4+ follicular helper T cells, and infiltrating B cells in IgG4-RD and propose a model for the pathogenesis of IgG4-RD.
| S-EPMC9841035 | biostudies-literature
Project description:Introduction Immunoglobulin subtype G4-related disease (IgG4-RD) is a fibroinflammatory disease of unknown etiology, with manifestations involving nearly every organ system. Its association with foreign bodies is not established. Here, we present a novel case of IgG4-RD in response to foreign body injection. Case Description A 58-year-old woman presented with history of persistent left facial pain, xerophthalmia, blurred vision, and trismus. The patient's medical history was significant for left-sided temporomandibular joint (TMJ) reconstruction with silicone injection into the joint. Magnetic resonance imaging revealed a lesion in the left skull base. Biopsies demonstrated the cardinal histopathological features of IgG4-RD. The patient was treated with a tapering dose of prednisolone followed by rituximab, resulting in tumor shrinkage and resolution of her symptoms. Discussion This is the first reported case of IgG4-RD potentially precipitated by a foreign body, in this case injected silicone into the TMJ. The pathogenesis and etiology of IgG4-RD is still not fully elucidated, but allergic and reactive inflammatory reactions have been implicated in the disease process. This case report should raise the idea of reactive foreign bodies as a causative agent for IgG4-RD.
| S-EPMC4648737 | biostudies-other