Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Immunoglobulin G4-related disease in a dog.

ABSTRACT: 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.

SUBMITTER: Colopy LJ

PROVIDER: S-EPMC6872619 | biostudies-literature | 2019 Nov

REPOSITORIES: biostudies-literature

ACCESS DATA

Json Xml

Publications

Immunoglobulin G4-related disease in a dog.

Colopy Lydia J LJ Shiu Kai-Biu KB Snyder Laura A LA Avery Anne C AC Rout Emily D ED Moore A R AR

Journal of veterinary internal medicine 20191026 6

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 e ...[more]

PMID: 31654456

Similar Datasets

Immune dysregulation in immunoglobulin G4-related disease.

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

Immunoglobulin G4-Related Disease Accompanied by Primary Myelofibrosis: Case Report.

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

A medical mirroring: chronic myelomonocytic leukemia mimicking immunoglobulin G4-related disease.

Project description:Chronic myelomonocytic leukemia (CMML) can be complicated by autoimmune features associated with rheumatologic disorders, which have been commonly reported by more researches currently. The intrinsic correlation between CMML and autoimmune diseases can create significant pitfalls in differential diagnosis. CMML occasionally presents with clinical and histopathological manifestations that are similar to those of Immunoglobulin G4-related disease (IgG4-RD), a newly recognized systemic autoimmune disorder. Both CMML and IgG4-RD can have significant overlaps due to the common nature of these systemic disorders, especially when atypical clinical phenotypes are present. It is significant for physicians to accurately distinguish CMML and IgG4-RD because the therapy modalities could differ extremely between the two entities. Here we present a unique case of a 70-year-old female who had a condition that mimicked the onset of IgG4-RD not only in terms of clinical manifestations but also in serology and histopathology analyses. Following a series of rigorous examinations, this patient was ultimately diagnosed as having CMML. Herein, we discuss the aspects of IgG4-RD's differential diagnosis and the need for careful comparison of clinical and laboratory features as well as consideration of the pathogenesis of both IgG4-RD and CMML. We also stress a concept that the presence of autoimmune conditions cannot be the sole basis to exclude diagnosis of CMML, as these disorders can appear concomitantly.

| S-EPMC6684895 | biostudies-literature

Update on classification, diagnosis, and management of immunoglobulin G4-related disease.

Project description:AbstractImmunoglobulin G4-related disease (IgG4-RD) is a newly recognized chronic fibro-inflammatory autoimmune disease, and its recognition has been constantly increasing worldwide over the last few years. A correct and timely recognition, as well as appropriate intervention, is crucial for the treatment of IgG4-RD. For certain subtypes of IgG4-RD, organ-specific criteria are formulated to make the diagnosis more accurate. New biomarkers have emerged in the recent years to aid the disease diagnosis, its prognosis prediction, as well as therapy response monitoring. Although recurrence is very common in IgG4-RD, glucocorticoid is still the first-line treatment for the majority of patients. The factors that affect the likelihood of disease relapse are multifaceted. The selection strategy of various steroid-sparing agents is still being explored. Besides, when patients have special sites involvement leading to severe clinical conditions, surgical operation or interventional therapy should also be considered. An update on classification, diagnosis, and management of IgG4-RD is provided in the current study to fully elucidate the recommended clinical practice of this mysterious disease.

| S-EPMC8869566 | biostudies-literature

Long-term clinical course and outcomes of immunoglobulin G4-related lung disease.

Project description:BACKGROUND:Immunoglobulin G4-related lung disease (IgG4-RLD) is the pulmonary manifestation of a systemic fibroinflammatory disease characterized by lymphoplasmacytic infiltration with an abundance of IgG4-positive plasma cells. Long-term clinical course and outcomes of IgG4-RLD remain unclear. We aimed to identify clinical characteristics, treatment outcomes, and longitudinal pulmonary function changes in patients with IgG4-RLD according to the radiologic classification. METHODS:Chest computed tomography findings of 37 subjects were classified into five subtypes: solid nodular, bronchovascular, alveolar interstitial, round ground glass opacity, and alveolar consolidative. Radiologic treatment outcomes and longitudinal pulmonary function changes were compared among the different radiologic subtypes. RESULTS:The mean age of the subjects was 55.6 years, and 78.4% were male. Among the five radiologic subtypes, alveolar consolidative and solid nodular type were most common, accounting for approximately 29.7% each of the total cases. Prednisone with or without azathioprine was administered to 31 patients (median treatment duration 14 months). In the treated patients, serial images showed complete response or partial response in 77.4%. However, relapse was documented in 25.0% of those who showed complete or partial response. In patients whose longitudinal lung function data were available (n?=?20), the lung function was found to be stable during follow-up. Alveolar consolidative type showed the highest complete response rate, whereas alveolar interstitial type showed the lowest response rate, either complete or partial. CONCLUSIONS:Most patients showed a favorable outcome with regards to radiologic improvement and maintenance of pulmonary function; however, the response differed according to the radiologic subtype.

| S-EPMC7574178 | biostudies-literature

Membranous nephropathy associated with immunoglobulin G4-related disease successfully treated with obinutuzumab.

Project description:Immunoglobulin G4 (IgG4)-releated disease is typically associated with interstitial nephritis, but rare cases of idiopathic membranous nephropathy as a renal manifestation have been described. Obinutuzumab was successfully used in refractory membranous nephropathy, but evidence for the treatment of IgG4-related disease with obinutuzumab is lacking. We report one patient's case with membranous nephropathy associated with IgG4-related disease who was treated with obinutuzumab following an anaphylactic reaction to rituximab. Obinutuzumab treatment resulted in a sustained complete remission of membranous nephropathy and a decrease of IgG4 to the normal range. This case demonstrates that membranous nephropathy associated with IgG4-related disease can be treated successfully with obinutuzumab.

| S-EPMC8862033 | biostudies-literature

Immunoglobulin G4-related kidney diseases: An updated review.

Project description:This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases (IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves multiple organs and is characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis. Cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RDs. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulopathy and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis. IgG4-RD diagnosis should be based on specific histopathological findings, confirmed by tissue immunostaining, typical radiological findings and an appropriate clinical context. The first line treatment is the steroids with two warnings: Steroid resistance and relapse after discontinuation. In the case of steroid resistance, B cell depleting agents as rituximab represent the second-line treatment. In the case of relapse after discontinuation, steroid treatment may be associated with steroid sparing agents. Since the disease has been only recently identified, more prospective, long-term studies are needed to an improved understanding and a more correct and safe treatment.

| S-EPMC5760510 | biostudies-literature

Clinical Images: Dacryoadenitis in eosinophilic granulomatosis with polyangiitis mimicking immunoglobulin G4-related disease.

Project description: Not available

| S-EPMC9746660 | biostudies-literature

Efficacy of Cyclophosphamide treatment for immunoglobulin G4-related disease with addition of glucocorticoids.

Project description:Aim to evaluate the efficacy and safety of glucocorticoid monotherapy vs combination therapy of cyclophosphamide (CYC) for IgG4 related disease (IgG4-RD). 102 newly diagnosed IgG4-RD patients were enrolled and assigned to 2 groups: Group I was prednisone monotherapy (0.5-1.0 mg/kg.d, tapered gradually) and Group II was glucocorticoid and CYC (50-100 mg per day). Patients were assessed at different periods. Primary end point was relapse rate; secondary end points included response, remission rate and adverse effects. 52 patients were in Group I and 50 in Group II. At 1 month, both groups achieved obvious improvement. Accumulated relapse rate during 1 year was 38.5% in Group 1, including 12 cases with clinical relapse and 8 patients manifesting only serological relapse; whereas there was 12.0% of relapse in Group 2, only 1 with clinical relapse and other 5 patients got serological relapse. The mean flare time in Group II was significantly longer than that in Group I. All relapsing patients in Group I were sensitive to immunosuppressants. Most patients involving more than 6 organs in Group I relapsed during 1 year. IgG4 levels of relapse cases were significantly higher than non-relapsing patients at baseline. Bile duct, lacrimal glands and lymph nodes were commonly relapsed organs in Group I.

| S-EPMC5522435 | biostudies-literature

Coronary artery disease concomitant with immunoglobulin G4-related disease: a case report and literature review.

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

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data