IgG4-related pseudo-tumor of the kidney and multiple organ involvement mimicked malignancy.
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ABSTRACT:
SUBMITTER: Surintrspanont J
PROVIDER: S-EPMC6606968 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:IntroductionIgG4-related disease (IgG4-RD) is a relapsing multisystem fibro-inflammatory disease, which may involve the kidney (IgG4-related kidney disease [IgG4-RKD]) and retroperitoneum (IgG4-related retroperitoneal fibrosis [IgG4-RPF]). The aim of this study was to describe IgG4-RKD and IgG4-RPF in the United Kingdom.MethodsWe conducted a retrospective observational study of patients with IgG4-RKD and IgG4-RPF in a multicenter IgG4-RD cohort. Data were collected through review of medical records. We describe clinical parameters at baseline, histological and radiological findings, treatment, and patient outcomes.ResultsOf 154 patients with IgG4-RD, 14 (9.1%) had IgG4-RKD, 10 (6.5%) had IgG4-RPF, and 4 (2.6%) had both. Patients were aged 58.2 ± 14.2 years, and 26 (92.9%) were male. Creatinine at presentation was worse in those with intrinsic renal disease (229 ?mol/l vs. 110 ?mol/l; P = 0.0076). Serum IgG4 was elevated in the majority of patients (87.5%), and hypocomplementemia was present in half of those with IgG4-RKD. Fifteen patients underwent renal biopsy; tubulointerstitial nephritis with abundant IgG4+ plasma cells was the most common finding (n = 14; 93.3%), and 4 (26.7%) patients had membranous nephropathy. Most patients (89.3%) were treated with corticosteroids, and 4 (16.0%) with additional azathioprine as initial management. Thirteen patients (46.4%) relapsed over 60 ± 48 months of follow-up, at median 18 (12-36) months after renal/RPF diagnosis; 61.5% of relapses were in the kidney. Renal function deteriorated in 5 patients (20.8%), including 2 (8.3%) who reached end-stage renal disease (ESRD).ConclusionIgG4-RKD and IgG4-RPF represent major organ manifestations of IgG4-RD, and should be identified early with prompt treatment to prevent progression to ESRD.
| S-EPMC6308386 | biostudies-literature
Project description:Introduction and importanceRetroperitoneal fibrosis (RPF) is a rare disease characterized by the replacement of normal tissue with fibrosis and inflammation. We present the case of a 40-years-old gentleman with RPF whose presenting complaints were bilateral flank pain and weight loss and was found to have IgG4 related disease. To the best of our knowledge, IgG4-related disease with isolated retroperitoneal involvement is a very rare occurrence.Case presentationThe diagnosis of IgG4-Related retroperitoneal fibrosis was made based on clinical, radiological and histopathological criteria. Imaging revealed isolated retroperitoneal involvement and the patient was started on oral steroids with a good clinical response after ten days. Repeated imaging months later showed significant regression in the fibrosis.Clinical discussionRPF can occur due to many etiologies and is categorized to idiopathic and secondary. Factors associated with secondary RPF include medications, autoimmune disease, malignancy and IgG4 related disease. Almost all the reported cases of IgG4 related disease had evidence of multifocal involvement such as the pancreas, aorta and kidneys. Our patient was diagnosed with isolated RPF due to IgG4 disease. He met all the suggested diagnostic criteria, was started on oral steroids and had an excellent clinical outcome.ConclusionIgG4 related disease can present with isolated retroperitoneal fibrosis without involvement of other organ systems. The diagnosis should be based on specific criteria. Treatment with corticosteroids can lead to remission both clinically and radiographically.
| S-EPMC7773681 | biostudies-literature
Project description:Although increased serum IgG4 level and tissue infiltration of IgG4-positive cells are key events in IgG4-related disease (IgG4RD), and nearly half of IgG4RD patients show hypocomplementemia, the role of IgG4 in the pathogenesis of IgG4RD remains unclear. Many reports show that altered IgG glycosylation, especially IgG with agalactosylated N-linked glycan (G0 N-glycan), have proinflammatory roles including complement activation, implicated in the pathogenesis of various inflammatory diseases. This study determined the concentration of N-linked glycans (N-glycan) released from serum IgG4 in IgG4RD patients and compared the difference of glycosylation changes to those in healthy controls. We also compared the concentration of each IgG4 glycoform between patients with and without hypocomplementemia and individual organ involvement (kidney, pancreas, lymph node) in IgG4RD.We collected sera from 12 IgG4RD patients and 8 healthy controls. IgG4 was isolated from sera via Melon™ Gel IgG Spin Purification Kit followed by Capture Select IgG4 (Hu) Affinity Matrix. IgG4 N-glycans were analyzed by S-BIO GlycanMap® Xpress methodology.Significant increases of IgG4 G0 N-glycan and IgG4 fucosylated N-glycan (F1 N-glycan) concentrations were observed in IgG4RD compared with healthy controls. Although we observed decreased levels of IgG4 F0 glycan in IgG4RD with hypocomplementemia, there were no significant differences in the galactosylation and sialyation of IgG4 N-glycans. Furthermore, there were no significant differences in the glycosylation of IgG4 N-glycans between patients with and without individual organ involvement of IgG4RD.Although IgG4 has anti-inflammatory properties, IgG4 G0 and F1 glycans were increased in patients with IgG4RD. Our results suggest that decreased galactosylation of IgG4 is not related to complement activation and the differences of individual organ involvement in IgG4RD. IgG4 fucosylation change may be related to complement activation in IgG4RD. Further investigation is needed to clarify the role of IgG4 in IgG4RD.
| S-EPMC5908088 | biostudies-literature
Project description:IgG4-Related Disease (IgG4-RD) is a fibroinflammatory condition characterized by a typical histopathological pattern (dense lymphoplasmacytic infiltrate with prevalent IgG4+ plasma cells and storiform fibrosis), which may involve the kidney both directly (IgG4-related kidney disease, IgG4-RKD) or indirectly, as a consequence of post-renal ureteral obstruction due to retroperitoneal fibrosis (IgG4-RD RF). The most frequent presentation of IgG4-RKD is IgG4-related tubulointerstitial nephritis (TIN), but a glomerular disease can be present, in most of the cases a membranous nephropathy. Albeit steroid-responsive, in some cases renal manifestations may lead to progressive and permanent organ damage. In this review we describe four clinical cases representative of typical and less typical renal manifestations of IgG4-RD, emphasizing a potential, subclinical, early involvement of the kidney in the disease.
| S-EPMC8044528 | biostudies-literature
Project description:Thrombosis is a dangerous complication of cancer. At least 20% of cancer patients are diagnosed with a venous thrombotic event and 1% with an intra-arterial thrombotic event. Here, we present a patient that developed separate thrombi that occurred simultaneously in both the venous and arterial circulation. An 80-year-old woman with a history of recently diagnosed squamous cell lung cancer presented to our institution with an out of hospital cardiac arrest. On arrival, she was found to have an inferior ST-elevation myocardial infarction on electrocardiogram and on examination was found to have right-sided neurological deficits. Computed tomography head and aortogram showed an acute ischaemic stroke and bilateral segmental pulmonary emboli. Coronary angiogram showed thrombotic occlusion distal to the right coronary artery, and the patient underwent aspiration thrombectomy with thrombolysis in myocardial infarction 3 flow established at the end of the procedure. The patient was then transferred to interventional radiology where she had successful clot retrieval of the cerebral thrombus. The patient progressed well and had full neurological recovery 72 h post-presentation. Subsequent transoesophageal echocardiography showed no evidence of a patent foramen ovale or other intracardiac shunt. The patient was prescribed long-term anticoagulant with Clexane. There was suspicion for a deep vein thrombosis with subsequent embolization to the lungs and paradoxical embolization through an intracardiac shunt. However, no such defect was detected and it appears that the patient did develop thrombi in the arterial and venous system separately. This case highlights the prothrombotic state of malignancy, with the patient suffering from multiple separate life-threatening thrombi.
| S-EPMC6601175 | biostudies-literature
Project description:IgG4-related disease (IgG4-RD) is an increasingly recognized pathological entity that tends to involve multiple organs with an elevated level of serum IgG4, which is easily misdiagnosed owing to sharing common clinical features with a variety of other diseases. Here, we report an interesting IgG4-RD case of a woman with progressive multi-organ involvement for over 19 years, started with swollen eyelids, dry eye and mouth, and polydipsia and hydruria. Imaging diagnosis revealed diffuse enlargement of the parotid glands, enlargement of the head of the pancreas, pulmonary infection and interstitial lung. Serological tests showed a remarkable elevation of the serum IgG4, and cytological analysis further revealed a large amount of lymphoplasmacytic infiltration into the focal lobule, and IgG4-positive cell infiltration in bladder mucosa. Therapeutically, the patient responded well to steroid therapy, and thus, she was diagnosed as IgG4-RD suspicious. This report highlights the importance of an early diagnosis in this autoimmune disease and suggests that patients with a clinically unclear cause of inflammation, swelling and refractory glands, rhinitis, pancreatitis, hypophysitis, and/or interstitial pneumonia should be considered for IgG4-RD. The plasma IgG4 level and lymphoplasmacytic infiltration may be useful indexes for screening, and a low dose of steroid maintaining therapy may offer benefits for patients with IgG4-RD.
| S-EPMC6232572 | biostudies-other
Project description:A 56-year-old male subject with bilateral eyelid swelling was diagnosed with an immunoglobulin G4–related disease. After whole-body surveillance, concomitant coronary arteritis with a mural thrombus and myocardial involvement were observed. In this case, multimodal diagnostic imaging assessment led to the diagnosis of both coronary arteritis and myocardial fibrosis associated with immunoglobulin G4–related disease. (Level of Difficulty: Advanced.) Graphical abstract
| S-EPMC10107001 | biostudies-literature
Project description:BackgroundThe relationship between IgG4-related disease (IgG4-RD) and the risk of malignancy is still controversial. This article focused on assessing the risk of cancer in patients with IgG4-RD by meta-analysis.MethodsWe conducted a systematic review of the literature and meta-analysis characterizing the associated risk of overall malignancy and four site-specific malignancies (pancreas, lung, gastric and lymphoma) in patients with IgG4-RD. A search from 2003 to 2020 was performed using specified terms from PubMed, Embase, Web of Science and SinoMed. Random-effects model analysis was used to pool standardized incidence ratios (SIRs) and 95% confidence intervals (CIs). Subgroup and sensitivity analyses were conducted to clarify the heterogeneity of the included studies. Begg's funnel plot and Egger's linear regression test were used to evaluate the bias of the meta-analysis. A P value < 0.05 indicated the existence of publication bias.ResultsA total of 10 studies were included in the article. The overall SIR estimates suggested an increased risk of overall cancer in IgG4-RD patients (SIR 2.57 95% CI 1.72-3.84) compared with the general population. The specific SIRs for pancreas and lymphoma were higher than those of the general population in IgG4-RD patients (SIR 4.07 95% CI 1.04-15.92, SIR 69.17 95% CI 3.91-1223.04, respectively). No significant associations were revealed in respiratory and gastric cancer (SIR 2.14 95% CI 0.97-4.75, SIR 0.95 95% CI 0.24-3.95, respectively). Four studies were found to be the major sources of heterogeneity by sensitivity analysis. There was no evidence of publication bias via Egger's test.ConclusionCompared with the general population, patients with IgG4-RD appear to have a higher risk of overall cancer, especially pancreatic and lymphoma. The risk of lung and gastric cancer was not different between IgG4-RD patients and the general population.
| S-EPMC8728936 | biostudies-literature
Project description:Non-small cell lung cancer is the predominant type of lung cancer, resulting in high mortality worldwide. Digoxin, a cardiac glycoside, has recently been suggested to be a novel chemotherapeutic agent. Src is an oncogene that plays an important role in cancer progression and is therefore a potential target for cancer therapy. Here, we investigated whether digoxin could suppress lung cancer progression through the inhibition of Src activity. The effects of digoxin on lung cancer cell functions were investigated using colony formation, migration and invasion assays. Western blotting and qPCR assays were used to analyze the mRNA and protein expression levels of Src and its downstream proteins, and a cell viability assay was used to measure cellular cytotoxicity effects. The results of the cell function assays revealed that digoxin inhibited the proliferation, invasion, migration, and colony formation of A549 lung cancer cells. Similar effects of digoxin were also observed in other lung cancer cell lines. Furthermore, we found that digoxin significantly suppressed Src activity and its protein expression in a dose- and time-dependent manner as well as reduced EGFR and STAT3 activity. Our data suggest that digoxin is a potential anticancer agent that may suppress lung cancer progression through inhibiting Src and the activity of related proteins.
| S-EPMC4425490 | biostudies-literature
Project description:Thrombocytopenia-associated multiple organ failure (TAMOF) is a clinical phenotype that encompasses a spectrum of syndromes associated with disseminated microvascular thromboses, such as the thrombotic microangiopathies thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS) and disseminated intravascular coagulation (DIC). Autopsies findings in TTP, HUS, or DIC reveal specific findings that can differentiate these 3 entities. Von Willebrand factor and ADAMTS-13 play a central role in TTP. Shiga toxins and the complement pathway are vital in the development of HUS. Tissue factor is the major protease that drives the pathology of DIC. Acute kidney injury (AKI) is a common feature in patients with TAMOF.
| S-EPMC5281060 | biostudies-literature