A Case of Coronary Arteritis and Myocardial Involvement With Associated IgG4-Related Disease
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SUBMITTER: Suwa K
PROVIDER: S-EPMC10107001 | biostudies-literature | 2023 Apr
REPOSITORIES: biostudies-literature
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Project description: Not available
| S-EPMC9071294 | biostudies-literature
Project description:BackgroundIgG4-related disease (IgG4-RD) is an inflammation-mediated autoimmune disease characterized by infiltration of IgG4 plasma cells in target organs, storiform fibrosis and obliterative phlebitis, accompanied by or without elevated serum IgG4 concentrations. Multiple sites can be involved, including large vessels. Coronary and pulmonary arteries are less involved, while simultaneous involvement of coronary and pulmonary arteries is less reported. This case is unique in terms of simultaneous involvement of coronary and pulmonary arteries in a female patient with possible IgG4-RD and the first review of relevant domestic literature.Case descriptionThis case is a middle-aged female patient with both coronary artery and pulmonary artery involvement, with cardiac insufficiency as the main manifestation. Cardiac ultrasound revealed the cardiac insufficiency and abnormal wrapping of multiple arteries. Imaging examinations including coronary artery computed tomography angiography (CTA), pulmonary artery CTA and cardiac magnetic resonance imaging (MRI) further confirmed the lesions of the left main coronary artery, anterior descending branch, circumflex branch and pulmonary artery. Then the patient was diagnosed with possible IgG4-RD. After glucocorticoid treatment, the patient's clinical symptoms and cardiac function improved, and her serum IgG4 levels decreased.ConclusionsWhen the arterial system is involved in IgG4 disease, it is known as IgG4-related artery disease. Combined with the case of this patient, this paper reviewed the literature on IgG4-related artery disease, and searched and summarized the related domestic literature on coronary/pulmonary artery disease to improve people's understanding of IgG4-related artery disease.
| S-EPMC10753237 | biostudies-literature
Project description: Not available
| S-EPMC11195725 | 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:BackgroundImmunoglobulin G4 (IgG4)-related diseases are systemic fibroinflammatory disease characterized by extensive infiltration of IgG4-positive plasma cells in the affected tissue(s), with high plasma levels of IgG4. However, coronary involvement is rare.Case summaryA 70-year-old man was diagnosed with IgG4-related coronary arteritis, pancreatitis, and cholangitis during full-body contrast computed tomography (CT) examination prior to surgery for an iliac artery aneurysm. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/CT showed increased uptake of 18F-FDG in the pancreas, extrahepatic bile ducts, and proximal right coronary artery (RCA). Despite the patient being asymptomatic, the RCA showed severe stenosis. The patient was administered a conservative treatment with prednisolone, 30 mg/day, gradually tapered to 5 mg/day, for 6 months. Two years later, contrast CT showed improvement of the pancreatic and bile duct lesions; however, the steroid therapy had not improved the coronary artery lesions, and gradual progression of the lesions was observed. Percutaneous coronary intervention was performed with a cutting balloon in the RCA, and good patency was maintained for 1 year after the procedure.DiscussionSteroid therapy is the first-line treatment for IgG4-related diseases; however, there may be some refractory cases. The stenotic and aneurysmal types of IgG4-related coronary arteritis are life-threatening; therefore, we performed revascularization using balloon angioplasty. Determining the optimal revascularization technique for drug-refractory cases requires further investigation.
| S-EPMC11635450 | 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:BackgroundThe present article describes two cases of patients with coronary arteritis (CA) whose identification of CA diagnosis (late vs. early) resulted in different clinical courses and outcomes.Case summaryCase 1 is a 53-year-old woman with multiple coronary risk factors who was admitted with acute coronary syndrome (ACS) and significant stenosis in the left main trunk (LMT). Although clues suggested arteritis (LMT lesion without any other stenosis, occlusion of left internal thoracic artery, etc.), the diagnosis of CA (coronary involvement of unclassified arteritis) was delayed and revascularization, including coronary artery bypass grafting (CABG) and percutaneous coronary intervention (PCI), was performed under uncontrolled inflammatory status. As a result, Case 1 experienced repeated ACS episodes due to graft failure and in-stent restenosis, and repeatedly underwent PCI. Case 2 is a 76-year-old woman with no significant coronary risk factors who was admitted with ACS. This patient was successfully diagnosed with coronary involvement of Takayasu arteritis before revascularization. Coronary artery bypass grafting was performed after stabilizing inflammation with prednisolone, and the patient remains angina-free beyond 1-year post-CABG. In both cases, intravascular imaging clearly identified the localization and degree of inflammation related to CA by demonstrating specific findings (ambiguous typical three-layer structure of arterial wall and extended low-echoic areas within adventitia).DiscussionAccurate and early diagnosis with meticulous diagnostic and therapeutic strategies appear to be important for favourable clinical outcomes in the medical treatment of patients with coronary involvement of arteritis. Intravascular imaging has the potential to contribute to optimizing clinical management of CA.
| S-EPMC7180521 | biostudies-literature
Project description:Background: IgG4-related disease is a rare, chronic inflammatory disorder characterized by lymphoplasmacytic infiltration, 'storiform' fibrosis, and elevated IgG4 levels in affected tissues. This disease has a broad and heterogeneous clinical spectrum that includes four main phenotypes: pancreatic-hepatobiliary disease, retroperitoneal/aortic fibrosis, head and neck disease, and Mikulicz syndrome. Case Description: An 85-year-old male patient with a clinical presentation, which is unusual outside Asia, of IgG4-related disease phenotype Mikulicz syndrome, characterized by bilateral dacryoadenitis, orbital pseudotumor, and no evidence of significant systemic participation. Despite extensive involvement in the orbital and glandular region, the patient did not develop serious organ complications, a behavior rarely documented in the literature. Despite the serum IgG4 levels being normal (<135 mg/dL), the clinical and radiological picture suggested IgG4-RD, emphasizing the need for a biopsy for a definitive diagnosis. Histopathological examination revealed a dense lymphoplasmacytic infiltrate, storiform fibrosis, and more than 40% IgG4-positive cells, confirming the diagnosis. Results: Treatment with prednisone was initiated alongside azathioprine for long-term control. Calcium and vitamin D3 supplementation were added to prevent glucocorticoid-induced osteoporosis. Remarkable clinical improvement was observed within 24 h, with progressive orbital and glandular symptoms resolution. Over a year, the patient exhibited complete resolution of the orbital tumors, total recovery of vision, and no relapses. The only sequelae observed were dry eye. Conclusions: This case highlights the need to consider IgG4-RD with normal serum IgG4 levels, the importance of histopathology for diagnosis, and the efficacy of steroids as first-line treatment. A multidisciplinary approach is essential for timely treatment.
| S-EPMC11818687 | biostudies-literature
Project description:BackgroundThe heart can be involved in immunoglobulin (Ig)-G4-related disease (IgG4-RD). This study aimed to summarize the clinical features and efficacy of treatment for IgG4-RD patients with heart involvement.MethodsWe conducted a retrospective study enrolling 42 IgG4-RD patients with heart involvement from the IgG4-RD cohorts of the Peking Union Medical College Hospital and Beijing An Zhen Hospital, from 2010 to 2022. Clinical, laboratory, radiological data were collected, and treatment responses to glucocorticoids and immunosuppressants were analyzed.ResultsIgG4-related cardiac involvement is a rare part of the IgG4-RD spectrum. The incidences of coronary periarteritis and pericarditis were 1.2%(13/1075) and 3.1%(33/1075), respectively in our cohort. Valvular disease possibly related to IgG4-RD was detected in two patients. None of the patients with myocardial involvement were identified. The average age was 58.2 ± 12.8 years, with a male predominance (76.7%). Coronary artery CT revealed that mass-like and diffuse wall-thickening lesions were the most frequently observed type of coronary periarteritis. Pericarditis presented as pericardial effusion, localized thickening, calcification and mass. After treatment with glucocorticoid and immunosuppressants, all patients achieved a reduced IgG4-RD responder index score and achieved radiological remission. Two patients with coronary peri-arteritis experienced clinical relapses during the maintenance period.ConclusionsCardiac involvement in IgG4-RD is rare and easily overlooked since many patients are asymptomatic, and the diagnosis relies on imaging. Patients showed a satisfactory response to glucocorticoid based treatment.
| S-EPMC11247878 | biostudies-literature
Project description:IgG4-related disease-associated hypertrophic pachymeningitis (IgG4RD-HP) is a fibroinflammatory autoimmune disorder in which diagnosis is difficult without biopsy. Guidance on management of disease refractory to glucocorticoids and intravenous rituximab is limited. We present the case of a 68-year-old woman with IgG4RD-HP who developed sensorineural hearing loss with associated bulky basilar pachymeningeal enhancement. Her cerebrospinal fluid was inflammatory and had an elevated IgG4 concentration, strongly suggestive of IgG4RD-HP. Biopsy of involved meninges was not possible due to surgical risk. Over years she developed bilateral optic neuropathies and hydrocephalus, requiring intravenous rituximab and ventriculoperitoneal shunt. Her disease was refractory to glucocorticoids. Despite maintenance intravenous rituximab, she developed slowly progressive symptoms of intracranial hypertension and hydrocephalus with persistently inflammatory spinal fluid. Switching to intrathecal rituximab therapy led to dramatic improvement in gait and headache and reduced pachymeningeal bulk and metabolic activity. In patients with IgG4RD-HP refractory to glucocorticoids and intravenous rituximab, intrathecal rituximab may be an efficacious therapy.
| S-EPMC10244657 | biostudies-literature