scholarly journals P57 IGG4-related disease causing sudden cardiac death

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Richard Haigh ◽  
Lucy Smyth ◽  
Andrew Ludman
Keyword(s):  
Cardiovascular Disease ◽  
Coronary Artery ◽  
Sudden Cardiac Death ◽  
Coronary Angiography ◽  
Cardiac Death ◽  
Case Reports ◽  
Post Mortem ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Storiform Fibrosis

Abstract Background IgG4-related disease (IgG4-RD) is a chronic systemic inflammatory disorder presenting with mass lesions in various organs and showing plasma cell infiltration, storiform fibrosis and obliterative phlebitis on histology. IgG4-RD has previously been identified as a rare cause of sudden cardiac death (SCD) in case reports, but in settings where pathology has been unexpectedly found on post-mortem examination. To our knowledge, there are no published case reports of SCD in patients during treatment for IgG4-RD. Methods A 43-year old chef with a 15 pack-year smoking history presented with portal vein thrombosis, widespread lymphadenopathy, proptosis and inflammatory lung changes. He was found to have IgG4-RD on submandibular lymph node biopsy with storiform fibrosis and abundance of IgG4-positive plasma cells. Test showed a high IgG and low complement levels and a persistent eosinophilia. He was anticoagulated with warfarin for the HPV thrombosis. Treatment with steroids (initially prednisolone 40mg/day) was initially successful and he continued at work and kept fit in the gym. There was also a good biochemical and radiological response to steroids, with normalisation of eosinophil count, IgG level, complements and CRP along with regression of imaging changes. After a period of 12 months steroid weaning (prednisolone 5mg) and having been well at OPD review, he was unexpectedly found dead at home. Post-mortem examination identified IgG4-RD coronary pan-arteritis as the cause of death. The finding of this unanticipated severe complication raises new questions about surveillance and prevention. Results Coronary involvement in IgG4-RD is thought to be rare but may reflect difficulty obtaining coronary artery tissue and clinically silent disease remaining undiagnosed. A systematic review of IgG4-RD outcomes (7 studies comprising 294 patients, follow-up mean of 29 months) identified 26 deaths (8.8%). One was from an aneurysm and four from other cardiovascular disease, while most deaths were from disease progression or cancer. Case reports describe IgG4-RD coronary artery disease pathologies, including inflammatory pseudotumour, periarterial thickening, coronary artery aneurysm and coronary artery stenosis. Conclusion This case raises important questions about identifying and monitoring for asymptomatic cardiovascular involvement in IgG4-RD. Steroid treatment may be less effective for coronary involvement, and in theory, could precipitate rupture or dilatation of the arteries. Recent IgG4-RD management guidelines have not addressed surveillance for cardiovascular disease. ECG and echocardiography are reasonable initial surveillance options, but we suggest clinicians have a low threshold for CT coronary angiography and/or cardiac MR. Invasive coronary angiography and CT-PET can then be used to provide additional information if required. We noted a normal CRP despite active coronary inflammation in our patient and therefore look forward to improved biomarkers for disease activity in IgG4-RD. Disclosures R. Haigh None. L. Smyth None. A. Ludman None.

scholarly journals Sudden Cardiac Death Due to Coronary Artery Involvement by IgG4-Related Disease: A Rare, Serious Complication of a Rare Disease

2014 ◽  
Vol 138 (6) ◽  
pp. 833-836 ◽  
Author(s):  
Nimesh R. Patel ◽  
Mary L. Anzalone ◽  
L. Maximilian Buja ◽  
M. Tarek Elghetany
Keyword(s):  
Sudden Cardiac Death ◽  
Coronary Arteries ◽  
Cardiac Death ◽  
Plasma Cells ◽  
Severe Stenosis ◽  
Immunoglobulin G4 ◽  
Emergency Center ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Systemic Disorder

Immunoglobulin G4–related disease (IgG4-RD) is a systemic disorder characterized by multiorgan fibrosis with IgG4-producing plasma cells, increased IgG4 serum concentration, and responsiveness to steroid therapy. Involvement of the pancreas, salivary glands, orbit, aorta, and other sites has been well documented in the literature; however, there have been limited reports of cases involving the coronary arteries. We report the case of a 53-year-old Hispanic man who was brought to the emergency center and diagnosed with sudden cardiac death. Autopsy was subsequently performed, revealing multiorgan involvement by IgG4-RD, including involvement of the coronary arteries. The inflammation and fibrosis, in combination with concomitant atherosclerotic disease, resulted in severe stenosis of the coronary arteries. Two of the coronary arteries were further occluded by thrombosis. These factors led to cardiac hypoperfusion, myocardial infarction and, ultimately, sudden cardiac death. Fatal involvement of the coronary arteries has not been previously reported, raising a new concern for a severe complication of IgG4-RD.

9 Vulnerable plaque detection in sudden cardiac death: post-mortem CT coronary angiography

2018 ◽  
Author(s):  
Alastair J Moss ◽  
Philip D Adamson ◽  
Jack PM Andrews ◽  
David E Newby ◽  
Ralph Bouhaidar ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Coronary Angiography ◽  
Vulnerable Plaque ◽  
Cardiac Death ◽  
Ct Coronary Angiography ◽  
Post Mortem ◽  
Plaque Detection

scholarly journals Sudden Cardiac Death due to Coronary Artery Vasculitis in a Patient with Relapsing Polychondritis

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Heather Bukiri ◽  
Steven M. Ruhoy ◽  
Jane H. Buckner
Keyword(s):  
Coronary Artery ◽  
Sudden Cardiac Death ◽  
Cardiac Disease ◽  
Cardiac Death ◽  
Plasma Cells ◽  
Clinical Manifestations ◽  
Relapsing Polychondritis ◽  
Tracheobronchial Tree ◽  
Systemic Autoimmune Disease ◽  
Igg4 Related Disease

Relapsing polychondritis (RP) is a systemic autoimmune disease characterized by relapsing and remitting inflammation of the cartilaginous structures of the ears, nose, tracheobronchial tree, and joints. Diagnosis is challenging due to the heterogeneity of clinical manifestations, the relapsing and remitting nature of the disease, the presence of coexistent diseases in at least one-third of patients, and the lack of a diagnostic blood test. Although RP-associated cardiac disease is the second most common cause of death behind tracheobronchial complications, coronary artery vasculitis is rare. This report describes a case of sudden cardiac death due to vasculitis affecting the coronary arteries in a patient with RP. The pathologic findings included obliterative coronary arteritis with plasma cells and storiform fibrosis, features suggesting that IgG4-related disease (IgG4-RD) may have contributed to the patient’s cardiac disease. The literature on vasculitis and cardiac disease in RP and the possible role of IgG4-RD in this setting is also reviewed. The primary take-home message from this case report is the importance of frequent screening for cardiac disease, regardless of symptoms, in patients with RP. In addition, considering the diagnosis of IgG4-RD in some cases thought to be RP may also be warranted.

scholarly journals 462 Accidental detection of an anomalous origin of the coronary arteries in a patient with STEMI: a case report

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Marialucia Milite ◽  
Roberto Celotto ◽  
Angela Sanseviero ◽  
Dalgisio Lecis ◽  
Daniela Benedetto ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Sudden Cardiac Death ◽  
Coronary Angiography ◽  
Contrast Agents ◽  
Coronary Arteries ◽  
Coronary Sinus ◽  
Cardiac Death ◽  
Anomalous Origin ◽  
Left Main ◽  
The Right

Abstract Coronary artery anomalies and variants are relatively uncommon congenital disorders of the coronary artery anatomy and constitute the second most common cause of sudden cardiac death in young competitive athletes. Normally there are two main coronary arteries, which stem from the sinuses of Valsalva and descend towards the cardiac apex. The most frequent anomalous origin of the coronary arteries associated with sudden cardiac death is the anomalous origin of a coronary artery from the contralateral sinus, particularly if the anomalous coronary artery has a course between the aorta and the pulmonary artery. The diagnosis of a coronary artery anomaly is insidious since patients are usually asymptomatic. Indeed, in most of the cases, coronary anomalies are discovered incidentally during coronary angiography or on autopsy following sudden cardiac death. However, in some cases, symptoms like angina, syncope, heart failure, and myocardial infarction may occur. We want to describe the case of a 54 years old man who went to another hospital of our district with chest pain. The EKG showed an infero-lateral STEMI. The patient underwent systemic thrombolysis complicated by arrhythmic storm, treated with effective DC shock, then he was urgently transferred to our Hospital to perform a PCI rescue. The coronary angiography showed a critical stenosis of the right coronary artery, treated with the implantation of a zotarolimus-eluting coronary stent system. Surprisingly, an abnormal origin of the anterior interventricular artery and circumflex artery from seperated hosts in the right coronary sinus was detected. A computed tomography showed the separated origin of the right coronary artery and the left main from the right coronary sinus; moreover, the left main presented a retroaortic course between the bulb and the left atrium; the left anterior intraventricular artery run anterior to the aortic root. During hospitalization, a diagnosis of diabetes was made with consequent setting of adequate hypoglycemic therapy. Serial echocardiograms showed an improval in the ejection fraction (from 35–40% to 50%). The patient was discharged in well clinical conditions and 1 month later at the follow-up, during an outpatient visit, he was fully asymptomatic. The dynamic EKG according to Holter showed rare isolated ventricular beats; laboratory tests were normal. The anomalous origin of a coronary ostium from the contralateral sinus is the most frequently anomaly which is associated with sudden cardiac death, in particular when the left coronary artery origins from the right sinus. The rapid advancement of the imaging techniques, including CT, CMR, intravascular ultrasound, and optical coherence tomography, have provided us with a wealth of new information on this subject. Coronary artery CT offers the best performance in terms of spatial resolution, acquisition time, and image contrast but the use is limited due the dose of ionizing radiation and the use of contrast agents, in particular taking into account that most patients are young. CMR is capable of visualizing the origin of the coronary arteries non-invasively, without use of ionizing radiation and contrast agents. Currently however, due to spatial resolution, the capability of CMR to visualize smaller coronary branches is still a limiting factor preventing full assessment of coronary arteries using this modality. Developments and more widespread access to advanced cardiac imaging will undoubtedly lead to earlier diagnoses. The taxonomies of the anomalous origin of coronary arteries are inconsistent and complex and so we want to make our contribution to the register of cases of abnormal origin of coronary arteries for the risk of sudden cardiac death and ischaemic events related to.

Sudden Cardiac Death Due To IgG4-Related Disease

2015 ◽  
Vol 139 (5) ◽  
pp. 571-571 ◽  
Author(s):  
Nobukazu Ishizaka ◽  
Jun Tanigawa ◽  
Shuji Suzuki
Keyword(s):  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals Left Main and Right Coronary Artery Arising from Single Coronary Trunk: A Very Rare Cardiac Anomaly

2020 ◽  
pp. 1-4
Author(s):  
Aditya Gupta ◽  
Rohit Rai ◽  
Shakil Shaikh ◽  
Kalyan Munde ◽  
Narendra O. Bansal
Keyword(s):  
Coronary Artery ◽  
Sudden Cardiac Death ◽  
Coronary Angiography ◽  
Cardiac Death ◽  
Anomalous Origin ◽  
Cardiac Anomaly ◽  
Single Coronary Artery ◽  
Rare Anomaly ◽  
One Year ◽  
Rule Out

Aim: Single coronary artery is a rare anomaly. Patients may present with syncope, angina or rarely sudden cardiac death. Presentation of Case: Patient presented with dyspnea on exertion for one year. On 2D Echo patient had small restrictive VSD. On coronary angiography single coronary artery from right cusp was seen. On CT angiography anomalous origin was confirmed. There was no malignant course of coronary artery. Discussion: Anomalous origin of coronary arteries are rare cardiac anomalies. It can present as syncope, angina, or sudden cardiac death especially in patients with malignant course of coronary artery passing between aorta and pulmonary artery. Hence CT coronary angiography is useful to rule out the malignant course. In our patient, there was no malignant course hence managed conservatively. Conclusion: Anomalous origin of coronary artery is a rare anomaly and requires tailored treatment according to type and course of artery.

Effect of Hypertension and Cardiac Hypertrophy on Coronary Artery Morphology in Sudden Cardiac Death

Circulation ◽  
1996 ◽  
Vol 94 (12) ◽  
pp. 3138-3145 ◽  
Author(s):  
Allen P. Burke ◽  
Andrew Farb ◽  
You-hui Liang ◽  
John Smialek ◽  
Renu Virmani
Keyword(s):  
Coronary Artery ◽  
Sudden Cardiac Death ◽  
Cardiac Hypertrophy ◽  
Cardiac Death

scholarly journals Comparison of variant detection rate in genes between two cohorts of Czech living patients versus victims of sudden cardiac death with clinical / post mortem diagnosis of non-ischemic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
E Borisincova ◽  
P Votypka ◽  
K Rucklova ◽  
A Pilin ◽  
M Kulvajtova ◽  
...  
Keyword(s):  
At Risk ◽  
Czech Republic ◽  
Primary Prevention ◽  
Sudden Cardiac Death ◽  
Genetic Counselling ◽  
Cardiac Death ◽  
Detection Rate ◽  
Funding Source ◽  
Post Mortem ◽  
Custom Made

Abstract Introduction Hereditary cardiomyopathy is associated with an increased risk of ventricular arrhythmia and sudden cardiac death (SCD). Genetic stratification substantiates risk assessment and enables the primary prevention of SCD in relatives at risk. We have analyzed the genetic aetiology of SCD in a representative Czech cohort with post mortem diagnosis of various forms of cardiomyopathy and compared it to living cases with these cardiac disorders. Patients and methods Between 2018 and 2019, altogether 47 victims of SCD with post mortem diagnosis of hypertrophic- (HCM; 18/47), arrhythmogenic- (ACM; 19/47) and dilated cardiomyopathy (DCM; 10/47) were identified. Concurrently, genetic testing was performed in 114 living patients (HCM 54/114, ACM 22/114, DCM 38/114). Genetic counselling and cardiologic examination had been carried out in first-degree relatives in all patients/SCD victims. Massively parallel sequencing (MiSeq platform; Illumina.com) was utilized for a custom-made panel comprising 100 candidate genes (Sophia Genetics, Switzerland). The presence of pathogenic variants was validated by Sanger DNA sequencing and through family segregation analyses. Results The causative detection rate (according to ACMG.net classes 4 or 5) in SCD victims with DCM was 60% (6/10) and in living patients with DCM 47.4% (18/38). Variants in TTN, RBM20, DES and FLNC (mainly truncating variants) prevailed in both groups. The detection rate in ACM was 5% (1/19 in SCN5A gene) in SCD victims and 31.8% (7/22) in living patients. Interestingly, the most prevalent mutated gene PKP2 in living patients was not detected in SCD victims. The detection rate in SCD victims with post mortem diagnosis of HCM was 16% (3/18) and in living patients 35% (19/54). The most prevalent gene was MYBPC3 in both groups, while PRKAG2 was detected in one SCD victim and in one living case who survived cardiac arrest. Conclusion Post-mortem genetic analysis in DCM yields a high detection rate and allows potentially effective primary prevention of SCD in relatives at risk. In contrast, the molecular autopsy of HCM and ACM renders a much lower yield which is below the mutation detection rate in living phenotype positive individuals. The results help to improve the genetic counselling in affected families in Czech Republic. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Ministry of Health of the Czech Republic

scholarly journals Needle biopsy compared with surgical biopsy: pitfalls of small biopsy in histologial diagnosis of IgG4-related disease

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Yanying Liu ◽  
Fei Yang ◽  
Xiying Chi ◽  
Yuxin Zhang ◽  
Jiangnan Fu ◽  
...  
Keyword(s):  
Needle Biopsy ◽  
Plasma Cells ◽  
Classification Criteria ◽  
Histological Diagnosis ◽  
Obliterative Phlebitis ◽  
Surgical Biopsy ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Significant Difference ◽  
Storiform Fibrosis

Abstract Objective The growing utilization of needle biopsy has challenged the current pathology consensus of IgG4-related disease (IgG4-RD). The aims of this study were to identify the histological characteristics of needle biopsy and surgical specimens and evaluate the ability of needle biopsy in histological diagnosis of IgG4-RD. Methods Biopsies from patients who were referred to as IgG4-RD by the 2019 ACR/EULAR IgG4-RD classification criteria in Peking University People’s Hospital from 2012 to 2019 were re-evaluated. Typical histological features and diagnostic categories were compared between needle biopsy and surgical biopsy. Results In total, 69 patients met the 2019 ACR/EULAR classification criteria and 72 biopsies of them were re-evaluated. All cases showed lymphoplasmacytic infiltrate, while storiform fibrosis and obliterative phlebitis were only present in 35 (48.6%) and 23 (31.9%) specimens, respectively. Storiform fibrosis was more likely to be seen in retroperitoneum lesion (P = 0.033). Surgical biopsy showed significantly higher IgG4+ plasma cells/high-power field (IgG4/HPF) count (P < 0.01) and higher proportion of IgG4/HPF > 10 (P < 0.01). No significant difference was observed with regard to the ratio of IgG4+ plasma cells/IgG+ plasma cells (IgG4/IgG) (P = 0.399), storiform fibrosis (P = 0.739), and obliterative phletibis (P = 0.153). According to the 2011 comprehensive diagnostic criteria, patients who performed a needle biopsy were less likely to be probable IgG4-RD (P = 0.045). Based on the 2011 pathology consensus, needle biopsy was less likely to be diagnosed as IgG4-RD (P < 0.01), especially to be highly suggestive IgG4-RD (P < 0.01). Only 1/18 (5.6%) needle salivary specimens fulfilled the cutoff of IgG4/HPF > 100, which was significantly less than 15/23 (65.2%) of surgical ones (P < 0.01). Conclusions Needle biopsy shows an inferiority in detecting IgG4/HPF count but not in IgG4/IgG ratio, storiform fibrosis, and obliterative phlebitis. Compared with surgical samples, needle biopsy is less likely to obtain a histological diagnosis of IgG4-RD. A different IgG4/HPF threshold for needle biopsy of the salivary glands may be considered.

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