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

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.

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

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2012-0614-cr ◽

2014 ◽

Vol 138 (6) ◽

pp. 833-836 ◽

Cited By ~ 31

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.

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P57 IGG4-related disease causing sudden cardiac death

Rheumatology ◽

10.1093/rheumatology/keaa111.056 ◽

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.

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Isolated Single Right Coronary Artery in a Pediatric Patient

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135119842872 ◽

2019 ◽

Vol 10 (4) ◽

pp. 515-517

Author(s):

Justin H. Berger ◽

Jonathan J. Rome ◽

Julie A. Brothers

Keyword(s):

Coronary Artery ◽

Sudden Cardiac Death ◽

Pediatric Patient ◽

Coronary Arteries ◽

Right Coronary Artery ◽

Cardiac Disease ◽

Cardiac Death ◽

Adult Onset ◽

First Case ◽

Single Right Coronary Artery

Anatomic single coronary arteries are quite rare but can be a potential cause of sudden cardiac death. We present the first case of a pediatric patient with an anatomic single right coronary artery (Lipton type R1 or Shirani-Roberts type IIA), of a type which has been associated with adult-onset ischemic cardiac disease. We review the evaluation and management of this young patient.

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Postmortem Specificity of Troponin for Acute Miocard Infarction Diagnosis throug Qualitative Dosing from Pericardial Fluid

Revista de Chimie ◽

10.37358/rc.18.9.6559 ◽

2018 ◽

Vol 69 (9) ◽

pp. 2482-2486

Author(s):

Iuliana Hunea ◽

Simona Irina Damian ◽

Carmen Corina Radu ◽

Sorin Moldoveanu ◽

Tatiana Iov

Keyword(s):

Sudden Cardiac Death ◽

Cardiac Disease ◽

Cardiac Death ◽

Troponin I ◽

Troponin T ◽

Morphological Changes ◽

Pericardial Fluid ◽

Histological Changes ◽

Lethal Arrhythmia ◽

Myocardial Lesions

Cardiac disease is the leading cause of death, and sudden cardiac death occupies the first place in sudden deaths of natural causes. Sudden cardiac death due to lethal arrhythmia may be the first manifestation of a cardiac disease, such cases becoming suspect dead, thus forensic cases. The autopsy performed in such cases may reveal important cardiovascular disease but not obvious macroscopic or histological changes of acute myocardial infarction (IMA), except for cases of survival for several hours after the onset of the symptomatology. Biochemical markers were used to test for myocardial lesions in the absence of morphological changes. Methods for determining myoglobin, CK-MB, troponin T (cTn T), troponin I (cTn I) were introduced to the clinic to diagnose the condition of patients with chest pain as early as the 1990s. The lack of pathognomonic elements in corps investigations, where part of the analysis cannot be carried out, requires verification of the value of the investigations that can be carried out, with reference to the biochemical in the present case, in establishing the diagnosis with certainty.

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