IgG4-related Disease with a Cardiac Mass Causing Cerebral Infarction

Nomura, Shun; Ishii, Wataru; Hara, Ryosuke; Shigeki, Nanasawa; Nishiwaki, Kei; Kagoshima, Mitsuru; Tamaki, Toru; Satomi, Hidetoshi; Usui, Tatsuya

doi:10.2169/internalmedicine.8049-21

Cited by 4 publications

(4 citation statements)

References 32 publications

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“…After conducting a thorough literature review, it was found that most cases of IgG4RD manifest as a tumor-like lesion surrounding the coronary arteries, within the right atrium, or around the valves, particularly the aortic or pulmonary valves. Nomura et al 17 reported a case of a 58-year-old male with cerebral infarction and atrial fibrillation. Computed tomography images of his heart revealed a mass in the right atrium and right atrioventricular groove, and histopathological analysis showed plasma cells and fibrosis with an IgG4 : IgG ratio of 72.3%.…”

Section: Discussionmentioning

confidence: 99%

“…Our case represents the first reported instance of an isolated cardiac IgG4 lesion in a known primary APS patient. Table 1 17 - 24 provides a detailed review of right atrial mass in IgG4RD. Macrophages in the valves or heart myocardium can be stimulated by pathogen-associated molecular patterns or damage-associated molecular patterns in the blood, leading to an abnormal immune response in IgG4-RD.…”

Section: Discussionmentioning

confidence: 99%

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Immunoglobulin G4-Related Lesions in Autoimmune Diseases: Unusual Presentations at Atypical Sites—A Tale of 2 Cases with Literature Review

2023

Eur J Rheumatol

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Immunoglobulin G4-related disease (IgG4-RD) coexisting with clinically apparent autoimmune diseases, such as rheumatoid arthritis (RA) or antiphospholipid syndrome (APS), is a rarely documented combination in the scientific literature. In this case-based review, we present 2 intriguing cases with preexisting autoimmune diseases, namely, RA and primary APS, who exhibited coexistent IgG4-related lesions at unusual sites. The first case pertains to a patient with known RA who presented with an encasing mass in the esophagus leading to stricture, with histopathological diagnosis of IgG4-RD.The second patient, diagnosed with primary APS, experienced breathlessness, and imaging revealed a right atrial mass. Histopathological examination of the mass confirmed IgG4-RD. Notably, both patients demonstrated significant clinical improvement upon initiation of steroid therapy. Rheumatoid arthritis patients commonly exhibit elevated levels of IgG4 in their sera; however, RA with coexisting IgG4-RD is rarely reported in the literature. Similarly, APS with IgG4-related lesions is exceedingly rare. Although there are few case reports and series on esophageal and cardiac IgG4-RD, the occurrence of such unusual location of IgG4-related lesions in the context of known autoimmunity is presented here for the first time.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-Related Lesions in Autoimmune Diseases: Unusual Presentations at Atypical Sites—A Tale of 2 Cases with Literature Review

2023

Eur J Rheumatol

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“…A detailed literature review showed that most of the cases present as a tumoriform lesion surrounding the coronary arteries, inside the right atrium or around the valves ( mainly aortic or pulmonary valves ). Nomura et al (17) reported a case of a 58-yearold male presented with cerebral infarction and atrial brillation, his computer tomography image of the heart showed a mass in the right atrium and right atrioventricular groove, histopathology showed plasma cells and brosis with IgG4/IgG 72.3%, serum IgG4 level was elevated, hence the diagnosis of IgG4RD was con rmed. Hajsadeghi M.D.et al ( 18) reported a 45-year-old male presented with an in ltrating mass in the right atrioventricular groove which was also biopsy-proven IgG4RD.…”

Section: Unusual Location Of Igg4rdmentioning

confidence: 96%

Autoimmune diseases presenting with IgG4 related lesions at atypical sites - a tale of two cases with literature review

Baisya

Yerram

2023

Preprint

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Introduction IgG4RD overlapping with clinically overt autoimmune diseases (Rheumatoid arthritis ( RA) or anti-phospholipid syndrome (APS) is rarely reported in the literature. Here we report a tale of two cases with known autoimmune diseases – RA and primary APS presented with IgG4-related lesions involving unusual sites – oesophagus and right atrium of the heart respectively Case – The first case was a known RA presented with dysphagia, imaging suggestive of encasing mass in oesophagus causing stricture , biopsy was suggestive of IgG4RD. The second patient was a known case of primary APS presented with breathlessness, imaging revealed right atrial mass with histopathology was suggestive of IgG4RD . Both patients had significant improvement after starting steroid. Discussion – A few cases of known RA presenting with IgG4 mass is reported in literature though RA patients are frequently found to have increased number of IgG4 in their sera. Primary APS with IgG4 lesion is extremely uncommon. Though a several case reports or series are published regarding oesophageal and cardiac IgG4 related lesions, such lesions in the background of known autoimmunity is first described by us . Conclusion – This is the first case series of two cases with IgG4 related lesions in atypical site in known autoimmune diseases .

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“…Previous studies have described the involvement of various organs in IgG4-RD, including autoimmune pancreatitis, sclerosing cholangitis, sialadenitis, dacryoadenitis, interstitial nephritis, lymphadenopathy, retroperitoneal fibrosis, IgG4-related hypophysitis, and IgG4-related lung disease. According to the literature, there are various types of cardiovascular involvement in IgG4-RD, including aortitis, periaortitis, periarteritis [ 5 , 6 ], pericardial [ 7 , 8 ] cardiac valves [ 9 ] and myocardial involvement [ 10 ]. Cardiac involvement in IgG4-RD presents with various clinical manifestations, including chest pain, dyspnea, palpitations, and heart failure.…”

Section: Introductionmentioning

confidence: 99%

Coronary periarteritis and pericarditis are rare but distinct manifestations of heart involvement in IgG4-related disease: a retrospective cohort study

Hua,

Du,

Guo

et al. 2024

Orphanet J Rare Dis

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Background The 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. Methods We 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. Results IgG4-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. Conclusions Cardiac 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.

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IgG4-related Disease with a Cardiac Mass Causing Cerebral Infarction

Cited by 4 publications

References 32 publications

Immunoglobulin G4-Related Lesions in Autoimmune Diseases: Unusual Presentations at Atypical Sites—A Tale of 2 Cases with Literature Review

Immunoglobulin G4-Related Lesions in Autoimmune Diseases: Unusual Presentations at Atypical Sites—A Tale of 2 Cases with Literature Review

Autoimmune diseases presenting with IgG4 related lesions at atypical sites - a tale of two cases with literature review

Coronary periarteritis and pericarditis are rare but distinct manifestations of heart involvement in IgG4-related disease: a retrospective cohort study

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