IgG4-related disease as a variable-vessel vasculitis: A case series of 13 patients with medium-sized coronary artery involvement

Katz, Guy; Hedgire, Sandeep H; Stone, James R.; Perez-Espina, Sebastian; Fernandes, Ana D.; Perugino, Cory A.; Wallace, Zachary S; Stone, John H.

doi:10.1016/j.semarthrit.2023.152184

Cited by 17 publications

(2 citation statements)

References 39 publications

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“…However, the symptoms are relatively serious, and can even lead to sudden cardiac death ( 20 ), and the prognosis is poor. There are relatively few clinical reports on IgG4 involvement of the pulmonary artery, most of which are manifested as a peri-pulmonary artery mass, pulmonary artery stenosis and/or dilatation, which can lead to pulmonary hypertension in severe cases, including dyspnea after activity, cough, wheezing and other symptoms, and these symptoms need to be differentiated from pulmonary embolism or malignant tumor clinically ( 21 , 22 ).…”

Section: Discussionmentioning

confidence: 99%

IgG4-related disease involving coronary and pulmonary arteries: a case report and literature review

Cao,

Yang

2023

Cardiovasc Diagn Ther

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Background IgG4-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 Description This 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. Conclusions When 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.

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Section: Discussionmentioning

confidence: 99%

IgG4-related disease involving coronary and pulmonary arteries: a case report and literature review

Cao,

Yang

2023

Cardiovasc Diagn Ther

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“…In one series, 58% of patients had damage in at least one organ at diagnosis [ 99 ]. Examples of damage include sicca syndrome from resection of a salivary gland, proptosis from a fibrotic orbital pseudotumour, anosmia from sinonasal involvement [ 100–102 ], large vessel aneurysms or dissections [ 103 , 104 ], and chronic ureteral obstruction or tubulointerstitial nephritis leading to chronic kidney disease, including end-stage kidney disease [ 99 , 105–107 ].…”

Section: Monitoring Disease Activity and Assessing Damage In Igg4-rdmentioning

confidence: 99%

Current and future advances in practice: IgG4-related disease

Wallace,

Katz,

Hernandez-Barco

et al. 2024

Rheumatology Advances in Practice

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IgG4-related disease (IgG4-RD) is an increasingly recognized cause of fibroinflammatory lesions in patients of diverse racial and ethnic backgrounds and is associated with an increased risk of death. The aetiology of IgG4-RD is incompletely understood, but evidence to date suggests that B and T cells are important players in pathogenesis, both of which are key targets of ongoing drug development programmes. The diagnosis of IgG4-RD requires clinicopathological correlation because there is no highly specific or sensitive test. Glucocorticoids are highly effective, but their use is limited by toxicity, highlighting the need for studies investigating the efficacy of glucocorticoid-sparing agents. B cell-targeted therapies, particularly rituximab, have demonstrated benefit, but no randomized clinical trials have evaluated their efficacy. If untreated or under-treated, IgG4-RD can cause irreversible organ damage, hence close monitoring and consideration for long-term immunosuppression is warranted in certain cases.

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A rare pathology that mimics lung cancer: IgG4‐related vasculitis

Sener,

Alpaydın,

Kılınc

2024

Respirology Case Reports

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Immunoglobulin‐G4 (IgG4)‐related disease is essentially a fibro‐inflammatory disease that can affect any organ simultaneously or at different times. The disease usually presents with organ growth that mimics a tumour and can affect the lacrimal glands, major salivary glands, pancreas, bile ducts, retroperitoneal area, lungs, kidneys, aorta, meninges and thyroid gland. The immunopathogenesis behind this new disease has not yet been elucidated. Histopathological distinguishing features of the disease include dense lymphoplasmocytic infiltrates dominated by IgG4 positive plasma cells, storiform fibrosis and obliterative phlebitis. The likelihood of developing with immunoglobulin G4 (IgG4‐RD) is a recently identified rare systemic fibroinflammatory disease with an estimated incidence of less than 1 in 100,000 people worldwide. We present our case, which was diagnosed with IGG4‐related vasculitis by lung fine needle aspiration biopsy, which is very rare in the literature.

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IgG4-related disease as a variable-vessel vasculitis: A case series of 13 patients with medium-sized coronary artery involvement

Cited by 17 publications

References 39 publications

IgG4-related disease involving coronary and pulmonary arteries: a case report and literature review

IgG4-related disease involving coronary and pulmonary arteries: a case report and literature review

Current and future advances in practice: IgG4-related disease

A rare pathology that mimics lung cancer: IgG4‐related vasculitis

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