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Immunoglobulin G4–related coronary periarteritis: a systematic literature review with a case series

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A Correction to this article was published on 11 August 2022

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Abstract

We aimed to assess the clinical and radiological characteristics of immunoglobulin G4–related coronary periarteritis through a systematic literature review and from our case series. In the systematic literature review, we assessed English language manuscripts on immunoglobulin G4–related coronary periarteritis cases. Additionally, we identified patients with immunoglobulin G4–related coronary periarteritis at St. Luke’s International Hospital in Tokyo, Japan, from 2014 to 2020. We summarized patients’ demographics, immunoglobulin-G and -G4 titers, site and morphological features of the coronary lesion, and other organ involvements. We identified 38 cases from the literature and four patients from our institute. Coronary lesions were detected using coronary computed tomography in 40 (95.2%) patients. Mass-like or diffuse wall–thickening lesion was the most frequently observed type in 33 (78.6%) patients. No trends at the site of the coronary arteries were identified. Overall, 32 (76.1%) patients had multiple-organ involvement, of which the most common lesion was peri-aortitis in 21 (50.0%) patients. Ten (23.8%) patients with an isolated coronary lesion had significantly lower immunoglobulin-G4 titers than those with other organ involvements (immunoglobulin-G4: 261 [161.0, 564.0] vs. 1355.0 [320.8, 2480.0] mg/dL, p = 0.033). The wall-thickening lesions responded well to immunosuppressive treatments. Mass-like or diffuse wall–thickening on coronary computed tomography is a characteristic radiographic finding of immunoglobulin G4–related coronary periarteritis, which can occur in any branch. Immunoglobulin G4–related coronary periarteritis showed similar characteristics to other organ lesions, including its relatively low serum immunoglobulin-G4 level in patients with a single-organ disease and its high responsiveness to glucocorticoids.

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Data used in this research are available on a reasonable request to the corresponding author (SF).

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Acknowledgements

We thank all the physicians, nurses, and other staff who cared for the enrolled patients. We thank Editage (www.editage.com) for editing services. This study did not receive any specific funding from any bodies in the public, commercial or not-for-profit sectors.

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  1. Takanori Ito and Sho Fukui contributed equally.

Authors and Affiliations

  1. Immuno-Rheumatology Center, St. Luke’s International Hospital, Tokyo, Japan

    Takanori Ito, Sho Fukui, Hiromichi Tamaki & Masato Okada

  2. Department of Emergency and General Medicine, Kyorin University, Tokyo, Japan

    Sho Fukui

  3. Department of Cardiology, St. Luke’s International Hospital, Tokyo, Japan

    Takayoshi Kanie

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Contributions

SF and TI conceptualized and originally designed the study, which was reviewed by TK, HT, and MO. TI and SF performed the literature review. Data was analyzed by TI and SF. TI and SF initially interpreted the analyzed data, which was subsequently reviewed by TK, TH, and MO. TI and SF drafted the original manuscript, which was critically reviewed and revised by TK, TH, and MO. All authors read and approved the final version of the manuscript.

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Correspondence to Sho Fukui.

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Ethics approval and consent to participate

This study was approved by the institutional review board of St. Luke's International Hospital in Tokyo, Japan (Number: 20-R145). The patient data were anonymized, and informed consent was obtained in the form of an opt-out option.

Competing interests

Hiromichi Tamaki reports receiving personal fees from Chugai pharmaceutical, AbbVie, Ono Pharmaceutical, Kyowa-Kirin, Takeda Pharmaceutical, Astellas Pharma, Tanabe-Mitsubishi, and Ayumi, outside the submitted work. Masato Okada received speaking fees and/or honoraria from Eli Lilly and Company, Santen Pharmaceutical, Tanabe-Mitsubishi Pharma, Pfizer, and Abbott Japan. The other authors declare that they have no competing interests.

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Ito, T., Fukui, S., Kanie, T. et al. Immunoglobulin G4–related coronary periarteritis: a systematic literature review with a case series. Clin Rheumatol 41, 2281–2295 (2022). https://doi.org/10.1007/s10067-022-06179-y

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