Atrioventricular Block: Granulomatosis with Polyangiitis or IgG4-related diseases?

HE Xin; ZHANG Guizhi; LU Tao; YANG Deyan; PENG Linyi; CHEN Hua; ZHANG Wen; ZENG Xiaofeng; ZHANG Fengchun

doi:10.12290/xhyxzz.2022-0079

Volume 14 Issue 2

Mar. 2023

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Medical Journal of Peking Union Medical College Hospital > 2023 > 14(2): 379-384. > DOI: 10.12290/xhyxzz.2022-0079

HE Xin, ZHANG Guizhi, LU Tao, YANG Deyan, PENG Linyi, CHEN Hua, ZHANG Wen, ZENG Xiaofeng, ZHANG Fengchun. Atrioventricular Block: Granulomatosis with Polyangiitis or IgG4-related diseases?[J]. Medical Journal of Peking Union Medical College Hospital, 2023, 14(2): 379-384. DOI: 10.12290/xhyxzz.2022-0079

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Atrioventricular Block: Granulomatosis with Polyangiitis or IgG4-related diseases?

HE Xin^{1, 2},
ZHANG Guizhi^{1, 2},
LU Tao³,
YANG Deyan⁴,
PENG Linyi^{1, 2, ,},
CHEN Hua^{1, 2},
ZHANG Wen^{1, 2},
ZENG Xiaofeng^{1, 2},
ZHANG Fengchun^{1, 2}

1.
Department of Rheumatology, Key Laboratory of Rheumatology, Ministry of Education, National Clinical Research Center for Dermatologic and Immunologic Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
2.
State Key Laboratory of Complex Severe and Rare Diseases, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
3.
Department of Pathology, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
4.
Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China

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the State Key Laboratory of Environmental Chemistry and Ecotoxicology KF2021-15

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Corresponding author:
PENG Linyi, E-mail: drpenglinyi@163.com
Received Date: February 22, 2022
Accepted Date: May 08, 2022
Available Online: January 03, 2023
Issue Publish Date: March 29, 2023

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Abstract

Abstract

We reported a case of an elderly female patient with multi-organ involvement of the ear, eye, nose and heart, with elevated serum inflammatory markers and IgG4, and negative anti-neutrophil cytoplasmic antibodies. The patient was suspected to have granulomatosis with polyangiitis (GPA) combined with IgG4-related disease (IgG4-RD), because both GPA and IgG4-RD can lead to multi-organ involvement, with similar clinical manifestations and consistency in treatment. After treatment with high-dose glucocorticoid combined with cyclophosphamide, the clinical symptoms were relieved. The patient also had a high degree of atrioventricular block (AVB), which is rare in both GPA and IgG4-RD. In this paper we discuss the characteristics of the overlap syndrome, the diagnosis and treatment of this patient and the possible pathological mechanism of AVB, in order to provide reference for clinical treatment.
- granulomatosis with polyangiitis,
- IgG4-related disease,
- atrioventricular block,
- immunosuppressive therapy

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Atrioventricular Block: Granulomatosis with Polyangiitis or IgG4-related diseases?

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