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Volume 14 Issue 2
Mar.  2023
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Article Navigation >  Medical Journal of Peking Union Medical College Hospital  > 2023  >  14(2): 379-384
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
Citation: 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
shu

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

doi: 10.12290/xhyxzz.2022-0079
  • 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

Funds:

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

More Information
  • Corresponding author: PENG Linyi, E-mail: drpenglinyi@163.com
  • Received Date: 2022-02-23
  • Accepted Date: 2022-05-09
    • Available Online: 2023-01-04
  • Publish Date: 2023-03-30
    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.
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  • [1] Pugnet G, Gouya H, Puéchal X, et al. Cardiac involvement in granulomatosis with polyangiitis: a magnetic resonance imaging study of 31 consecutive patients[J]. Rheumatology, 2017, 56: 947-956. doi:  10.1093/rheumatology/kew490
    [2] Colin GC, Vancraeynest D, Hoton D, et al. Complete heart block caused by diffuse pseudotumoral cardiac involvement in granulomatosis with polyangiitis[J]. Circulation, 2015, 132: 207-210.
    [3] Abe N, Takahashi H, Inoue T, et al. Wegener granulo-matosis of the heart[J]. J Thorac Cardiovasc Surg, 2018, 156: 93-97. doi:  10.1016/j.jtcvs.2018.03.007
    [4] Santos LPS, Bomfim VG, Bezerra CF, et al. Heart conduction system defects and sustained ventricular tachycardia complications in a patient with granulomatosis with polyangiitis. A case report and literature review[J]. Rev Bras Ter Intensiva, 2017, 29: 386-390.
    [5] Shakir A, Wheeler Y, Krishnaswamy G. The enigmatic immunoglobulin G4-related disease and its varied cardiova-scular manifestations[J]. Heart, 2021, 107: 790-798. doi:  10.1136/heartjnl-2020-318041
    [6] Comai G, Cuna V, Fabbrizio B, et al. A case report of IgG4-related disease: an insidious path to the diagnosis through kidney, heart and brain[J]. BMC Nephrol, 2019, 20: 418. doi:  10.1186/s12882-019-1587-4
    [7] Danlos FX, Rossi GM, Blockmans D, et al. Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome[J]. Autoimmun Rev, 2017, 16: 1036-1043. doi:  10.1016/j.autrev.2017.07.020
    [8] Chang SY, Keogh K, Lewis JE, et al. Increased IgG4-Positive Plasma Cells in Granulomatosis with Polyangiitis: A Diagnostic Pitfall of IgG4-Related Disease[J]. Int J Rheumatol, 2012, 2012: 121702.
    [9] Chang SY, Keogh KA, Lewis JE, et al. IgG4-positive plasma cells in granulomatosis with polyangiitis(Wegener's): a clinicopathologic and immunohistochemical study on 43 granulomatosis with polyangiitis and 20 control cases[J]. Hum Pathol, 2013, 44: 2432-2437. doi:  10.1016/j.humpath.2013.05.023
    [10] Robson JC, Grayson PC, Ponte C, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis[J]. Arthritis Rheumatol, 2022, 74: 393-399. doi:  10.1002/art.41986
    [11] Yoo J, Ahn SS, Jung SM, et al. No overlap between IgG4-related disease and microscopic polyangiitis and granulomatosis with polyangiitis despite elevated serum IgG4 at diagnosis: a retrospective monocentric study[J]. Clin Rheumatol, 2019, 38: 1147-1154. doi:  10.1007/s10067-018-4402-x
    [12] Kaushik P, Stone JH, Anderson JT, et al. Medical mirroring: granulomatosis with polyangiitis (formerly Wegener's) mimicking immunoglobulin-G4 related disease[J]. Int J Rheum Dis, 2018, 21: 885-889. doi:  10.1111/1756-185X.12687
    [13] Jiang Z, Wang D, Jurkiewicz M, et al. Immunoglobulin G4-related disease as a mimicker of granulomatosis with polyangiitis: a case report[J]. Scand J Rheumatol, 2020, 49: 163-164. doi:  10.1080/03009742.2019.1672088
    [14] Li ZY, Wang X, Xia X, et al. An overlap of antineutrophil cytoplasmic antibody (ANCA)-associated glomerulone-phritis and IgG4-related kidney disease[J]. Clin Chim Acta, 2020, 501: 12-19. doi:  10.1016/j.cca.2019.11.030
    [15] Walsh M, Flossmann O, Berden A, et al. Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis[J]. Arthritis Rheum, 2012, 64: 542-548. doi:  10.1002/art.33361
    [16] McGeoch L, Carette S, Cuthbertson D, et al. Cardiac Involvement in Granulomatosis with Polyangiitis[J]. J Rheumatol, 2015, 42: 1209-1212. doi:  10.3899/jrheum.141513
    [17] Yamauchi H, Satoh H, Yamashita T, et al. Immunog-lobulin G4-related disease of the heart causing aortic regurgitation and heart block[J]. Ann Thorac Surg, 2013, 95: 151-153. doi:  10.1016/j.athoracsur.2012.11.057
    [18] Kosugi S, Okada M, Iwata K, et al. Immunoglobulin G4-related disease of the thickened aortic valve extending to the left ventricular outflow tract causing severe aortic regurgitation and complete atrioventricular block: a case report[J]. Eur Heart J Case Rep, 2018, 2: 1-5.
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