Background: IgG4-related disease is a group of diseases reported from Japan that shows high level
of serum IgG4 and tissue infiltration of IgG4 positive plasma cells, such as organ
swelling and nodular lesions. Lesions are known to affect multiple organs of the whole
body. However, cardiovascular disorder report is rare. The involvement of autoimmune
mechanism is considered, PSL therapy is the first choice. We report two cases of complete
AV block during the treatment of IgG4-related diseases. Case 1: 67-year-old male. He had undergone CABG and PCI. At about 2 months from the start
of the PSL treatment, complete AV block was presented. Because coronary angiography
did not change from before, permanent pacemaker implantation was done. Case 2: 67-year-old female. We diagnosed her with this disease due to pancreatic enlargement
and high level of serum IgG4. After PSL treatment started, a complete AV block was
recognized. Since she was asymptomatic and low in activity, we decided not to implant
a pacemaker. Pancreatic enlargement shrank due to continuation of treatment, complete
AV block also has disappeared. Conclusion: The clinical course of the two cases suggested a link between IgG4 related diseases
and AV block. Because of one case improved the AV block with administration of PSL,
it may be reversible by disease control.
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