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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