Abstract
IgG4-related disease (IgG4 RD) has recently been established as a new clinical entity characterized by plasma cell infiltration resulting in sclerosis in various organs, but the pathophysiology and clinical features are not fully understood. Here, we report a rare case of IgG4 RD that developed during chronic hemodialysis. A 61-year-old male with polycystic kidney disease who had been on hemodialysis for seven years was referred to our hospital because of nausea, cough, and asthma that had recently appeared during a hemodialysis session. The symptoms continued even after dialyzers were changed to other types. He had been experiencing submaxillary gland swelling for five years. His blood tests showed eosinophilia (8,000/μL), hypergammaglobulinemia (serum IgG 5,462 mg/dL), and a rise in IgG4 concentration (1,540 mg/dL). A biopsy of the gland revealed infiltration of plasma cells, including more than 50% IgG4-positive cells, without evidence of tumor ; thus, he was diagnosed with IgG4 RD. No involvement was found in other organs, including pancreas. Oral prednisolone (30 mg/day) was initiated and the symptoms during hemodialysis immediately disappeared, with gradual improvement of eosinophilia and submaxillary gland swelling. We should consider the possibility of IgG4 RD when we see patients with episodic asthma during chronic hemodialysis.
