IgG4-RD was suspected; however, biopsy of the periaorta had risk of the injury of abdominal aorta, and kidney biopsy was contraindicated owing to hydronephrosis. He did not agree on submandibulectomy for fear surgical risk. A fine needle biopsy of SMG would be inadequate for sampling in IgG4-RD patients. Thus, we performed less invasive lip biopsy in an attempt to obtain pathological specimen with patient agreement. Histological examination of his LSG revealed heavy infiltration of lymphocytes and hyperplastic germinal center formation (Fig. 3A), adding massive plasma cells infiltration revealed by CD138 staining (Fig. 3B). Forty-seven percent of those plasma cells with IgG immunoreactivity (Fig. 3C) were positively immunolabeled with antibody for IgG4 (Fig. 3D).
In summary, we report a case of IgG4-RD affecting SMGs, periaorta, and kidney that could be pathologically diagnosed by lip biopsy. With lip biopsy being minimally invasive, convenient, and useful for pathological confirmation for IgG4-RD, we emphasize this procedure as one of the options when affected organs are difficult to approach. Early recognition and diagnosis of this disease is clinically important because most patients respond well to glucocorticoid, which prevents irreversible organ damage.
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