Clinical characteristics of endocrine glands involvement in patients with IgG4-related diseases

Abstract

Objective To explore the clinical manifestations, imaging and pathology features, treatment, and prognosis of endocrine glands involved patients with IgG4-related disease (IgG4-RD). Methods Ten patients admitted in Peking Union Medical College Hospital from 1 January 2014 to 30 June 2018 diagnosed as IgG4-RD with endocrine glands involved were enrolled in this study. All the clinical data were collected and analyzed. Results Ten patients, 4 males and 6 females, median 55 years old at the onset were enrolled, five patients with single organ involvement (1 case involved in pituitary, and 4 cases involved in thyroid), while another 5 patients with 2 and more organs involved. C-reactive protein or erythrocyte sedimentation rate was elevated in 8 patients, antinuclear antibodies were positive in 7, and serum total IgE was elevated in 6 patients. Nine patients were treated with oral glucocorticoids, among whom 3 patients were treated in combination with immunosuppressive agents or rituximab. After treatment, the clinical symptoms were alleviated and imaging was improved in all patients. Serum IgG levels were significantly decreased in all patients and normalized in 5 patients. Conclusion For the patients with multiple endocrine gland diseases and elevated IgG4 level, the possibility of IgG4-RD should be carefully considered and should be confirmed by tissue biopsy and histopathology. (Chin J Endocrinol Metab, 2018, 34: 839-843) Key words: IgG4-related disease; Pituitary; Thyroid; Pancreas