To explore the clinical characteristics, serology, imaging, histopathology, treatment, and prognosis of IgG4-related diseases (IgG4-RD).

Clinical data of 76 patients diagnosed with IgG4-RD at the Second Hospital of Shanxi Medical University from 2013 to 2019 were retrospectively analyzed.

Among the 76 patients, there were 39 males (51.3%) and 37 females (48.7%), with an average age of (56.1±11.8) years old. The most common involved organ was the submandibular gland (32.9%, 25/76), followed by the labial gland (15.8%, 12/76), pancreas (13.2%, 10/76), parotid gland (11.8%, 9/76), retroperitoneum (7.9%, 6/76), lymph nodes (5.3%, 4/76), sinus (2.6%, 2/76), lung (2.6%, 2/76), kidney (2.6%, 2/76), bile duct (2.6%, 2/76), skin (1.3%, 1/76), and colon (1.3%, 1/76). IL-6 was positively correlated with IgG4 (r=0.483, P<0.01), Interferon-γ was positively correlated with IgG4 (r=0.228, P<0.05), IL-2, IL-4, IL-10 and TNF-α had no correlation with IgG4 (P>0.05). Histopathological examination was performed in 61 cases, which were mainly characterized by infiltration of lymphocytes and plasmacytes and fibrosis,>10 IgG4(+) plasma cells per high-power field, or ratio of IgG4(+)/IgG(+) cells > 40%. Of all patients included, 23.7%(18/76) were treated with glucocorticoid alone, 28.9% (22/76) were treated with hormone combined with immunosuppressive therapy, 44.7% (34/76) were treated with hormone combined with immunoregulatory therapy, 2.6% (2/76) were untreated, and 90.8% (69/76) responded well to initial treatment.

IgG4-RD is an autoimmune disease with multiple organ involvement. The most frequently involved organs are the submandibular gland, labrum gland, pancreas, and parotid gland. Combined detection of IL-6 and interferon-γ may play a predictive role in the pathogenesis of IgG4-RD, and they may become new targets for clinical treatment of IgG4-RD.