Application of ultrasonography scoring system in the assessment of IgG4-related sialadenitis / 北京大学学报(医学版)

OBJECTIVE@#To assess the diagnostic value of salivary gland ultrasonography (SGUS) for IgG4-related sialadenitis.@*METHODS@#Ultrasonography examination of major salivary glands was conducted for 48 IgG4-related sialadenitis patients and 50 Sjögren's syndrome patients, whose ages and disease duration were matched. The imaging features were graded using two different scoring systems (0-16 and 0-48, respectively) obtained from the grades of bilateral parotid and submandibular glands. The scores were used to further evaluate the features of salivary gland ultrasonography in IgG4-related sialadenitis and to compare them with Sjögren's syndrome patients. The association of SGUS scores of IgG4-related sialadenitis group with serological tests was analyzed.@*RESULTS@#The mean age of IgG4-related sialadenitis group and Sjögren's syndrome group was 49.23 years and 50.44 years, respectively. The serum IgG4 level of the patients in the IgG4-related sialadenitis group was increased, with an average (9.60±6.43) g/L. And the serum IgE level was at a median of 251.5 (123.4-543.6) IU/mL. In the 0-16 system, the scores of submandibular glands of the patients in IgG4-related sialadenitis and Sjögren's syndrome were 6.0 (6.0-8.0) and 4.0 (2.0-8.0), and the scores of the total four glands were 10.0 (8.0-14.0) and 8.0 (4.0-12.0) respectively. In the 0-48 system, the scores of submandibular glands with IgG4-related sialadenitis and Sjögren's syndrome were 18.0 (14.5-20.0) and 11.0 (7.0-14.0), and the scores of the total four glands were 26.0 (18.5-34.0) and 21.5 (15.0-26.3) respectively. It suggested that in the 0-16 system and the 0-48 system, scores of submandibular glands and the total of four glands of IgG4-related sialadenitis were higher than those of Sjögren's syndrome. Meanwhile, the association analysis of 0-48 system showed a positive correlation of SGUS scores with serum IgG4, which also showed a positive correlation of SGUS scores with serum IgE in 0-16 system.@*CONCLUSION@#Semi-quantitative ultrasonography scoring systems can evaluate and quantify the lesions of salivary glands, which can be helpful in the diagnosis and differential diagnosis of IgG4-related sialadenitis combined with the clinical manifestations, serological indicators and/or histopathological manifestations. Ultrasonography can also assess the activity of IgG4-related sialadenitis preliminarily.

Analysis of the clinical features and misdiagnosis reasons of 17 patients misdiagnosed with IgG4-related disease / 北京大学学报(医学版)

OBJECTIVE@#To summarize the clinical characteristics of patients misdiagnosed with IgG4-related disease, to analyze the reasons of misdiagnosis and to improve the clinical recognition of the disease.@*METHODS@#The general data, clinical manifestations, laboratory examination results and pathological features of 17 patients with IgG4-related diseases misdiagnosed outside the hospital were retrospectively analyzed.@*RESULTS@#Among the 17 patients, there were 9 males and 8 females with a median age of 45 years, and the median time from onset to diagnosis was 12 months. Most patients' initial admission department was not rheumatology or immunology department. Six of the 17 patients were eventually diagnosed with lymphoproliferative disease, 4 with autoimmune disease, and 2 with infectious disease, Rosai Doffman disease, desmofibromatosis, highly differentiated mucoepidermoid carcinoma of the bottom of the mouth, hypereosinophilic syndrome, asthma and allergic rhinitis in 1 case each. The typical sites of IgG4-related disease were involved in 14 patients, including 6 cases of parotid gland, 2 cases of submandibular gland, 3 cases of pancreas and 2 cases of retroperitoneal lesions. Serum IgG4 was elevated in 10 patients, serum IgG4/IgG value was higher than 10% in 7 patients, serum IgE was increased in 7 patients, complement was decreased in 4 patients, and eosinophilic granulocytes were increased in 3 patients. Pathological biopsy was performed in 15 patients, and infiltration of lymphocyte was observed in 10 patients, IgG4+ plasma cells were present in 5 patients, the ratio of IgG4+ plasma cells to IgG+ plasma cells was less than 40% in 4 patients and greater than 40% in 1 patient. However, none of the 15 patients had the storiform pattern of fibrosis and obliterative phlebitis.@*CONCLUSION@#A variety of diseases can perform as IgG4-related disease witih typical sites involved, elevated serum IgG4, even can be characterized by pathological IgG4+ plasma cells infiltration. Physicians should pay attention to the differential diagnosis and comprehensively evaluate the patient's clinical manifestations, and laboratory results. Timely and even repeated pathological biopsy is also needed for definite diagnosis.