Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: a prospective study of 403 patients.

To study the impact of sex on the clinical presentation of IgG4-related disease (IgG4-RD). We prospectively enrolled 403 newly diagnosed IgG4-RD patients. We compared the demographic features, clinical manifestations, organ involvement, laboratory tests and treatment outcomes between female and male patients. The organs involved were divided into superficial organs (salivary glands, lacrimal glands, orbit, sinus and skin) and internal organs (all the other organs). The patients treated with glucocorticoids with or without additional immunosuppressants were included in the assessment of treatment outcomes, and potential confounding factors were corrected by propensity score matching or multivariate Cox regression analysis. Female patients showed younger age at both symptom onset and diagnosis, and a longer interval between symptom onset and diagnosis. Allergy history, Mikulicz's disease and thyroiditis were more common in female patients, while autoimmune pancreatitis, sclerosing cholangitis and retroperitoneal fibrosis were more common in male patients. In accordance, female patients more frequently presented with superficial organ involvement, while male patients more frequently had internal organ involvement, and the discrepancy was more prominent in the patients with older age. Male sex was associated with higher peripheral eosinophils, CRP and IgG4 levels at baseline. In response to glucocorticoid-based therapies, male sex was associated with a higher IgG4-RD responder index during follow-up as well as a greater risk of relapse (hazard ratio 3.14, P = 0.003). Our study revealed the sex disparities in clinical characteristics of IgG4-RD, and indicated that male sex was independently associated with worse prognosis in response to glucocorticoid-based therapies.

Wang L ，Zhang P ，Zhang X ，Lin W ，Tang H ，Li J ，Wang M ，Liu X ，Fei Y ，Chen H ，Peng L ，Zhang L ，Lai Y ，Zeng X ，Li X ，Xue H ，Zhao Y ，Zhang F ，Zhang W ... -  《-》

[The clinical characteristics of 346 patients with IgG(4)-related disease].

Zhang PP ，Zhao JZ ，Wang M ，Feng RE ，Liu XW ，Lai XM ，Li XJ ，Zeng JG ，Shi HJ ，Zhu HD ，Xue W ，Zhang H ，Chen YY ，Fei LY ，Peng XF ，Zeng FC ，Zhang YM ，Zhang W ... -  《-》

Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients.

To characterize the clinical features of IgG4-related disease (IgG4-RD) in China. A prospective cohort study of IgG4-RD was carried out in Peking Union Medical College Hospital between 2011 and 2013. Patients with newly diagnosed IgG4-RD were enrolled. A total of 118 patients with IgG4-RD were enrolled, including 82 males and 36 females, aged 53.1 (s.d. 13.6) years. The most common symptom at onset was lacrimal gland swelling (38/32.2%). A range of organs were involved: 77 patients (65.3%) had lymphadenopathy, 76 (64.4%) had sialadenitis, 60 (50.8%) had dacryoadenitis, 45 (38.1%) had autoimmune pancreatitis, 32 (27.1%) had pulmonary involvement, 31 (26.3%) had periaortitis/retroperitoneal fibrosis, 29 (35.4% of male patients) had prostatitis and 29 (24.6%) had renal involvement. In addition, there were 21 (17.8%) cases of sclerosing cholangitis, 15 (12.7%) of sinusitis and 10 (8.5%) of inflammatory pseudotumour. Uncommon manifestations included mediastinal fibrosis, skin involvement, sclerosing thyroiditis, hypophysitis, orchitis and colitis. Multiple organ involvement was observed in 93 patients, whereas only 4.2% had only a single organ involved. A history of allergy was reported in 73 (61.9%) patients. The serum IgG4 level was elevated in 97.5% and was correlated with the number of organs involved. Most patients were treated with glucocorticoids alone or in combination with immunosuppressive drugs, and the majority usually improved within 3 months. IgG4-RD is a systemic inflammatory and sclerosing disease. Parotid and lacrimal involvement (formerly called Mikulicz's disease), lymphadenopathy and pancreatitis are the most common manifestations. Patients with IgG4-RD showed favourable responses to treatment with glucocorticoids and immunosuppressive agents.

Lin W ，Lu S ，Chen H ，Wu Q ，Fei Y ，Li M ，Zhang X ，Tian X ，Zheng W ，Leng X ，Xu D ，Wang Q ，Shen M ，Wang L ，Li J ，Wu D ，Zhao L ，Wu C ，Yang Y ，Peng L ，Zhou J ，Wang Y ，Sha Y ，Huang X ，Jiao Y ，Zeng X ，Shi Q ，Li P ，Zhang S ，Hu C ，Deng C ，Li Y ，Zhang S ，Liu J ，Su J ，Hou Y ，Jiang Y ，You X ，Zhang H ，Yan L ，Zhang W ，Zhao Y ，Zeng X ，Zhang F ，Lipsky PE ... -  《-》

Clinical features and relapse risks factors of IgG4 related disease: a single-center retrospective study.

Zhang X ，Zeng Z ，Tian H ，Wang N ，Wang Y ，Tong J ，Chang B ，Jin X ，Huang D ，Wang Y ，Cui H ，Guan L ，Li Y ... -  《-》

Differences in clinical characteristics of IgG4-related disease across age groups: a prospective study of 737 patients.

The aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups. We conducted a prospective study of 737 patients who were newly diagnosed with IgG4-RD and compared detailed demographic features, organ involvements, laboratory tests, treatments and outcomes across age groups. The patients were divided into five groups according to their age at diagnosis: ≤39, 40-49, 50-59, 60-69 and ≥70 years. The clinical characteristics of paediatric patients were also described. Sex ratio, disease duration, allergy history and clinical symptoms were significantly different across age groups. Besides, the proportions of superficial organ involvement (lacrimal gland and sinus) decreased with age, while the proportions of internal organ involvement (pancreas, biliary tract, retroperitoneal tissue, lung and prostate) increased with age, which was more prominent in male patients. Mikulicz's disease was the most common manifestation (70%) in paediatric IgG4-RD patients. Multiple Cox analysis identified that age ≤56 years at diagnosis was an independent risk factor of relapse. We revealed the impact of age on clinical characteristics of IgG4-RD, which indicated that different management might be required among different age groups.

Lu H ，Teng F ，Zhang P ，Fei Y ，Peng L ，Zhou J ，Wang M ，Liu X ，Zhu L ，Wang L ，Luo X ，Liu Z ，Li J ，Zhao Y ，Zhang W ，Zeng X ... -  《-》