Comparison of clinical features and outcomes of proliferative, fibrotic, and mixed subtypes of IgG4-related disease: A retrospective cohort study.

Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized immune-mediated disorder that can affect almost any organ in the human body. IgG4-RD can be categorized into proliferative and fibrotic subtypes based on patients' clinicopathological characteristics. This study aimed to compare the clinical manifestations, laboratory findings, and treatment outcomes of IgG4-RD among different subtypes. We prospectively enrolled 622 patients with newly diagnosed IgG4-RD at Peking Union Medical College Hospital from March 2011 to August 2021. The patients were divided into three groups according to their clinicopathological characteristics: proliferative, fibrotic, and mixed subtypes. We compared demographic features, clinical manifestations, organ involvement, laboratory tests, and treatment agents across three subtypes. We then assessed the differences in treatment outcomes among 448 patients receiving glucocorticoids alone or in combination with immunosuppressants. Moreover, risk factors of relapse were revealed by applying the univariate and multivariate Cox regression analysis. We classified the 622 patients into three groups consisting of 470 proliferative patients, 55 fibrotic patients, and 97 mixed patients, respectively. We found that gender distribution, age, disease duration, and frequency of allergy history were significantly different among subgroups. In terms of organ involvement, submandibular and lacrimal glands were frequently involved in the proliferative subtype, while retroperitoneum was the most commonly involved site in both fibrotic subtype and mixed subtype. The comparison of laboratory tests revealed that eosinophils ( P = 0.010), total IgE ( P = 0.006), high-sensitivity C-reactive protein ( P <0.001), erythrocyte sedimentation rate ( P <0.001), complement C4 ( P <0.001), IgG ( P = 0.001), IgG1 (P <0.001), IgG4 (P <0.001), and IgA ( P <0.001), at baseline were significantly different among three subtypes. Compared with proliferative and mixed subtypes, the fibrotic subtype showed the lowest rate of relapse (log-rank P = 0.014). Our study revealed the differences in demographic characteristics, clinical manifestations, organ involvement, laboratory tests, treatment agents, and outcomes across proliferative, fibrotic, and mixed subtypes in the retrospective cohort study. Given significant differences in relapse-free survival among the three subtypes, treatment regimens, and follow-up frequency should be considered separately according to different subtypes.

Peng L ，Zhang X ，Zhou J ，Li J ，Liu Z ，Lu H ，Peng Y ，Fei Y ，Zhao Y ，Zeng X ，Zhang W ... -  《-》

Clinical characteristics and outcome of IgG4-related disease with hypocomplementemia: a prospective cohort study.

Peng L ，Lu H ，Zhou J ，Zhang P ，Li J ，Liu Z ，Wu D ，Zhang S ，Yang Y ，Bai W ，Wang L ，Fei Y ，Zhang W ，Zhao Y ，Zeng X ，Zhang F ... -  《-》

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 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 ... -  《-》