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

Abstract

Objectives To study the impact of sex on the clinical presentation of IgG4-related disease (IgG4-RD). Methods 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. Results 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). Conclusion 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.

Identifying clinical subgroups in IgG4-related disease patients using cluster analysis and IgG4-RD composite score

“…We have known that IgG4-RD has a gender predilection [1]. Female patients present more frequently with superficial organ involvement, while male patients more frequently have internal organ involvement [20]. Similarly, our cluster analysis results indicated that cluster 2 was characterized by internal organ-dominant (male to female, 3.53:1) and cluster 3 was characterized by superficial organ-dominant (male to female, 0.94:1).…”

“…The number of organs involved was determined by a review of the patient's history, physical examination, blood tests, imaging, and tissue biopsies. The organs involved were divided into superficial organs (i.e., salivary glands, lacrimal glands, orbit, sinus, and skin) and internal organs (i.e., all of the other organs) [20][21][22]. Lymph nodes were not taken into account when assessing the involvement of the superficial/internal organs [20].…”

“…The organs involved were divided into superficial organs (i.e., salivary glands, lacrimal glands, orbit, sinus, and skin) and internal organs (i.e., all of the other organs) [20][21][22]. Lymph nodes were not taken into account when assessing the involvement of the superficial/internal organs [20]. Disease activity was assessed using the 2012 IgG4-RD RI [5], and the involved organs were evaluated individually.…”

Malignancy and IgG4-related disease: the incidence, related factors and prognosis from a prospective cohort study in China

“…Our team at Peking Union Medical College Hospital (PUMCH) established an IgG4-RD cohort in 2011. In previous studies, we prospectively described the clinical characteristics 11 , sex disparities 12 , radiographic presentation 13 and histopathologic features 14 of patients with IgG4-RD in our cohort. The present study focused on the Data collection.…”

Immunoglobulin G4–Related Disease: What an Allergist Should Know

“…31 (3) © 2020 Esmon Publicidad doi: 10.18176/jiaci.0633 [15,16]. In general, women with IgG4-RD are younger, with a higher prevalence of allergic diseases, lower levels of blood eosinophils, lower C-reactive protein (CRP), lower serum IgG4, and better prognosis of the disease [17].…”