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Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: a prospective study of 403 patients.
Rheumatology (Oxford) 2019; 58(5):820-830R

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.

Authors+Show Affiliations

Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing. Tsinghua University School of Medicine, Beijing, China.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing.Department of Rheumatology, Hebei General Hospital, Shijiazhuang.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing. Tsinghua University School of Medicine, Beijing, China.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing.Department of Stomatology, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.Department of Ophthalmology, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing.Department of Gastroenterology, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.Department of General Internal Medicine, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.Department of Nephrology, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing.Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

30561747

Citation

Wang, Liwen, et al. "Sex Disparities in Clinical Characteristics and Prognosis of Immunoglobulin G4-related Disease: a Prospective Study of 403 Patients." Rheumatology (Oxford, England), vol. 58, no. 5, 2019, pp. 820-830.
Wang L, Zhang P, Zhang X, et al. Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: a prospective study of 403 patients. Rheumatology (Oxford). 2019;58(5):820-830.
Wang, L., Zhang, P., Zhang, X., Lin, W., Tang, H., Li, J., ... Zhang, W. (2019). Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: a prospective study of 403 patients. Rheumatology (Oxford, England), 58(5), pp. 820-830. doi:10.1093/rheumatology/key397.
Wang L, et al. Sex Disparities in Clinical Characteristics and Prognosis of Immunoglobulin G4-related Disease: a Prospective Study of 403 Patients. Rheumatology (Oxford). 2019 May 1;58(5):820-830. PubMed PMID: 30561747.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: a prospective study of 403 patients. AU - Wang,Liwen, AU - Zhang,Panpan, AU - Zhang,Xia, AU - Lin,Wei, AU - Tang,Hanqi, AU - Li,Jieqiong, AU - Wang,Mu, AU - Liu,Xiaowei, AU - Fei,Yunyun, AU - Chen,Hua, AU - Peng,Linyi, AU - Zhang,Li, AU - Lai,Yamin, AU - Zeng,Xuejun, AU - Li,Xuemei, AU - Xue,Huadan, AU - Zhao,Yan, AU - Zhang,Fengchun, AU - Zhang,Wen, PY - 2018/07/24/received PY - 2018/11/08/revised PY - 2018/12/19/pubmed PY - 2018/12/19/medline PY - 2018/12/19/entrez KW - IgG4-related disease KW - clinical characteristics KW - glucocorticoids KW - prognosis KW - sex SP - 820 EP - 830 JF - Rheumatology (Oxford, England) JO - Rheumatology (Oxford) VL - 58 IS - 5 N2 - 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. SN - 1462-0332 UR - https://www.unboundmedicine.com/medline/citation/30561747/Sex_disparities_in_clinical_characteristics_and_prognosis_of_immunoglobulin_G4_related_disease:_a_prospective_study_of_403_patients_ L2 - https://academic.oup.com/rheumatology/article-lookup/doi/10.1093/rheumatology/key397 DB - PRIME DP - Unbound Medicine ER -