Salivary gland involvement disparities in clinical characteristics of IgG4-related disease: a retrospective study of 428 patients

Abstract

Objectives IgG4-related disease (IgG4-RD) has recently been recognized as a fibro-inflammatory condition featuring tumefactive lesions in multiple organs, and the salivary gland is one of the most commonly involved sites. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with salivary gland lesions (IgG4-RD SG+) and salivary-gland-free IgG4-RD (IgG4-RD SG−) in a large cohort. Methods We carried out a retrospective review of the medical records of 428 cases of IgG4-RD diagnosed at Peking University People’s Hospital between March 2006 and May 2018. Results Among 428 patients, 249 had salivary glands that were affected. IgG4-RD SG+ patients showed younger age at disease onset and diagnosis, and a longer interval between symptom onset and diagnosis. The IgG4-RD SG+ group involved more female patients, and allergic diseases were more common in this group. In terms of organ involvement, the IgG4-RD SG+ group were more frequently presented with lacrimal gland involvement, while lymph node, retroperitoneal fibrosis, pancreas, biliary system, kidney and aorta were more prominent in the IgG4-RD SG− group. In addition, the serum IgG4 level, IgG4/IgG ratio and IgE level were significantly higher in IgG4-RD SG+ patients. Patients with eosinophilia were more common in the IgG4-RD SG+ group, while elevated ESR, CRP and positive ANA were more common in the IgG4-RD SG− group. Conclusion We have revealed demographic, clinical and laboratory differences between IgG4-RD SG+ and SG− patients, which indicated potential differences in pathogenesis and important implications for the diagnosis and management of these two phenotypes.

Advances in the diagnosis and management of IgG4 related disease

“…Patients with head and neck limited disease (group 3) were far more likely to be female and Asian than were patients in the other groups (fig 3). 24101 They were also significantly younger and needed histological confirmation to achieve a final diagnosis more often than did other groups 102. Referral to the emergency department because of symptoms attributed to onset of IgG4-RD occurred more often in patients with pancreato-hepatobiliary disease (group 1) 103.…”

Clinical features and relapse risks of IgG4-related ophthalmic disease: a single-center experience in China

“…Serum IgG4 level is elevated in the majority of patients with IgG4-RD, but this is a nonspecific finding [3]. Characteristic histopathologic features are lymphoplasmacytic infiltration rich in IgG4-immunopositive plasma cells, storiform fibrosis, and obliterative phlebitis [4,5]. IgG4-RD is a highly heterogenous disorder that can affect almost every organ system in the body [6][7][8][9], and the ocular lesions are also extremely common.…”

Disparities between IgG4-related kidney disease and extrarenal IgG4-related disease in a case–control study based on 450 patients

“…IgG4-RD is manifested by typical clinical features, including tumor-like lesions, dense infiltration with IgG4-positive plasma cells, and extensive fibrosis of multiple organs. There is a great variability of disease manifestations for IgG4-RD, and the identification of different IgG4-RD subgroups is crucial, as a consequence of significant disparities in the characteristics of IgG4-RD regarding different organs 6 , 15 . In our study, the frequency of kidney involvement in IgG4-RD patients was 11.8%, which is lower than that of Japan (23.7%) and Mexico (24.6%) 6 , 16 , but similar to that of UK study 11 .…”