Abstract
Background IgG4 related disease (IgG4-RD) is recently recognized as a fibro-inflammatory condition featured by tumefactive lesions in multiple organs, among which salivary gland is one of the most commonly involved sites. Some previous studies have suggested that salivary gland involved IgG4-RD (IgG4-RD SG+) patients may present different features compared to IgG4-RD patients without salivary gland lesions (IgG4-RD SG-), however, only dozens of patients were examined and the comparison has not been studied in detail. Objectives We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with salivary gland lesion (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 gland affected. IgG4-RD SG+ patients showed younger age at disease onset and diagnosis, and a longer interval between symptom onset and diagnosis. IgG4-RD SG+ group involved more female patients, and allergic diseases were more common in this group. In terms of organ involvement, 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 IgG4-RD SG- group. In addition, the serum IgG4 levels, IgG4/IgG ratio and IgE level were significantly higher in IgG4-RD SG+ patients. Patients with eosinophilia were more common in IgG4-RD SG+ group, while elevated ESR, CRP, and positive ANA were more common in 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.
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