Involvement of two or more sets of lacrimal glands and/or major salivary glands is related to greater systemic disease activity due to multi-organ involvement in IgG4-related dacryoadenitis/sialadenitis
Involvement of two or more sets of lacrimal glands and/or major salivary glands is related to greater systemic disease activity due to multi-organ involvement in IgG4-related dacryoadenitis/sialadenitis
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DOI：
10.1080/14397595.2021.1878623
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发表时间：
2021-01-21
影响因子：
2.2
通讯作者：
Kawano, Mitsuhiro
中科院分区：
文献类型：
--
作者：
Takahashi, Yoshinori;Mizushima, Ichiro;Kawano, Mitsuhiro
在igg4相关性泪腺炎和/或涎腺炎（IgG4-DS）中，累及两组或多组泪腺（LGs）和/或大唾液腺（MSGs）被认为是具有诊断意义的特殊发现。本研究旨在阐明该因素对IgG4-DS整体临床表现的影响。方法回顾性分析130例igg4相关疾病患者的病历，其中97例诊断为IgG4-DS。我们根据>= 2组LGs和/或msg是否受损伤来确定他们的临床特征，并将结果与33例ds限制患者的结果进行比较。结果IgG4-DS患者男性60例，女性37例，中位年龄65岁。诊断时血清IgG4水平中位数为548 mg/dL。>= 2套患者（n = 44）受影响器官明显多于未受累患者（n = 53），血清C3和C4水平较低，血清IgG水平和IgG4-RD应答指数有升高趋势。在33例ds限制患者中，两组临床特征无显著差异。结论>= 2组LGs和/或msg受累提示全身性疾病活动性较大，主要表现为受累更多器官。
ObjectivesIn IgG4-related dacryoadenitis and/or sialadenitis (IgG4-DS), involvement of two or more sets of lacrimal glands (LGs) and/or major salivary glands (MSGs) is regarded as a specific finding with diagnostic significance. This study aimed to clarify the influence of this factor on the overall clinical picture of IgG4-DS.MethodsWe retrospectively reviewed the medical records of 130 patients with IgG4-related disease, 97 of whom were diagnosed with IgG4-DS. We determined their clinical features according to the presence/absence of involvement of >= 2 sets of LGs and/or MSGs and compared the results with those obtained in 33 DS-limited patients.ResultsThe IgG4-DS patients comprised 60 men and 37 women (median age 65 years). The median serum IgG4 level at diagnosis was 548 mg/dL. The patients with involvement of >= 2 sets (n = 44) had significantly more affected organs, lower serum C3 and C4 levels, and a tendency to have higher serum IgG levels and IgG4-RD responder index than did those without it (n = 53). In the 33 DS-limited patients, these two groups had no significant differences in clinical features.ConclusionsInvolvement of >= 2 sets of LGs and/or MSGs suggests greater systemic disease activity mainly reflected by involvement of more organs.