Sasaki Takanori, Akiyama Mitsuhiro, Kaneko Yuko, Mori Takehiko, Yasuoka Hidekata, Suzuki Katsuya, Yamaoka Kunihiro, Okamoto Shinichiro, Takeuchi Tsutomu
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
Division of Hematology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
RMD Open. 2017 Jul 18;3(1):e000432. doi: 10.1136/rmdopen-2017-000432. eCollection 2017.
Differentiating IgG4-related disease (IgG4-RD) from multicentric Castleman's disease (MCD) is challenging because both diseases present high serum IgG4. The objective of this study is to clarify the differences in characteristics and identify a clinically useful approach to differentiate these two diseases.
Forty-five consecutive patients with untreated active IgG4-RD and 33 patients with MCD were included in this study, who visited our institution from January 2000 to August 2016. The clinical and laboratory findings for the patients of the two diseases were compared. Various combinations of the distinctive findings were evaluated to identify the most efficient differentiating features between IgG4-RD and MCD.
The levels of serum IgG4 were not different between the two diseases. Orbits, lacrimal glands, salivary glands or pancreas were involved in 88.9% of IgG4-RD cases and only in 3.0% of MCD cases. All MCD cases involved lymph nodes. Atopic history was characteristic for IgG4-RD. The levels of C reactive protein (CRP) with a cut-off of 0.80 mg/dL and IgA with a cut-off of 330 mg/dL were the most distinctive. The combination of 'Orbits, lacrimal glands, salivary glands or pancreas involvement, atopic history, or non-involvement of lymph node' and 'CRP ≤ 0.8 mg/dL or IgA ≤ 330 mg/dL' yielded the probability of 97.8% in IgG4-RD, while that of 3.0 % in patients with MCD.
Our study revealed distinct features between IgG4-RD and MCD. Differentiating between the diseases based on those distinct features, including distribution of organ involvement, atopic history, levels of IgA and CRP, was a useful approach.
将IgG4相关疾病（IgG4-RD）与多中心Castleman病（MCD）区分开来具有挑战性，因为这两种疾病均表现为血清IgG4水平升高。本研究的目的是阐明两者特征上的差异，并确定一种临床上有用的方法来区分这两种疾病。
本研究纳入了45例未经治疗的活动性IgG4-RD连续患者和33例MCD患者，这些患者于2000年1月至2016年8月期间就诊于我院。比较了这两种疾病患者的临床和实验室检查结果。评估了各种独特发现的组合，以确定IgG4-RD和MCD之间最有效的鉴别特征。
两种疾病的血清IgG4水平无差异。88.9%的IgG4-RD病例累及眼眶、泪腺、唾液腺或胰腺，而MCD病例仅3.0%累及这些部位。所有MCD病例均累及淋巴结。特应性病史是IgG4-RD的特征。C反应蛋白（CRP）水平截断值为0.80mg/dL以及IgA水平截断值为330mg/dL时最具鉴别性。“眼眶、泪腺、唾液腺或胰腺受累、特应性病史或无淋巴结受累”与“CRP≤0.8mg/dL或IgA≤330mg/dL”的组合在IgG4-RD中的概率为97.8%，而在MCD患者中为3.0%。
我们的研究揭示了IgG4-RD和MCD之间的不同特征。基于这些不同特征，包括器官受累分布、特应性病史、IgA和CRP水平来区分这两种疾病是一种有用的方法。
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