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[1例伴有淋巴结病和IgG4高球蛋白血症的干燥综合征]

[A case of Sjögren's syndrome accompanied by lymphadenopathy and IgG4 hypergammaglobulinemia].

作者信息

Suzuki S, Kida S, Ohira Y, Ohba T, Miyata M, Nishimaki T, Morito T, Kasukawa R, Hojyo H, Wakasa H

机构信息

Department of Internal Medicine II, Fukushima Medical College.

出版信息

Ryumachi. 1993 Jun;33(3):249-54.

PMID:8346468
Abstract

A 78-year-old male patient suffered from slight dryness of his mouth and eyes, which was followed by swelling of the parotid and submandibular glands on both sides. Subsequently, he developed generalized lymphadenopathy accompanied by hypergammaglobulinemia IgG 7740 mg/dl, main subclass: IgG4, 5800 mg/dl. Histopathological examination of lymph nodes from his left neck showed follicular proliferation of plasma cells containing cytoplasmic kappa and lambda chains of IgG4, without destruction of the lymph node structure. His serum showed very low levels of complement: 0 U/ml of CH 50, 14 mg/ml of C3 and less than 11 mg/ml of C4. During treatment with prednisolone at 30 mg/day, the swelling of the salivary glands and lymph nodes reduced the IgG and IgG4 decreased and the complement increased. Finally, all lymph nodes, IgG and complement abnormalities were normalized. Anticomplementariness of IgG4 was demonstrated in this patient.

摘要

一名78岁男性患者出现轻微口干、眼干,随后双侧腮腺和颌下腺肿大。随后,他出现全身淋巴结病,并伴有高球蛋白血症,IgG为7740mg/dl,主要亚类:IgG4为5800mg/dl。对其左颈部淋巴结进行组织病理学检查,结果显示含有IgG4胞质κ和λ链的浆细胞呈滤泡性增生,淋巴结结构未被破坏。他的血清补体水平极低:CH 50为0U/ml,C3为14mg/ml,C4低于11mg/ml。在每天服用30mg泼尼松龙治疗期间,唾液腺和淋巴结的肿胀减轻,IgG和IgG4下降,补体增加。最后,所有淋巴结、IgG和补体异常均恢复正常。该患者证实存在IgG4的抗补体性。

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