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硬皮病伴散在性红斑狼疮痕迹,合并桥本甲状腺炎及甲状腺乳头状癌(作者译)

[Scleroderma with traces of disseminated lupus erythematosus associated with Hashimoto's thyroiditis and papillary carcinoma of the thyroid gland (author's transl)].

作者信息

Sánchez de Rivera J M, Barbado Hernández F J, Vázquez Rodríguez J J, Gil Aguado A, García Puig J, Viguer García-Moreno J M

出版信息

Med Clin (Barc). 1979 Nov 10;73(8):342-6.

PMID:583290
Abstract

The coexistence of organ-specific and nonorgan-specific autoimmune diseases is an interesting phenomenon. A 52-year-old woman was admitted with fever, general discomfort, polyarthritis, and Raynaud's phenomenon. Physical examination revealed a goiter of stony consistency, hardening, paleness, and atrophy of the skin on the face and upper limbs, and blood hypertension (180/110 mmHg). The biological data included leukopenia, moderate anemia, and a very high sedimentation rate. The latex test was positive (+++); LE cells positive (+); hypergammaglobulinemia (3.5 g); antinuclear antibodies, 1/1280 with an immunofluorescence granular pattern; antithyroid antibodies, 1/160. There was pulmonary, renal, and gastrointestinal involvement compatible with scleroderma, which was confirmed by skin biopsy. A thyroidectomy revealed the existence of a papillary carcinoma with thyroiditis. Responde to treatment with immunosuppressive agents, hypotensive drugs, and thyroid substitution therapy was initially good. The patient was readmitted 8 months later with general discomfort and a severe hyperproteinemia (10 g/100 ml), including 65 percent gammaglobulin and requiring various sessions of plasmapheresis. The patient was discharged, but died suddenly 4 months later. The association of lupus and scleroderma in this patient is discussed and the possibility of its being a mixed connective tissue disease is discarded. The association of this condition with Hashimoto's thyroiditis, and the latter with papillary carcinoma of the thyroid are analyzed. The peculiar features of this case are pointed out. The authors postulate that the cause of the sudden death was a vascular cerebral complication induced by the extreme hyperproteinemia.

摘要

器官特异性和非器官特异性自身免疫性疾病并存是一种有趣的现象。一名52岁女性因发热、全身不适、多关节炎和雷诺现象入院。体格检查发现有坚硬如石的甲状腺肿、皮肤硬化、面色苍白以及面部和上肢皮肤萎缩,还有血压升高(180/110 mmHg)。生物学检查数据包括白细胞减少、中度贫血和血沉极快。乳胶试验呈阳性(+++);狼疮细胞阳性(+);高球蛋白血症(3.5 g);抗核抗体,免疫荧光颗粒型为1/1280;抗甲状腺抗体,1/160。存在与硬皮病相符的肺、肾和胃肠道受累情况,皮肤活检证实了这一点。甲状腺切除术显示存在伴有甲状腺炎的乳头状癌。最初对免疫抑制剂、降压药和甲状腺替代疗法的治疗反应良好。8个月后患者因全身不适和严重高蛋白血症(10 g/100 ml)再次入院,其中γ球蛋白占65%,需要多次进行血浆置换。患者出院,但4个月后突然死亡。讨论了该患者狼疮和硬皮病的关联,并排除了其为混合性结缔组织病的可能性。分析了这种情况与桥本甲状腺炎的关联,以及后者与甲状腺乳头状癌的关联。指出了该病例的特殊特征。作者推测猝死原因是极度高蛋白血症诱发的脑血管并发症。

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