Orija Israel B, Gupta Manjula, Zimmerman Robert S
Atlanta Medical Center, Atlanta, Georgia 30312-1212, USA.
Endocr Pract. 2005 Jul-Aug;11(4):259-64. doi: 10.4158/EP.11.4.259.
To report an association between two autoimmune conditions, Graves' disease and stiff-person (stiff-man) syndrome, and discuss the relevant literature.
We present a case of a 52-year-old white woman with stiff-person syndrome who also had Graves' disease, discuss her management, and review the related literature. Pertinent published reports from 1950 through 2004 were researched with use of MEDLINE and PubMed, and cross-references to other articles were reviewed.
A 52-year-old white woman presented with symptoms of hyperthyroidism due to Graves' disease. Laboratory data were as follows: thyrotropin <0.005 m IU/mL, thyroxine 11.1 microg/dL, free thyroxine index (FTI) 10.7, and triiodothyronine 170 ng/dL. Thyroid-stimulating immunoglobulins (TSI) and thyrotropin-binding inhibitory immunoglobulins (TBII) were positive at 1,986% and 82.5 U/L, respectively. The hyperthyroidism was treated with propranolol. She had a long-standing history of musculoskeletal complaints and was ultimately diagnosed with stiff-person syndrome. During her thyroid evaluation, she had severe neurologic deterioration that necessitated hospitalization and treatment with clonazepam, baclofen, intravenous immunoglobulin, and subsequently prednisone and azathioprine for appreciable symptomatic relief. The aggressive immunosuppression had a profound effect on her symptoms of hyperthyroidism, results of thyroid function tests, and thyrotropin receptor antibodies (TRABs). Thyrotropin was 0.52 microIU/mL, thyroxine was 6.9 microg/dL, and FTI was 5.7. The TSI decreased from 1,986% to 248%, and her TBII normalized from 82.5 U/L to <5 U/L. She was clinically and biochemically euthyroid at last follow-up in May 2004.
This case illustrates the association between TRAB-positive Graves' disease and stiff-person syndrome and the improvement of Graves' disease with immunosuppressive therapy.
报告两种自身免疫性疾病——格雷夫斯病(Graves病)和僵人综合征之间的关联,并讨论相关文献。
我们报告一例52岁患有僵人综合征的白人女性病例,该患者同时患有格雷夫斯病,讨论其治疗情况,并回顾相关文献。利用MEDLINE和PubMed检索了1950年至2004年的相关已发表报告,并查阅了其他文章的交叉引用文献。
一名52岁白人女性因格雷夫斯病出现甲状腺功能亢进症状。实验室检查数据如下:促甲状腺激素<0.005 mIU/mL,甲状腺素11.1 μg/dL,游离甲状腺素指数(FTI)10.7,三碘甲状腺原氨酸170 ng/dL。促甲状腺素受体刺激性免疫球蛋白(TSI)和促甲状腺素结合抑制性免疫球蛋白(TBII)分别为1986%和82.5 U/L,均呈阳性。甲状腺功能亢进症采用普萘洛尔治疗。她有长期的肌肉骨骼不适病史,最终被诊断为僵人综合征。在甲状腺评估期间,她出现严重的神经功能恶化,需要住院治疗,并使用氯硝西泮、巴氯芬、静脉注射免疫球蛋白,随后使用泼尼松和硫唑嘌呤以获得明显的症状缓解。积极的免疫抑制治疗对她的甲状腺功能亢进症状、甲状腺功能检查结果以及促甲状腺素受体抗体(TRABs)产生了深远影响。促甲状腺激素为0.52 μIU/mL,甲状腺素为6.9 μg/dL,FTI为5.7。TSI从1986%降至248%,TBII从82.5 U/L恢复正常至<5 U/L。在2004年5月的最后一次随访中,她的临床和生化指标均处于甲状腺功能正常状态。
本病例说明了TRAB阳性的格雷夫斯病与僵人综合征之间的关联,以及免疫抑制治疗对格雷夫斯病的改善作用。