Bartalena Luigi, Fatourechi Vahab
Department of Clinical and Experimental Medicine, University of Insubria, Endocrine Unit, Ospedale di Circolo, Viale Borri, 57, 21100, Varese, Italy.
Department of Endocrinology and Metabolism, Mayo Clinic, Rochester, MN, USA.
J Endocrinol Invest. 2014 Aug;37(8):691-700. doi: 10.1007/s40618-014-0097-2. Epub 2014 Jun 10.
Graves' orbitopathy (GO), thyroid dermopathy (also called pretibial myxedema) and acropachy are the extrathyroidal manifestations of Graves' disease. They occur in 25, 1.5, and 0.3 % of Graves' patients, respectively. Thus, GO is the main and most common extrathyroidal manifestation. Dermopathy is usually present if the patient is also affected with GO. The very rare acropachy occurs only in patients who also have dermopathy. GO and dermopathy have an autoimmune origin and are probably triggered by autoimmunity to the TSH receptor and, likely, the IGF-1 receptor. Both GO and dermopathy may be mild to severe.
Mild GO usually does not require any treatment except for local measures and preventive actions (especially refraining from smoking). Currently, moderate-to-severe and active GO is best treated by systemic glucocorticoids, but response to treatment is not optimal in many instances, and retreatments and use of other modalities (glucocorticoids, orbital radiotherapy, cyclosporine) and, in the end, rehabilitative surgery are often needed. Dermopathy is usually managed by local glucocorticoid treatment. No specific treatment is available for acropachy.
Novel treatments are presently being investigated for GO, and particular attention is paid to the use of rituximab. It is unknown whether novel treatments for GO might be useful for the other extrathyroidal manifestations. Future novel therapies shown to be beneficial for GO in randomized studies may be empirically used for dermopathy and acropachy.
格雷夫斯眼眶病（GO）、甲状腺皮肤病（也称为胫前黏液性水肿）和杵状指是格雷夫斯病的甲状腺外表现。它们分别发生在25%、1.5%和0.3%的格雷夫斯病患者中。因此，GO是主要且最常见的甲状腺外表现。如果患者同时患有GO，则通常会出现皮肤病。非常罕见的杵状指仅发生在同时患有皮肤病的患者中。GO和皮肤病具有自身免疫起源，可能是由针对促甲状腺激素受体以及可能的胰岛素样生长因子-1受体的自身免疫引发的。GO和皮肤病都可能从轻到重。
轻度GO通常除了局部措施和预防行动（特别是戒烟）外不需要任何治疗。目前，中度至重度活动性GO最好用全身性糖皮质激素治疗，但在许多情况下对治疗的反应并不理想，并且经常需要再次治疗以及使用其他方式（糖皮质激素、眼眶放疗、环孢素），最终还需要进行康复手术。皮肤病通常通过局部糖皮质激素治疗。对于杵状指没有特效治疗方法。
目前正在研究针对GO的新疗法，尤其关注利妥昔单抗的使用。尚不清楚针对GO的新疗法是否对其他甲状腺外表现有用。在随机研究中显示对GO有益的未来新疗法可能会凭经验用于皮肤病和杵状指。
