Finsterer Josef, Höflich Sonja
Krankenanstalt Rudolfstiftung, Juchg, 25, 1030, Vienna, Austria.
J Med Case Rep. 2010 Nov 8;4:356. doi: 10.1186/1752-1947-4-356.
Although myasthenia gravis is frequently associated with other disorders, it has not been reported together with primary sclerosing cholangitis, complicating the administration of liver-toxic immunosuppressive therapy.
A 73-year-old Caucasian woman with a history of arterial hypertension, thyroid dysfunction, glaucoma, right-sided ptosis and later generalized weakness, was diagnosed with myasthenia gravis. Additionally, primary sclerosing cholangitis was detected, initially prohibiting the administration of immunosuppressants. Despite treatment with steroids and pyridostigmine she repeatedly experienced myasthenic crises. After the fifth crisis and after antibody titers had reached levels > 100 nmol/L during two years of follow-up, it was decided to restart azathioprine. Interestingly, low-dose azathioprine (1.5 mg/kg/day) was well tolerated, had a positive clinical and immunological effect and did not worsen primary sclerosing cholangitis.
Myasthenia gravis may occur together with primary sclerosing cholangitis in the same patient. Mild immunosuppression with azathioprine is feasible and effective in such a patient, without worsening myasthenia gravis or primary sclerosing cholangitis.
虽然重症肌无力常与其他疾病相关，但尚未有与原发性硬化性胆管炎同时出现的报道，这使得肝毒性免疫抑制治疗的管理变得复杂。
一名73岁的白种女性，有动脉高血压、甲状腺功能障碍、青光眼病史，右侧上睑下垂，随后出现全身无力，被诊断为重症肌无力。此外，还检测出原发性硬化性胆管炎，最初禁止使用免疫抑制剂。尽管使用了类固醇和吡啶斯的明治疗，她仍反复出现重症肌无力危象。在第五次危象后，且在两年随访期间抗体滴度达到>100 nmol/L后，决定重新开始使用硫唑嘌呤。有趣的是，低剂量硫唑嘌呤（1.5 mg/kg/天）耐受性良好，具有积极的临床和免疫学效果，且未使原发性硬化性胆管炎恶化。
重症肌无力可能与原发性硬化性胆管炎在同一患者中同时出现。对于此类患者，使用硫唑嘌呤进行轻度免疫抑制是可行且有效的，不会加重重症肌无力或原发性硬化性胆管炎。
