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báo cáo khoa học: " Successful low-dose azathioprine for myasthenia gravis despite hepatopathy from primary sclerosing cholangitis: a case report"

Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Successful low-dose azathioprine for myasthenia gravis despite hepatopathy from primary sclerosing cholangitis: a case report | Finsterer and Hoflich Journal of Medical Case Reports 2010 4 356 http www.jmedicalcasereports.eom content 4 1 356 jAc JOURNALOF medical ÌỤr case REPORTS CASE REPORT Open Access Successful low-dose azathioprine for myasthenia gravis despite hepatopathy from primary sclerosing cholangitis a case report Josef Finsterer Sonja Hoflich Abstract Introduction 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. Case presentation 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. Conclusion 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. Introduction Myasthenia gravis MG may be due to a genetic defect intoxication or most frequently autoimmune mechanisms 1 . Although autoimmune MG is frequently associated with other autoimmune disorders 2-13 to the best of our knowledge the association of MG with primary sclerosing cholangitis PSC has not been reported. Case presentation Our patient is a 73-year-old Caucasian woman with a history of elevated liver function