Successful Low-dose Azathioprine for Myasthenia Gravis Despite Hepatopathy from Primary Sclerosing Cholangitis: A Case Report

Josef Finsterer; Sonja Höflich

Disclosures

J Med Case Reports. 2010;4(360) 

In This Article

Abstract and Introduction

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.

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