Clinical outcome of generalized myasthenia gravis in Hong Kong Chinese.

ABSTRACT

BACKGROUND: Myasthenia gravis (MG) is an organ-specific autoimmune disease characterized by autoantibody-mediated impairment of skeletal muscle neuromuscular transmission. MG causes significant morbidity and even mortality. We studied the long-term clinical outcome of generalized MG (gMG) patients. METHODS: Records of Chinese gMG patients managed in Queen Mary Hospital from 1997 to 2012 were reviewed. Clinical, serological and radiological characteristics were studied for independent predictors of good long-term clinical outcome. RESULTS: A total of 123 Chinese gMG patients were studied. Their mean onset age was 44.8 years (range 7-83 years), 87 (70.7%) were female, and median follow-up duration was 114 months (interquartile range 67-188 months). Thymoma were detected in 45 patients (36.6%). Acetylcholine receptor autoantibodies were detected in 99 patients (87.6%). Ninety-three patients (75.6%) received immunosuppressant therapy (corticosteroid 75.6%, azathioprine 58.5%, mycophenolate mofetil 5.7%, cyclosporin 5.7%) and 77 (62.6%) received thymectomy. Thirty-five (28.5%) patients experienced MG crisis and two died. Ninety-six (78.0%) patients had good outcome defined by Myasthenia Gravis Foundation of America (MGFA) post-intervention status (PIS) of complete stable remission (CSR), pharmacological remission (PR) or minimal manifestation (MM) at latest follow-up, whereas 24 patients (19.5%) had intermediate outcome defined by MGFA PIS of Improved (I); 3 patients (2.4%) had poor prognosis defined by MGFA PIS of unchanged (U), worse (W), exacerbation (E) or died of MG (D). Azathioprine therapy was the only independent predictor of good outcome (OR 3.57, 95% CI 1.05-12.10, p=0.042). CONCLUSION: 78.0% of gMG patients had good long-term clinical outcome. Azathioprine therapy independently predicted good clinical outcome.