Clinical outcome of generalized XXXX in Hong Kong Chinese.

@entity1 @entity704 @entity500 @entity1737 @entity205 @entity618 @entity792 @entity934
BACKGROUND: @entity1737 ( @entity1737 ) is an organ-specific @entity792 characterized by autoantibody-mediated impairment of skeletal muscle neuromuscular transmission. @entity1737 causes significant morbidity and even mortality. We studied the long-term clinical outcome of generalized @entity1737 (gMG) @entity1 . METHODS: Records of Chinese gMG @entity1 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 @entity1 were studied. Their mean onset age was 44.8years (range 7-83years), 87 (70.7%) were female, and median follow-up duration was 114months (interquartile range 67-188months). @entity704 were detected in 45 @entity1 (36.6%). @entity934 receptor autoantibodies were detected in 99 @entity1 (87.6%). Ninety-three @entity1 (75.6%) received immunosuppressant therapy (corticosteroid 75.6%, @entity500 58.5%, @entity618 5.7%, @entity205 5.7%) and 77 (62.6%) received thymectomy. Thirty-five (28.5%) @entity1 experienced @entity1737 crisis and two died. Ninety-six (78.0%) @entity1 had good outcome defined by @entity1737 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 @entity1 (19.5%) had intermediate outcome defined by MGFA PIS of Improved (I); 3 @entity1 (2.4%) had poor prognosis defined by MGFA PIS of unchanged (U), worse (W), exacerbation (E) or died of @entity1737 (D). @entity500 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 @entity1 had good long-term clinical outcome. @entity500 therapy independently predicted good clinical outcome.
