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Treatment | Evidence of efficacy in generalised JMG | Reference |
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Acetylcholinesterase inhibitors | First line therapy.May be sufficient in ocular JMG or mild generalised JMG | Skeie et al., 2010 [29] |
Thymectomy | Recommended to increase remission rates in postpubertal, seropositive children. Not recommended in prepubertal children | Hennessey et al., 2011 [30] Rodriguez et al., 1983 [31] Tracy et al., 2009 [32] Lindner et al., 1997 [20] |
Steroids | Often used in combination with steroid sparing agents. Significant side-effect profile if used long-term at high dose | Schneider-Gold et al., 2005 Cochrane review: one JMG study, others adult or unspecified age ranges [33] Zhang et al., 1998 [6] |
Steroid sparing agents | | |
Azathioprine | Usually used in combination with corticosteroids. Occasionally used alone. | Mertens et al., 1981 [34] includes some children but no subgroup analysis Gold et al., 2008 [35] Palace et al., 1998 [36] adult study |
Cyclosporin A | As monotherapy or in conjunction with corticosteroids | Tindall et al., 1987 [37] adult study Hart et al., 2009 (Cochrane) [38] series include some children |
Cyclophosphamide | | De Feo et al., 2002 [39] adult population Hart et al., 2009 [38] (Cochrane) series include some children |
Tacrolimus | | Furukawa et al., 2008 [40] Ponseti et al., 2008 [41] both include post pubertal children Ishigaki et al., 2009 [42] prepubertal female |
MMF | | Hehir et al., 2010 [43] includes some peri-/postpubertal children |
Rituximab | | Wylam et al., 2003 [44] pediatric case |
IVIG/Plasma exchange | | Selcen et al., 2000 [45] Gajados et al., 2008, Cochrane database [46] |
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