BACKGROUND: @entity7271 ( @entity7271 ) is a rare autoimmune blistering condition. Treatment typically combines @entity288 with another immunosuppressive agent, such as @entity500 , @entity618 ( @entity2098 ) or rituximab. AIMS: To compare these second agents for their clinical efficacy and @entity134 -sparing effect METHODS: This was a single centre, retrospective observational cohort study of 21 @entity1 with oral @entity7271 over a six-year period, 18 of whom were newly diagnosed. Of the latter, the first 13 were initially given @entity500 , progressing to @entity2098 and then rituximab on treatment failure, whilst the next five @entity1 started directly on @entity2098 . RESULTS: Of the 13 newly diagnosed @entity1 , 2/13 were intolerant of @entity500 and only 1/11 was controlled, with a median time to treatment failure (MTTF) of 254 days. @entity2098 was given to 17 @entity1 , either de novo (5) or after @entity500 (12), and was significantly more effective, controlling activity in 4/17 @entity1 , and for a significantly longer time (MTTF 395 days, p=0.019). All 13 @entity1 failing @entity2098 received rituximab, seven required a second dose, and three, a third dose. All @entity1 responded, with 11/13 able to cease @entity230 . Control was maintained for a similar time to @entity2098 (MTTF 364 days, p=ns). @entity2408 also had the best steroid-sparing effect followed by @entity2098 , then @entity500 . Side effects were common with @entity500 whilst the other two agents were well-tolerated. CONCLUSION: Rituximab was the most effective of the three immunosuppressives for @entity7271 , although repeat dosing was frequently required. These observations have significant implications for the choice of drugs for this condition.

Treatment Outcomes in a Cohort of @entity1 with Mucosal-predominant XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity2408 :: ('CHEBI:64357', 'Chemical') :: ['Rituximab'] @entity500 :: ('MESH:D001379', 'Chemical') :: ['azathioprine'] @entity7271 :: ('MESH:D010392', 'Disease') :: ['PV', 'Pemphigus Vulgaris'] @entity134 :: ('CHEBI:35341', 'Chemical') :: ['steroid'] @entity2098 :: ('CHEBI:8764', 'Chemical') :: ['MMF'] @entity618 :: ('MESH:C063008', 'Chemical') :: ['mycophenolate mofetil'] @entity230 :: ('MESH:D013256', 'Chemical') :: ['steroids'] @entity288 :: ('MESH:D000305', 'Chemical') :: ['corticosteroids']
@entity7271 :: (MESH:D010392,Disease) :: ['Pemphigus Vulgaris']
