@entity17758 ( @entity17758 ) is an idiopathic @entity1333 confined to the central nervous system. In @entity1 with @entity17758 , small-vessel (SV) involvement is characterized clinically by progressive @entity16 , @entity75 on brain imaging, occasional pseudo- @entity5 presentation, and normal angiogram results in most @entity1 . Small case series of @entity1 with SV @entity17758 with short follow-up usually reveal favorable outcomes in @entity1 treated with immunosuppressive therapy. We report here the cases of 3 @entity1 with biopsy-confirmed SV @entity17758 and long-term follow-up who developed different patterns of @entity16 despite immunosuppressive therapy. One @entity1 had transient @entity63 attacks shortly after initiation of @entity228 treatment. Early @entity63 events probably result from residual thrombogenicity or residual inflammation of recently affected vessels, which supports the use of antiplatelet agents and suggests potential benefits of stronger immunosuppressive therapy. In contrast, the other 2 @entity1 had later @entity16 after @entity228 withdrawal, which suggests failure of initial immunosuppressant treatment and the need for stronger agents, longer treatment duration, or both. All @entity1 responded to long-term treatment with @entity288 combined with cytotoxic agents. This particular combination is probably indicated in many cases of SV @entity17758 , including those with @entity16 that occurs during maintenance corticotherapy or after @entity228 withdrawal. In 1 case, SV @entity17758 recurred several years after discontinuation of combination therapy. Long-term relapses may reflect intrinsic predispositions to SV @entity17758 rather than treatment failure. These cases highlight different chronological patterns of @entity16 despite immunosuppressive therapy, which supports the relevance of monitoring clinical, laboratory, and radiologic responses to treatment and of long-term follow-up of @entity1 with SV @entity17758 .

XXXX : @entity16 despite treatment.

@entity1 :: ('9606', 'Species') :: ['children', 'patients', 'patient'] @entity1333 :: ('MESH:D014657', 'Disease') :: ['vasculitis'] @entity75 :: ('MESH:D001927', 'Disease') :: ['multifocal lesions'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neurologic symptoms', 'neurologic deterioration'] @entity63 :: ('MESH:D007511', 'Disease') :: ['ischemic'] @entity228 :: ('CHEBI:50858', 'Chemical') :: ['corticosteroid'] @entity17758 :: ('MESH:C535276', 'Disease') :: ['PACNS', 'Primary angiitis of the central nervous system'] @entity288 :: ('MESH:D000305', 'Chemical') :: ['corticosteroids']
@entity17758 :: (MESH:C535276,Disease) :: ['Primary angiitis of the central nervous system']
