Pediatric-onset XXXX : clinical features and short-term outcome.

@entity1 @entity101 @entity12040 @entity613 @entity393
UNASSIGNED: The aim of this was to assess clinical features and outcome in pediatric-onset @entity12040 ( @entity12040 ). Retrospective data analysis of @entity1 diagnosed with @entity12040 over last 13  years with onset before 18  years of age was done. Their presenting features, activity (by NIH criteria, ITAS2010, ITAS-A), disease extent (by DEI.Tak) and angiographic findings were retrieved from clinic files. Treatment received and follow-up data on disease activity and damage by @entity12040 damage score (TADS) were also analyzed. Wherever repeated angiography data were available, the same was analyzed. Values are expressed as median with interquartile range in brackets. There were 29 @entity1 (19 females) with median age at diagnosis of 14 (13-16) years and delay to diagnosis of 1 (0.4-2)  year. Common presenting symptoms were @entity393 (23/29) and @entity101 (22/29). @entity1 had extensive disease at presentation with median DEI.Tak of 12 (9.5-15); 23/29 had elevated acute-phase reactants, and 28/29 were active at presentation [median ITAS2010 13 (8-15.5), ITAS-A 14 (10-17)]. Numano's type V was the commonest angiographic type (22/29). At a median follow-up of 2.4 (1.5-5.1) years, 2/20 were active whereas all had sustained damage despite a majority (17/20) being on immunosuppression. The median TADS was 8 (6.3-9.8) with @entity393 , @entity613 and @entity101 being the commonest damage item. Two needed renal artery stenting to control @entity101 . Angiographic assessment at least 2  years apart demonstrated disease progression in 5 of 6 @entity1 despite immunosuppression. Significant damage accrued on follow-up despite immunosuppression and control of disease activity. @entity101 remains the major long-term morbidity.
