The natural history of pediatric-onset XXXX .

@entity1 @entity66 @entity1910 @entity75 @entity1252 @entity9821 @entity262 @entity1503 @entity854
BACKGROUND: @entity9821 ( @entity9821 ) is rare. The risk of progression to @entity1252 ( @entity1252 ) is uncertain. OBJECTIVE: We sought to determine the risk of progression of pediatric @entity9821 to @entity1252 and to characterize its phenotype. METHODS: This was a retrospective review of 40 @entity1 with @entity9821 . RESULTS: Six (15%) of 40 @entity1 presented with @entity9821 as a manifestation of concurrent @entity1252 . Of the remaining 34, 9 (26%) eventually met @entity1252 criteria and 15 (44%) developed @entity854 without meeting @entity1252 criteria. Only 10 (29%) maintained skin-limited disease. The average age at progression to @entity1252 was 11 years, with greatest risk in the first year after @entity9821 diagnosis. Most (89%) @entity1 with @entity1252 met diagnostic criteria with @entity66 ( @entity75 , @entity1910 , oral and nasal ulcers, photosensitivity), positive antibodies, and/or @entity1503 without developing end-organ damage over 5 years of median follow-up. LIMITATIONS: The study was retrospective. CONCLUSIONS: In pediatric @entity1 , @entity9821 carries a significant risk of progression to @entity1252 but may predict a milder phenotype of @entity262 . All @entity1 require careful monitoring for @entity1252 , particularly within the first year of diagnosis.
