Clinical course and outcomes of childhood-onset granulomatosis with polyangiitis.: Influence Statistics


Clinical course and outcomes of childhood-onset granulomatosis with polyangiitis.


Rui Xiao Peter A Merkel Pamela F Weiss Karen E James

Expert Impact

Concepts for which they have has direct influence: Polyangiitis gpa , Onset granulomatosis , Granulomatosis polyangiitis , Diagnosed gpa , Retrospective cohort study , Age onset , Female granulomatosis .

Key People For Polyangiitis Gpa

Top KOLs in the world
Gary S Hoffman
takayasu arteritis wegener granulomatosis polyangiitis humans
Loic P Guillevin
polyarteritis nodosa plasma exchange systemic sclerosis
Peter A Merkel
outcome measures systemic sclerosis plasma exchange
Cees G M Kallenberg
systemic lupus erythematosus antineutrophil cytoplasmic apoptotic cells
Alfred Daniel Mahr
plasma exchange giant cell arteritis microscopic polyangiitis
Wolfgang Ludwig Gross
wegeners granulomatosis polyangiitis humans pancreatic cancer

Clinical course and outcomes of childhood-onset granulomatosis with polyangiitis.


. OBJECTIVES: To characterise the clinical course and outcomes of a cohort of children with granulomatosis with polyangiitis (GPA). METHODS: Retrospective cohort study of children diagnosed with GPA in a tertiary care facility from 2000-2014. All subjects met the American College of Rheumatology 1990 criteria for GPA or the 2008 European League against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society criteria for GPA. Predictors of readmission were determined using univariate logistic regression. Kaplan-Meier analysis was used to demonstrate the relapse-free survival probability in follow-up. RESULTS: Twenty-eight children (median age 14.7 years) were diagnosed during the study period. At presentation 14 (50%), 5 (18%), and 4 (14%) children required intensive care unit care, ventilator support, and dialysis, respectively. One-third of the children in our cohort had gastrointestinal involvement, one-quarter of whom were previously diagnosed with inflammatory bowel disease. Two-thirds of children were readmitted. Renal failure and infections accounted for most readmissions. Twenty-three (85%) patients achieved remission, however, 11 subsequently flared (median time to flare 21.5 months). Haematuria at diagnosis was significantly associated with readmission (OR 6.25). At a median follow-up of 3.3 years (range 5 months to 6 years) 10 (37%) children had chronic kidney disease (> stage 2) and none of the children died. CONCLUSIONS: Children with GPA frequently have severe disease presentations including significant renal, respiratory and gastrointestinal involvement. While most children with GPA achieve remission, nearly half have subsequent relapses.