Clinical course and outcomes of childhood-onset granulomatosis with polyangiitis.
Year of Publication
2016 Oct 6
<p><strong>OBJECTIVES: </strong>To characterise the clinical course and outcomes of a cohort of children with granulomatosis with polyangiitis (GPA).</p>
<p><strong>METHODS: </strong>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.</p>
<p><strong>RESULTS: </strong>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.</p>
<p><strong>CONCLUSIONS: </strong>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.</p>
Clin. Exp. Rheumatol.