Background The EULAR/PRINTO/PRES criteria for Henoch–Schönlein purpura (HSP) was published in 20101. However, Asian patients participating in a study of the new criteria account for only 3% (26/827) of cases2 and adult HSP has yet to be evaluated using the criteria.
Objectives To estimate the clinical characteristics and long-term outcomes of Japanese pediatric and adult patients with HSP that fulfill the criteria.
Methods We retrospectively examined data from pediatric cases (aged <18 years) and adult cases (aged ≥18 years) that met the criteria, as well as clearly had typical leukocytoclastic vasculitis with predominant IgA deposit as determined on skin biopsy in adult cases. All patients were treated at Kitasato University Hospital, Japan between January 2001 and December 2011. We statistically analyzed the clinical characteristics and long-term outcomes of the two groups.
Results We identified 220 patients with HSP according to the criteria. Of the 220 patients, 86 were children (42 boys, 44 girls) and 110 were adults (47 men, 63 women). Mean (SD) follow up was 18.5±22.5 months for pediatric cases and 24.4±28.5 months for adult cases. Mean age at diagnosis was 7.2±3.8 years (42 aged <7 years, 44 aged ≥7 years) for pediatric cases and 51.3±18.2 years (78 aged <65 years, 32 aged ≥65 years) for adult cases. Purpura was widespread or localized in 38 and 48 pediatric cases and 49 and 61 adult cases, respectively. Correspondingly, recurrent and/or prolonged (>1 month) purpura was seen in 38 (44%) and 59 (54%) of pediatric and adult cases, respectively, nephropathy in 52 (60%) and 86 (78%) cases, respectively, and digestive symptoms in 53 (62%) and 31 (28%) cases, respectively. The Kaplan Meier method was used to analyze the duration of symptom improvement over 72 months by age, sex, purpura area, recurrent and/or prolonged purpura, nephropathy, and digestive symptoms. Duration was significantly prolonged in those aged ≥7 years (p=0.0033), those with recurrent and/or prolonged purpura (p=0.0091), and those with nephropathy (p<0.0001) among the pediatric cases. On the other hand, among the adult cases, duration was significantly prolonged in those with recurrent and/or prolonged purpura (p=0.0063), those with nephropathy (p<0.0001), and those with digestive symptoms (p=0.0472). Factors significantly influencing the duration, determined using the Cox proportional hazards model, were having recurrent and/or prolonged purpura (hazard ratio (HR) 1.75, 95% confidence interval (CI) 1.07–2.91, p=0.027), and nephropathy (HR 3.81, 95% CI 2.21–6.61, p<0.0001) among pediatric cases, and having recurrent and/or prolonged purpura (HR 2.42, 95% CI 1.46–4.00, p=0.0006), nephropathy (HR 3.60, 95% CI 2.08–6.08, p<0.0001), and digestive symptoms (HR 1.74, 95% CI 1.02–3.10, p=0.0417) among the adult cases.
Conclusions The findings reveal that recurrent and/or prolonged purpura for >1 month and nephropathy are independent factors influencing the prognosis of both pediatric and adult HSP. Digestive symptoms were also considered to be an influencing factor in adult HSP.
Seza Ozen, Angela Pistorio, Silvia M Iusan, et al. Ann Rheum Dis 2010; 69: 798–806. doi:10.1136/ard.2009.116657
Nicolino Ruperto, Seza Ozen, Angela Pistorio, et al. Ann Rheum Dis 2010; 69: 790–797. doi:10.1136/ard.2009.116624
Disclosure of Interest None Declared