Initial Hematochezia and Kidney Involvement are Important Prognostic Factors of Adult Onset Henoch-Schönlein Purpura in Korea

Abstract

Objective: Henoch-Schönlein purpura (HSP) is a systemic vasculitis, characterized by small-vessel leukocytoclastic vasculitis with the deposition of immune complexes containing IgA. It is the most common acute vasculitic disorder affecting children but is relatively uncommon in adults. We investigated the clinical features and factors affecting the prognosis of adult HSP in Korea.

Methods: From 1996 to 2011 seventy patients over 15 years of age with HSP were retrospectively analyzed.

Results: Thirty eight patients (54.3%) were female and the age at disease onset ranged from 15 to 75 years (35.0±15.8 years). Purpuric skin rash was observed in all patients and arthralgia was present in 34 patients (48.6%). GI symptoms and kidney involvements were observed in 28 patients (40.0%) and 34 patients (48.6%), respectively. Complete remission was achieved in 46 patients (65.7%). The remission group showed a lower incidence of hematochezia (p=0.044), hematuria (p=0.008), and proteinuria (p=0.011) at diagnosis than the no remission group. About 10% of adult HSP patient progressed to chronic kidney disease (CKD), which showed higher a incidence of nephrotic range proteinuria. Only nephrotic range proteinuria at diagnosis was a significant risk factor for CKD (OR= 16.7, p=0.008, 95% CI=2.1∼133.1).

Conclusion: Hematochezia, hematuria and proteinuria at the diagnosis of HSP are important prognostic factors in predicting remission. In addition, HSP patients with nephrotic range proteinuria at diagnosis have an increased risk of renal failure.