Severe erosive lesions in the digestive tract of patients with Henoch-Schönlein Purpura (HSP) and its impact on prognosis - presentation of two cases and statistical review of adult-onset Japanese HSP.

ABSTRACT

INTRODUCTION: Although many pediatric patients with Henoch-Schönlein Purpura (HSP) recover spontaneously, disease activity in adult patients often cannot be controlled by treatment. PURPOSE: To assess the specific signs not formerly considered to be those of uncontrollable adult HSP patients. PATIENTS AND METHODS: Clinical records of 2 adult patients who died during HSP were reviewed and previous reports on HSP were consulted. RESULTS: Both patients had lesions in the digestive tract diagnosed as hemorrhagic erosion in the small intestine and colon. They were elderly and showed renal dysfunction. They died from severe infection after potent immunosuppressive treatment. A univariate analysis showed that age of over 60 years, severe renal symptoms (nephrotic syndrome and/or end-stage renal failure), Birmingham Vasculitis Activity Score (BVAS) of more than 18 points, massive immunosuppression and melena had significantly higher prevalence among patients who died. Multivariate statistical analysis with theoretical quantification analysis II revealed that age of over 60 and severe renal symptoms (nephrotic syndrome and/or end-stage renal failure) contributed to poor prognosis. The presence of melena did not contribute to poor prognosis despite results of the univariate analysis and our clinical impressions. DISCUSSION: In multivariate statistical analysis, melena was selected as a sign of severe erosive lesions in the digestive tract because some of the patients were not examined by fiberscopy. Melena is caused by various lesions in the digestive tract and each of them has different effects on prognosis. CONCLUSION: Elderly HSP patients with severe renal impairment should be carefully treated. Examination of the digestive tract by fiberscopy is recommended when melena is observed in these patients.