Abstract
Henoch Schonlein purpura (HSP) is an immune mediated disease associated Immunoglobulin A (IgA) deposition within the affected organs. While the disease is commonly seen in the pediatric age group, it is rarely seen in adults. We report the case of a 93-year-old Caucasian lady who presented with nonthrombocytopenic purpuric rash and acute kidney injury after an episode of bronchitis. Rapid and progressive deterioration of renal function prompted a kidney biopsy, which showed findings consistent with IgA nephropathy confirming the diagnosis of HSP. The patient was treated with high dose intravenous methylprednisolone followed by oral prednisone; however, her kidney disease progressed to end stage renal disease requiring hemodialysis. HSP is usually a self-limiting disease in children. However, adults are at an increased risk of severe renal involvement including end stage renal disease. Purpuric skin rash with renal involvement should raise suspicion for HSP. This is the oldest known patient with HSP.
Highlights
Henoch Schonlein purpura, known as Immunoglobulin A vasculitis (IgAV) is a small vessel vasculitis associated with IgA deposition in the affected organs
Management guidelines for Henoch Schonlein purpura (HSP) nephritis in an adult remain obscure and more research is needed in this area
Renal transplant may be an option in patients who progress to end stage renal disease, HSP nephritis frequently recurs after renal transplantations [12]
Summary
Henoch Schonlein purpura, known as Immunoglobulin A vasculitis (IgAV) is a small vessel vasculitis associated with IgA deposition in the affected organs. HSP is primarily a disease of childhood with a peak incidence between 4 and 6 years of age [1]. It is rarely seen in adults with an incidence of 0.1–1.2 per million in adults over 20 years of age [2]. The American College of Rheumatology has identified 4 criteria for the diagnosis of HSP: age less than or equal to 20 years at disease onset, palpable purpura, acute abdominal pain, and biopsy showing granulocytes in the walls of small arterioles or venules. The presence of any 2 or more of these criteria distinguishes HSP from other forms of vasculitis with a sensitivity of 87.1% and a specificity of 87.7% [3]. Management guidelines for HSP nephritis in an adult remain obscure and more research is needed in this area
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