Case Presentation:

Henoch–Schönlein purpura (HSP), an IgA mediated systemic vasculitis, presents with a tetrad of lower extremity purpuric rash, bowel angina, acute nephritis and arthritis. HSP is mostly seen in the pediatric population, but rarely also reported in adults with a much lower incidence of (3.4 to 14 cases per million). Hence less is known about the natural course and precipitants in the adult population. Here we present a young female who developed HSP nephritis following staph aureus bacteremia.

Discussion:

A 34–year–old female presented with upper back pain and fever. She was seen for MSSA skin and soft tissue infection that was treated with bactrim. Admission blood cultures revealed MSSA bacteremia prompting further evaluation of the back pain with imaging which revealed T11–12 epidural abscess without clinical and radiological spinal cord involvement. She was started on Vancomycin, which was later switched to daptomycin due to suspected drug reaction. Despite the change in antibiotics she continued to develop progressive lower extremity purpuric rash, marked edema, acute nonoliguric renal failure with glomerular hematuria and proteinuria. She underwent six sessions of hemodialysis for refractory and recalcitrant edema. Renal biopsy was performed due to suspicion of systemic illness given unexplained acute renal failure with active urinary sediments, marked edema and purpuric skin lesions; this revealed marked mesangial expansion, crescent formation (Figure 1) and mesangial IgA deposits (Figure 2) suggestive of systemic IgA nephritis consistent with Henoch Schonlein Purpura. HSP is a systemic leukocytoclastic vasculitis with multi organ involvement. Adult manifestations of HSP include a purpuric rash in 96%, arthritis in 61%, GI disease in 48% and renal disease in 32% cases. Renal disease results in proteinuria in 99% cases and hematuria in 93% cases. One third of cases with renal involvement are at risk progression to renal insufficiency, with severity related to the histological severity and persistence of renal failure with proteinuria for >6 months. Skin biopsy is helpful in the diagnosis, and should be undertaken preferentially from sites that are < 24 h old which typically shows leukocytoclastic vasculitis along with IgA deposits.

Conclusions:

We postulate that circulating antigen antibody complex likely resulted in this patient’s clinical presentation. Our review of literature demonstrated some association with bacterial infections but no confirmed causation has been identified. There is no established standard treatment for HSP but steroids have been tried for severe disease. She was started on prednisone, valsartan and high dose fish oils (some benefit in IgA mediated disease) for persistent nephrotic range proteinuria. Her renal function improved with normalization of Creatinine but urinary sediments and high–grade nephrotic range proteinuria persisted.

Figure 1Renal biopsy: increased mesangial immunoglobulins and cell deposition along with crescent formation.

Figure 2Renal biopsy immunofluorescence with pathognomic IgA deposits.