Long-Term Therapeutic Plasma Exchange to Prevent End-Stage Kidney Disease in Adult Severe Resistant Henoch-Schonlein Purpura Nephritis

Abstract EN

A 27-year-old man presented with a palpable purpuric skin rash and joint and abdominal pain in April 2010.

He had acute kidney injury and his creatinine quickly deteriorated to 687 μmol/L, with associated nephrotic range proteinuria.

Kidney biopsy showed crescentic Henoch-Schonlein nephritis.

He was treated with intravenous cyclophosphamide and prednisolone despite which his renal function deteriorated; he required haemodialysis for a short duration and seven sessions of therapeutic plasma exchange (TPE).

Renal function improved, but after discharge from hospital he suffered 2 further relapses, each with AKI, in 4 months.

Cyclophosphamide was not effective and therefore Rituximab was introduced.

He initially had a partial response but his renal function deteriorated despite continued therapy.

TPE was the only treatment that prevented rapid renal functional deterioration.

A novel long-term treatment strategy involving regular TPE every one to two weeks was initiated.

This helped to slow his progression to end-stage kidney disease over a 3-year period and to prolong the need for renal replacement therapy over this time.