title: Long-Term Therapeutic Plasma Exchange to Prevent End-Stage Kidney Disease in Adult Severe Resistant Henoch-Schonlein Purpura Nephritis.
abstract: 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.
authors.affiliation: Renal Department, Salford Royal NHS Foundation Trust, Salford, Greater Manchester M6 8HD, UK.
authors.forename: Patrick
authors.initials: P
authors.lastname: Hamilton
authors.affiliation: Renal Department, Salford Royal NHS Foundation Trust, Salford, Greater Manchester M6 8HD, UK.
authors.forename: Olumide
authors.initials: O
authors.lastname: Ogundare
authors.affiliation: Renal Department, Salford Royal NHS Foundation Trust, Salford, Greater Manchester M6 8HD, UK.
authors.forename: Ammar
authors.initials: A
authors.lastname: Raza
authors.affiliation: Renal Department, Salford Royal NHS Foundation Trust, Salford, Greater Manchester M6 8HD, UK.
authors.forename: Arvind
authors.initials: A
authors.lastname: Ponnusamy
authors.affiliation: Renal Department, Salford Royal NHS Foundation Trust, Salford, Greater Manchester M6 8HD, UK.
authors.forename: Julie
authors.initials: J
authors.lastname: Gorton
authors.affiliation: Renal Department, Salford Royal NHS Foundation Trust, Salford, Greater Manchester M6 8HD, UK.
authors.forename: Hana
authors.initials: H
authors.lastname: Alachkar
authors.affiliation: Histopathology Department, Salford NHS Foundation Trust, Salford, Greater Manchester M6 8HD, UK.
authors.forename: Jamil
authors.initials: J
authors.lastname: Choudhury
authors.affiliation: John Walls Renal Unit, Leicester General Hospital, Gwendolen Road, Leicester LE5 4PW, UK.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Barratt
authors.affiliation: Renal Department, Salford Royal NHS Foundation Trust, Salford, Greater Manchester M6 8HD, UK.
authors.forename: Philip A
authors.initials: PA
authors.lastname: Kalra