Childhood vasculitis and plasma exchange.

INTRODUCTION: A retrospective review of therapeutic plasma exchange (TPE) for vasculitis of any form during the period 1993-2003 was carried out in our unit.

SUBJECTS AND METHODS: The case histories of 32 children undergoing TPE were analysed to determine short-term outcome. The vasculitides consisted of polyarteritis nodosa (PAN), ANCA-associated vasculitis (AAV) [encompassing Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA)/idiopathic crescentic nephritis (ICN)], Henoch-Schönlein purpura (HSP) and non-classified vasculitis (NCV).

RESULTS: All children with PAN (n=5) had a good response to treatment except for one with gastro-intestinal symptoms. One child was treated for crescentic nephritis, with 42% crescents seen on biopsy. The estimated glomerular filtration rate (eGFR) (Schwartz formula) for this child improved from 81 to 130 ml/min/1.73 m(2) post-treatment. Inflammatory markers decreased immediately following TPE and were maintained at the lower level for all the children. Ten children (n=12) with AAV received treatment for renal manifestations. Median creatinine clearances improved from 29 ml/min/1.73 m(2) (p<0.01) pre-treatment to 62 ml/min/1.73 m(2) (p<0.01) immediately following TPE and 69 ml/min/1.73 m(2) at 2 months post-TPE (p<0.01). Four children required dialysis; one child remained dialysis-dependent and one child progressed to chronic renal failure. Inflammatory markers decreased following TPE. All of the children with HSP (n=5) were treated for crescentic nephritis. Two regained normal renal function, and one became dialysis-dependent later. Most of the children with NCV (n=10) presented with CNS or general vasculitic features, with the greatest benefit for CNS symptoms.

CONCLUSIONS: In our experience TPE appears to be of benefit during the acute phase of illness, especially in children with organ-specific disease.