Childhood vasculitis and plasma exchange
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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).
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 m2 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 m2 (p<0.01) pre-treatment to 62 ml/min/1.73 m2 (p<0.01) immediately following TPE and 69 ml/min/1.73 m2 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.
In our experience TPE appears to be of benefit during the acute phase of illness, especially in children with organ-specific disease.
KeywordsChildren Immunosuppression Outcome Plasma exchange Vasculitis
- 1.Cole E, Cattran D, Magil A, Greenwood C, Churchill D, Sutton D, Clark W, Morrin P, Posen G, Bernstein K (1992) A prospective randomised trial of plasma exchange as additive therapy in idiopathic crescentic glomerulonephritis. The Canadian Apheresis Study Group. Am J Kidney Dis 20:261–269PubMedGoogle Scholar
- 3.Frasca GM, Zoumparidis NG, Borgnino LC, Neri L, Vangelista A, Bonomini V (1991) Plasma exchange treatment in rapidly progressive glomerulonephritis associated with anti-neutrophil cytoplasmic autoantibodies. Int J Artif Organs 15:181–184Google Scholar
- 6.Guillevin L, Fain O, Lhote F , Jarrousse B, Le Thi Huong D, Bussel A, Leon A (1992) Lack of superiority of steroids plus plasma exchange to steroids alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome. A prospective, randomized trial in 78 patients. Arthritis Rheum 35:208–215PubMedGoogle Scholar
- 7.Guillevin L, Lhote F, Cohen P, Jarrousse B, Lortholary O, Genereau T, Leon A, Bussel A (1995) Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome patients with factors predicting poor prognosis. A prospective randomized trial in sixty-two patients. Arthritis Rheum 38:1638–1645PubMedGoogle Scholar