Treating severe Henoch-Schönlein and IgA nephritis with plasmapheresis alone

Abstract

The aim of our study was to determine the outcome of children with severe Henoch-Schönlein nephritis (HSN) and immunoglobulin A (IgA) nephritis (IgAN) treated with early plasmapheresis alone. Children with acute renal impairment, heavy proteinuria or both and histology greater than grade 3 were treated with early plasmapheresis alone. Glomerular filtration rate (GFR) estimated from plasma creatinine (eGFR), urine albumin:creatinine ratio (UA/UC) and blood pressure 2 weeks after treatment and were measured at the last follow-up. Sixteen children (14 HSN, 2 IgAN) had a mean eGFR of 56 (17-136) ml/min per 1.73 m2 and UA/UC of 590 (12-1,379) mg/mmol. Fifteen were referred at presentation and one after 2 months, and all commenced plasmapheresis within 6 (2-13) days. All had at least nine exchanges of 90 ml/kg over 2 weeks. At 2 weeks, the eGFR had increased by 51 (95% CI 34-68; P=0.002), and the UA/UC fell by 457 (95% CI 241-673; P=0.0001). At last review after 4 (1-7.5) years, the late-referred child had required a renal transplant but the other 15 had normal eGFRs (98-142), did not require hypotensive medication, and had normal or minimally elevated UA/UC (maximum 42). Children with severe HSN and IgAN recover well if treated with plasmapheresis alone without the need for immunosuppressive therapy. A randomised trial is needed.

Is it the time to offer rituximab as a cost-benefit treatment for immunoglobulin A nephropathy? A short-review to current concepts

“…This contradiction between these studies may be explained by the fact that initial plasma exchange may have further improved renal function in the investigation by Lundberg et al (20). The rationale behind these results, which has had favorable findings in some earlier reports on moderate to severe IgAN, is to rapidly delete circulating IgAcontaining immune complexes (22).…”

Therapeutic plasma exchange for the treatment of pediatric renal diseases in 2013

“…Retrospective studies of children with crescentic HSP nephritis have demonstrated improvement in those treated with TPE alone [77,78] and in combination with medical immunosuppression [79], although no prospective data exist. Neither HSP nor IgA nephropathy is a recommended indication for TPE at this time [3].…”

Pitfalls in recommending evidence-based guidelines for a protean disease like Henoch–Schönlein purpura nephritis

“…1) The relationship between the severity of initial clinical and histological signs and the long-term prognosis [1][2][3][4][5]; 2) The correlation between chronicity score and time elapsed between clinical onset of kidney involvement and renal biopsy [10]; 3) The possible rapid evolution of crescentic glomerular lesions to complete glomerulosclerosis [11]; 4) A worse evolution when treatment is delayed even shortly [12][13][14][15]; 5) CKD developing years after apparent complete resolution [1,12].…”

“…However, PE is not recommended in cases of ANCAassociated crescentic glomerulonephritis with moderate increases in plasma creatinine values only because no adequate RCT has yet been performed. In HSPN, PE alone has been used with success in two series of pediatric patients [13,14] presenting with acute renal function impairment, heavy proteinuria or nephrotic syndrome and a histological grade of ≥III [14] or equal to V [13]. At last review, after 4 [14] and 10 years [13], 13 of 14 [14] and six of nine patients [13], respectively, had a normal GFR and complete or almost complete resolution of other renal symptoms.…”

“…In HSPN, PE alone has been used with success in two series of pediatric patients [13,14] presenting with acute renal function impairment, heavy proteinuria or nephrotic syndrome and a histological grade of ≥III [14] or equal to V [13]. At last review, after 4 [14] and 10 years [13], 13 of 14 [14] and six of nine patients [13], respectively, had a normal GFR and complete or almost complete resolution of other renal symptoms. Two patients with ISKDC histological grade V (2/9; 22 %) [13] and one with grade IIIb (1/14; 7 %) [14] had reached end-stage renal disease (ESRD), which is less than in large historical series [1,2].…”