Plasmapheresis in a Patient with Rapidly Progressive Idiopathic IgA Nephropathy: Removal of IgA-Containing Circulating Immune Complexes and Clinical Recovery

Coppo, Rosanna; Basolo, B; Giachino, Osvaldo; Roccatello, Dario; D, Lajolo; Mazzucco, Gianna; Amore, Alessandro; Piccoli, Giuseppe

doi:10.1159/000183527

Cited by 39 publications

(18 citation statements)

References 7 publications

(7 reference statements)

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“…The results lasted for a decade after the end of treatment [26]; however, the side effects of this long-term heavy therapy were of some relevance. In Europe, much of the interest was focused on treating acute cases presenting with crescents and compromised renal function, with a few months of treatment obtaining encouraging results without side effects in some case series [18, 27, 28]. …”

Section: Treatment Of Pediatric Igan In Europementioning

confidence: 99%

“…The severity and frequency of side effects is not reported to be severe, probably because prolonged treatment is avoided. The Japanese reports on an increase in T lesions in children in whom the decision for renal biopsy was delayed or when CS were not used [25, 26] have further stressed the attitude for Europe to treat progressive IgAN in children with aggressive therapy [18, 27, 28]. In Europe, the results of the long-term follow-up of the VALIGA study [13] clearly indicated that when T lesions develop, the disease enters a relentless phase of progression, and that M1, S1, and also C lesions in untreated cases can have an impact on disease outcome decades later in children as well as in adults.…”

Section: Treatment Of Pediatric Igan In Europementioning

confidence: 99%

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Pediatric IgA Nephropathy in Europe

Coppo

2019

Kidney Dis

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Background: In Europe IgA nephropathy (IgAN) is detected in 20% of children with glomerular diseases diagnosed by renal biopsy. The outcome during childhood is generally good, but progression in the long-term follow-up may occur in about 20% of children after 20 years. Summary: In Europe, urine screening programs are not active, and there is variability in the policy to perform renal biopsies in oligo-symptomatic children. Hence, a suitable observational approach to pediatric IgAN is offered by the VALIGA study which included 174 children aged < 18 years from 13 European countries followed over a median of 4.4 (2.5–7.5) years. Renal pathology lesions were centrally scored according to the Oxford Classification of IgAN (mesangial hypercellularity, M; endocapillary hypercellularity, E; segmental glomerulosclerosis, S; tubular atrophy/interstitial fibrosis, T; crescents, C [MEST-C]). Children had renal biopsy mostly with normal estimated glomerular filtration rate (eGFR) and moderate proteinuria of a median of 0.84 g/day/1.73 m² (< 0.30 g/day/1.73 m² in 30% of the cases). Children showed M1 in 21.8%, E1 in 13.8%, S1 in 42.5%, T1–2 in 6.3%, and C1 in 14.9%. The survival at the combined endpoint of 50% eGFR decrease or end-stage renal disease at 15 years was 94%. The slow progression rate and the limited number of cases progressing to the combined endpoint (6.4%) did not allow the detection of a predictive value of the MEST-C score. Moreover, the predictive value of clinical and pathological features was likely blunted by the use of corticosteroid/immunosuppressive treatment (CS/IS) in 50% of the cases. The survival tree analysis also proved that children < 16 years old with IgAN without mesangial hypercellularity (M0) and well preserved eGFR (> 90 mL/min/1.73 m²) had a high probability of proteinuria remission during follow-up. Moreover, in this subgroup of children, the benefits of CS/IS therapy reached statistical significance. In Europe, the use of CS/IS treatment in IgAN is still a debated issue, but most children tend to be treated more commonly than adults with CS/IS. A recent uncontrolled study reports a favorable outcome in European children with IgAN and very active acute forms of IgAN with improvement in eGFR and reduction in proteinuria. Key Messages: In Europe, children with IgAN have a favorable prognosis in the short term, and this may be due also to the frequently adopted CS/IS therapy, particularly with acute and active pathological features. The risk of progression over decades of follow-up remains an unsolved problem which needs to be addressed by controlling subtle chronic pathogenetic factors which work in children as well as in adult cases of IgAN.

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Section: Treatment Of Pediatric Igan In Europementioning

confidence: 99%

Section: Treatment Of Pediatric Igan In Europementioning

confidence: 99%

Pediatric IgA Nephropathy in Europe

Coppo

2019

Kidney Dis

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“…Removal of circulating IgA-containing immune complexes may prevent worsening of renal function in such patients with IgA nephropathy [2,3]. Several studies showed that plasmapheresis can result in successful treatment of IgA nephropathy, even without immunosuppression [4,5].…”

Section: Iga Nephropathymentioning

confidence: 99%

Clinical applications of therapeutic apheresis

Balogun

Kaplan

Ward

et al. 2010

J of Clinical Apheresis

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“…Recent reports have suggested that patients with a rapidly progressive form of this disease may benefit from plasma exchange therapy [4,5]. We report the course of two patients with rapidly progressive IgA nephropathy treated with plasmapheresis in addition to prednisone and cyclophosphamide.…”

Section: Introductionmentioning

confidence: 97%