Apheresis Therapy for Steroid-Resistant Idiopathic Nephrotic Syndrome: Report on a Case Series

Bennani, Hamza Naciri; Jouve, Thomas; Noble, Johan; Rostaing, Lionel; Malvezzi, Paolo; Tétaz, Rachel

doi:10.1155/2019/7304786

Cited by 1 publication

(2 citation statements)

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“…With the exception of studies on LDL-A, all the available data relating to the potential value of apheresis for treating refractory INS in adulthood originate exclusively from case reports. 13 , 26 , 27 , 28 Our study focusing exclusively on adult patients with refractory INS suggests that apheresis may be a relevant treatment option in some cases.…”

Section: Discussionmentioning

confidence: 99%

“…Naciri Bennani et al. 26 recently described 3 cases that suggested that double-filtration plasmapheresis or semi-specific IA could successfully treat INS in patients in whom conventional therapies had failed. With the exception of studies on LDL-A, all the available data relating to the potential value of apheresis for treating refractory INS in adulthood originate exclusively from case reports.…”

Section: Discussionmentioning

confidence: 99%

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Apheresis in Adult With Refractory Idiopathic Nephrotic Syndrome on Native Kidneys

Moret

Ganea

Dao

et al. 2021

Kidney International Reports

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Background Apheresis is the gold standard for idiopathic nephrotic syndrome (INS) relapse after transplantation, but it remains unknown whether such treatment is useful for adults with refractory INS on native kidneys. Methods This retrospective study included patients older than 16 years with biopsy-proven refractory (persistent nephrotic syndrome on corticosteroids plus at least 1 immunosuppressive drug) INS treated by apheresis and followed for at least 3 months. Results Between September 1997 and January 2020, 21 patients (focal segmental glomerulosclerosis: 12, minimal change nephrotic syndrome: 9, men: 67%, median age: 34 years) were identified. At last follow-up (12 months), 7 of 21 patients were in complete or partial remission. Remission was associated with older age (51 vs. 30 years, P = 0.05), lower proteinuria (3.9 vs. 7.3 g/d, P = 0.03), and lower estimated glomerular filtration rate (eGFR) (28.0 vs. 48.5 ml/min per 1.73 m 2 , P = 0.05) at apheresis. The need for dialysis before apheresis (odds ratio [OR] 22.0 [1.00–524], P = 0.026), age ≥50 years (OR: 22.6 [1.00–524], P = 0.006), a marked (>4.5 g/d) decrease in proteinuria (OR: 9.17 [1.15–73.2], P = 0.041), and a short (<12 months) time between diagnosis and apheresis (OR: 10.8 [1–117], P = 0.043) were significantly associated with remission. Three of 7 patients in remission who were initially on dialysis became dialysis-free; by contrast, none of the 14 patients without remission was initially on dialysis, but 5 of 14 had become dialysis-dependent ( P = 0.01). Conclusion Apheresis may result in remission in adult patients with refractory INS, particularly in those at risk of renal failure, with limited sensitivity to medical treatments, if apheresis is initiated within a year of diagnosis.

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