Mycophenolate mofetil therapy for steroid-resistant IgA nephropathy with the nephrotic syndrome in children

Abstract

BackgroundImmunoglobulin A nephropathy (IgAN) presents as nephrotic syndrome (NS) relatively rarely, and the current treatment experience of IgAN patients with NS is mostly with adults. The objective of our study was to investigate the efficacy of corticosteroids and mycophenolate mofetil (MMF) in treating childhood immunoglobulin A nephropathy (IgAN) with nephrotic syndrome.MethodsA total of 58 children (39 boys and 19 girls) diagnosed with nephrotic syndrome and primary IgAN were enrolled in the study. All the patients were administered prednisone 2 mg/kg per day for 8 weeks. Steroid-resistant patients were treated with the combined use of MMF (dose of 20 ~ 30 mg/kg per day) and prednisone for 6–12 months. The prednisone dose was reduced stepwise during the combined treatment.ResultsOf the 58 children, 14 were steroid-sensitive (M, S, and T variants of the Oxford classification were 0 in most children), and 44 cases who presented serious pathological damage to the kidney were steroid-resistant. The estimated glomerular filtration rate (eGFR) of the steroid-resistant children (86.69 ± 26.85 ml/min/1.73 m2) was significantly lower (P < 0.05) than that of the steroid-sensitive children (106.89 ± 26.94 ml/min/1.73 m2). After 4 months of combined MMF treatment in 33 steroid-resistant children, complete remission of proteinuria was found in 21 cases, partial remission of proteinuria in 6 cases, and no response was found in 6 cases. Except for the T variant, other variants of the Oxford classification, including M, E, and S morphological variables, was not significantly different among patients complete remission, those with partial remission, and those with no response. The eGFR of children with complete remission of proteinuria (100.04 ± 18.47 ml/min/1.73 m2), that of those with partial remission (92.24 ± 27.63 ml/min/1.73 m2), and that of those with no response (72.17 ± 27.55 ml/min/1.73 m2) were significantly different (P < 0.05).ConclusionCorticosteroid therapy showed satisfactory efficacy in IgAN children with nephrotic syndrome and slight pathological damage. The effect of MMF was good for steroid-resistant IgAN children, but poor for those with tubular atrophy/interstitial fibrosis and renal function impairment.

Serum IgG Level and IgG/IgM Ratio on Admission Predict Steroid-Resistant Response in Vietnamese Children with Idiopathic Nephrotic Syndrome

“…In particular, it was observed that the serum level of IgA, IgG, and IgG/IgM ratio in the group of patients with steroid resistance were significantly lower than those of the ones with steroid sensitivity (P < 0.05). The current study results also coincided with those of previously published authors' study (20)(21)(22)(23)(24). Biopsy was performed on 19 patients with steroid-resistant NS, and histopathological results showed that the rate of FSGS, MesPGN, and MPGN accounted for 78.9% of them.…”

The response of the Oxford classification to steroid in IgA nephropathy: a systematic review and meta-analysis

“…The characteristics of the included studies are summarized in the Table 1 . The inclusion criteria used in two trials [ 13 , 14 ] was IgAN patients with nephrotic syndrome, and the other one [ 15 ] was biopsy-proved IgAN and urinary protein excretion ranging from 1.0 to 3.5 g/24h. Another two studies [ 16 , 17 ] did not limit the scope of proteinuria, and the last study [ 16 ] used the immunosuppressive therapy and was only provided the data of M1, E1, S1, T0, and T1/2, without M0, E0, and S0.…”

“…Other studies have also showed the good response to steroid in patients with minimal histopathological changes and resistant to steroid therapy with severe pathological change [ 19 – 21 ]. Kang Z et al found that patients with M1, S1, and T1/2 were resistant to steroid therapy [ 14 ], however the results have not been validated in the Oxford classification. To our knowledge, it was the first meta-analysis that found the presence of the M1, S1, or T1/2 lesions in the Oxford Classification was strongly associated with the resistance to steroid/immunosuppressive therapy.…”

Clinical practice guidelines for the provision of renal service in Hong Kong: General Nephrology

“…Another European study using an enteric formulation of budesonide also demonstrated significant renoprotective effects after 9 months of therapy For steroid‐resistant patients, MMF may be individually considered in Chinese subjects without advanced tubulointerstitial changes, but it is not recommended for Caucasians . (Not Graded) For IgAN/MCD overlap syndrome, treatment should be analogous to that of MCD (see item 1).…”