Efficacy of mycophenolate mofetil for steroid and cyclosporine resistant membranoproliferative glomerulonephritis type I

Abstract

Sirs,We read with great interest the article entitled "Remission of resistant MPGN type I with mycophenolate mofetil and steroids" by De et al. [1]. We encountered a similar case and would like to comment on the efficacy of mycophenolate mofetil (MMF) for refractory membranoproliferative glomerulonephritis (MPGN) type I. A 13-year-old boy was transferred to our hospital because of steroid-resistant nephrotic syndrome with hypocomplementemia. He had been treated with 2 mg/kg of prednisolone (PSL) for 4 weeks, but systemic edema, massive proteinuria and hypertension had not improved. When he was admitted to our hospital, the results of urinalysis were: protein 2,498 mg/dl, creatinine 399 mg/dl, β2 microglobulin 5,691 μg/dl (normal <250 μg/dl), and red blood cell (RBC) count over 50 per high-power field (HPF). Those of blood biochemistry were: 4.4 g/dl of total protein, albumin 2.0 g/dl, serum creatinine 0.83 mg/dl, blood urea nitrogen 33 mg/dl, uric acid 7.3 mg/dl, total cholesterol 404 mg/dl, triglycerides 296 mg/dl, complement (C)3 21 mg/dl (86~160 mg/dl), C4 21 mg/dl (17~45 mg/dl), CH50 20 U/ml (30~40 U/ml), anti-nuclear antibody (negative), and anti-double stranded DNA antibody (negative). Renal biopsy showed diffuse and severe MPGN type I. All the glomeruli showed marked lobulation, intracapillary hypercellularity, thickening of the capillary walls and double contour. Immunofluorescence showed intense staining of all capillary walls and mesangial cells with C3 and immunoglobulin (Ig)G. An electron micrograph showed electron-dense deposits only in the subendothelial space. The patient was initially treated with three courses of methyl prednisolone pulse therapy (MPT, one course; 1 g of methyl prednisolone for 3 days) followed by PSL orally. Lisinopril and losartan were used for hypertension and proteinuria. Proteinuria was dramatically reduced after three courses of MPT, but it was still in the nephrotic range (Fig. 1). Additional cyclosporin was also insufficient to induce remission of proteinuria. In the end, he was started on MMF 3 months from the initiation of treatment. Proteinuria decreased promptly soon after he had started on MMF. Two months later the proteinuria had completely resolved, even though PSL had been withdrawn. Under treatment with PSL, cyclosporin A (CsA) and MMF, he has not had proteinuria for 8 months. Hematuria has also Pediatr Nephrol (

Efficacy of Mizoribine for Steroid‐resistant Membranoproliferative Glomerulonephritis Type Iii

“…Interestingly, MZR treatment has been reported to significantly suppress the progression of histological chronicity in patients with lupus nephritis and immunoglobulin A nephropathy 4,5 . Thus, we believe that long‐term MZR treatment may be beneficial for selected young patients with refractory MPGN, just like MMF 2,3 …”

“…Idiopathic membranoproliferative glomerulonephritis (MPGN) is an uncommon disease, and often progresses to end‐stage kidney disease 1 . Recently, several reports have focused on the efficacy of mycophenolate mofetil (MMF) in the treatment of refractory MPGN in adolescents 2,3 . However, the clinical use of MMF has not yet been approved for indications other than recipients of organ transplantation by the Ministry of Health and Welfare in Japan.…”