Abstract
Management of steroid-resistant minimal change disease remains elusive with international guidelines suggesting high-dose corticosteroids and/or Calcineurin inhibitors for months similar to those with refractory idiopathic FSGS. Unfortunately, with such approach, the overall remission rates were 47% - 66%. Moreover, complete remission rates were 32% - 47% and partial remission ones were 19% - 29%. Those limited options of treatment and their poor outcomes led us to conduct the present study to assess the efficacy and safety of a new combined drug-therapy at induction and subsequent maintenance of such disease. The regimen consisted of an initial induction phase of 3-month Prednisone, Calcineurin-inhibitor, Mycophenolate and ACEI/ARB. The latter was followed by a maintenance phase of minimal dose Prednisone and nearly 1/2 the induction dose of Calcineurin inhibitors to decrease their long-term side effects. The results were satisfactory with 14 of the 22 patients, had complete remission. Moreover, 5 patients manifested partial remission and only 3 did not respond. Creatinine clearance was maintained in patients with complete remission yet, was mildly reduced in the partial and non-responsive ones. The safety and efficacy of such new combined drug-therapy provide new tool and future prospective in management of such relentless disease.
Highlights
Minimal change disease (MCD) manifests as an acute nephrotic syndrome (NS) and a renal biopsy that shows no glomerular lesions on light microscopy, negative staining on immunofluorescence microscopy, and foot process effacement but no electron-dense deposits on electron microscopy [1]
In the 1970s, Shalhoub proposed that the cause of lipoid nephrosis is a T-cell secreted circulating factor that alters the negative charge on glomerular basement membrane [9]
Our patients did not benefit from previous corticosteroid/Calcineurin inhibitors (CNIs) therapy yet benefited after addition of MMF
Summary
Minimal change disease (MCD) manifests as an acute nephrotic syndrome (NS) and a renal biopsy that shows no glomerular lesions on light microscopy (or only minimal mesangial prominence), negative staining on immunofluorescence microscopy (or low-level staining for C3 and IgM), and foot process effacement but no electron-dense deposits on electron microscopy [1].In children, MCD is the cause of 90% of cases of idiopathic NS and is usually exquisitely responsive to steroids. On the other hand; management of SR MCD remains elusive with international guidelines suggesting high-dose corticosteroids and/or CNIs for months similar to those with refractory idiopathic FSGS [6]. With such approach, the overall remission rates were 47% - 66%. Complete remission rates were 32% - 47% and partial remission ones were 19% - 29% [7] Such limited options of treatment and their poor outcomes led us to conduct our present study to assess the efficacy and safety of a new combined drug-therapy at induction and subsequent maintenance of such relentless disease
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