HDCN Article Review/Hyperlink

Mak SK, Short CD, Mallick NP

Long-term outome of adult-onset minimal-change nephropathy

Nephrol Dial Transplant (Nov) 11:2192-2201 1996

This was a retrospective review of adult-onset minimal change disease (MCD) treated at a single center. The questions asked were: Is steroid responsiveness and clinical course similar to, or worse than, that in children? In particular, the long-term outcome was of interest.

There were 101 patients with nephrotic syndrome (> 3.5 g/day and serum albumin less than 30 g/L) and no light microscopic changes on renal biopsy identified between 1950 and 1993. Somehow, this number dwindled to only 54 whose dx was consistent with MCD. Patients were excluded if nephrotic syndrome was not of abrupt onset, or if a subsequent biopsy revealed another diagnosis (It would have been of interest to specify these exclusions in more detail). Age range was 15-77, median 37 years. 33% had history of allergy and 33% history of a recent URI. A raised serum creatinine (> 130 umol/l) was found in 55-60%, but these returned to normal with remission. Microscopic hematuria was present in 33% at presentation. Age, increased BP, and presence of arteriosclerosis and/or hyaline degenerative change on biopsy predicted impaired renal function.

The tx used was 1.5 mg/kg/day or 2.0 mg/kg/alternate day prednisolone until remission (mean 4.3 weeks), followed by a prednisone taper. Only 37/51 patients were given a full course of steroids and 12 went untreated. Of the 37 treated with steroids, overall response rate was 92%. The response time was slower than in children, as it was only 50% at 4 weeks (vs. about 80-90% in children). This slower time to response is comparable to that of other series. Of the patients achieving remission with steroids, 76% relapsed, and 50% were steroid dependent. Cyclophosphamide was used to treat steroid-dependent patients and those with multiple relapses. Considering all cyclophosphamide treated patients, 74% and 63% had sustained remission at 3 and 5 years after presentation, respectively.

Comment: This is a descriptive series, with the advantage that it reports long-term follow-up. As fully half of the original nephrotic patients with no light microscopic changes were excluded, the excluded patients should have been commented on in more detail. (John T. Daugirdas, M.D., University of Illinois at Chicago)

The full text of this abstract is available from Oxford Press at this site.