Adult Minimal-Change Disease

Abstract

Minimal-change disease (MCD) counts for 10 to 15% of cases of primary nephrotic syndrome in adults. Few series have examined this disease in adults. A retrospective review was performed of 95 adults who had MCD and were seen at a single referral center. Examined were presenting features, response to daily versus alternate-day steroids, response to second-line agents, relapse patterns, complications of the disease and therapy, presence of acute renal failure (ARF), and outcome data. Sixty-five patients received daily and 23 received alternate-day steroids initially. There were no differences in remissions, time to remission, relapse rate, or time to relapse between daily-and alternate-day-treated patients. More than one quarter of patients were steroid resistant. At least one relapse occurred in 73% of patients; 28% were frequently relapsing. A significant proportion of frequently relapsing patients became steroid dependent. Second-line agents were used for steroid dependence, steroid resistance, or frequent relapses. No single agent proved superior. There were more remissions with second-line agents in steroid-dependent patients compared with steroid-resistant patients, and remissions were more likely to be complete in steroid-dependent patients. ARF occurred in 24 patients; they tended to be older and hypertensive with lower serum albumin and more proteinuria than those without ARF. At follow up, patients with an episode of ARF had higher serum creatinine than those without ARF. Four patients progressed to ESRD. These patients were less likely to have responded to steroids and more likely to have FSGS on repeat renal biopsy. In this referral MCD population, response to daily and alternate-day steroids is similar. Second-line agents give greater response in patients who are steroid dependent. ARF occurs in a significant number of adult MCD patients and may leave residual renal dysfunction. Few patients progress to ESRD. M inimal-change disease (MCD) is the most common cause of the nephrotic syndrome (NS) in children. It accounts for 70 to 90% of the NS in children who are younger than 10 yr and 50% in older children. It is also an important cause of primary NS in adults of all ages, accounting for 10 to 15% of cases (1,2). Although there are many data regarding the course, response to treatment, and outcomes in pediatric patients, only a few series have examined these issues in adults (3-8). Relatively less is known about the presentation of MCD in adult patients, response to agents other than steroids, the risk for acute renal failure (ARF), and the long-term outcome. Moreover, most series of adults with MCD have not been in US populations, and it is unknown whether the features and course of the disease are the same in differing parts of the world.We therefore performed a retrospective review of 95 patients who had primary adult-onset MCD and were treated at a single tertiary care center in the past 15 yr. Many of the patients were referred by outside nephrologists for opinions regarding treatment. This review...

Clinical–Morphological Features and Outcomes of Lupus Podocytopathy

“…Similar findings were reported in other studies (12,17,32). The rapid response to glucocorticoid, high response rate, and high relapse rate shown by the patients with lupus podocytopathy are similar to those shown by adult patients with idiopathic MCD (33). In addition, none of the patients in this study progressed to ESRD or died during a median follow-up of 5 years.…”

The Treatment of Minimal Change Disease in Adults

“…MCD is the etiology of NS in 10%-25% of adults. [13][14][15] On urinalysis, microscopic hematuria is present in 10%-30% of adults with MCD [16][17][18][19] and may resolve with disease remission. Most cases are idiopathic, but secondary etiologies must be considered in adults.…”

Comparison of Methylprednisolone Plus Prednisolone with Prednisolone Alone as Initial Treatment in Adult-Onset Minimal Change Disease

“…Although few patients with MCD progress to ESRD, a series of studies reported that approximately 30%-70%, most commonly 60%, of adult MCD patients experienced at least one relapse of proteinuria (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18) and that 50%-60% of patients with a first relapse subsequently experienced a second relapse (7)(8)(9)(10)(11)(12). Thus, clinical goals in MCD patients are early induction of remission of proteinuria and more importantly, prevention of relapse of proteinuria.…”