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Minimal Change Disease CJASN ePress. Published on December 23, 2016 as doi: 10.2215/CJN.05000516 Minimal Change Disease Marina Vivarelli,* Laura Massella,* Barbara Ruggiero,† and Francesco Emma* Abstract Minimal change disease (MCD) is a major cause of idiopathic nephrotic syndrome (NS), characterized by intense proteinuria leading to edema and intravascular volume depletion. In adults, it accounts for approximately 15% of patients with idiopathic NS, reaching a much higher percentage at younger ages, up to 70%–90% in children >1 year of age. In the pediatric setting, a renal biopsy is usually not performed if presentation is typical and the patient *Division of responds to therapy with oral prednisone at conventional doses. Therefore, in this setting steroid-sensitive NS Nephrology and can be considered synonymous with MCD. The pathologic hallmark of disease is absence of visible alterations by Dialysis, Bambino light microscopy and effacement of foot processes by electron microscopy. Although the cause is unknown and it is Gesu` Children’s likely that different subgroups of disease recognize a different pathogenesis, immunologic dysregulation and Hospital, IRCCS, Rome, Italy; and modifications of the podocyte are thought to synergize in altering the integrity of the glomerular basement †Clinical Research membrane and therefore determining proteinuria. The mainstay of therapy is prednisone, but steroid-sensitive Center for Rare forms frequently relapse and this leads to a percentage of patients requiring second-line steroid-sparing immu- Diseases “Aldo e Cele nosuppression. The outcome is variable, but forms of MCD that respond to steroids usually do not lead to chronic Dacco`”, IRCCS – renal damage, whereas forms that are unresponsive to steroids may subsequently reveal themselves as FSGS. Istituto di Ricerche Farmacologiche Mario However, in a substantial number of patients the disease is recurrent and requires long-term immunosuppression, Negri, Ranica, with significant morbidity because of side effects. Recent therapeutic advances, such as the use of anti-CD20 Bergamo, Italy antibodies, have provided long-term remission off-therapy and suggest new hypotheses for disease pathogenesis. Clin J Am Soc Nephrol 12: ccc–ccc, 2016. doi: 10.2215/CJN.05000516 Correspondence: Dr. Marina Vivarelli, Division of Nephrology and Introduction Therefore, the terms steroid-sensitive NS on the basis Dialysis, Ospedale In adults, minimal change disease (MCD) represents of clinical features and MCD on the basis of histo- Pediatrico Bambino – Gesu`–IRCCS, Piazza approximately 10% 15% of patients with idiopathic logic picture are not entirely equal. In clinical practice, S. Onofrio 4, 00165 nephrotic syndrome (Figure 1) (1,2). In children .1year however, especially in children who do not systemat- Rome, Italy. Email: of age, MCD is the most common cause of nephrotic ically undergo a renal biopsy, these terms are often marina.vivarelli@ syndrome, accounting for 70%–90% of patients; used as synonyms. In this review, the term MCD will opbg.net around puberty, this proportion decreases signifi- include all children with classic clinical features of cantly as other glomerular diseases, such as membra- steroid-sensitive NS in which a renal biopsy was not nous nephropathy, become more frequent (Figure 1) performed, and all adults with the histologic pattern of (3,4). The histologic picture of MCD is identical in MCD regardless of response to therapy. adults and children. Because most children respond to steroid treatment, the disease is termed “steroid- sensitive nephrotic syndrome” (steroid-sensitive NS) Definitions and Epidemiology on the basis of clinical features, and renal biopsy is The reported incidence of MCD in children varies not performed unless steroid resistance is observed. If between two and seven new cases per 100,000 chil- performed, it usually shows the absence of significant dren (3,7). The exact prevalence is not known, but on changes by light microscopy (LM), a finding that has the basis of disease evolution and average age of puzzled physicians for decades. When ultrastructural onset, it can be estimated at approximately 10–50 cases analyses were first performed in the 1950s, extensive per 100,000 children. The disease is slightly more com- fusion of podocyte foot processes was observed (5). mon in Asia and has a male predominance (approxi- Soon after, the term “lipoid nephrosis,” introduced mately 2:1) in young children that disappears in in the early 1900s to describe the presence of micro- adolescents and adults (3,8). MCD is much less fre- scopic lipid droplets in urine and tubular cells, was quent in adults, but the exact incidence in this popula- replaced by MCD, to highlight the existence of mini- tion is less well documented. mal alterations of the glomerulus by LM. However, a Defining steroid sensitivity represents a first critical minority of patients that have FSGS lesions on their aspect for prognosis, as forms that respond do not evolve renal biopsy do respond to steroids and, conversely, toward chronic renal failure. In MCD, unlike other patients with steroid-resistant nephrotic syndrome glomerular diseases, steroid response when present (steroid-resistant NS) may have MCD at disease onset, is often complete, with total disappearance of protein- before developing FSGS lesions later in the course of uria. However, time to remission is very different in their disease (6). children compared with adults. As shown in Figure 2, www.cjasn.org Vol 12 February, 2016 Copyright © 2016 by the American Society of Nephrology 1 2 Clinical Journal of the American Society of Nephrology Figure 1. | Etiology of nephrotic syndrome according to age from 1 year onwards. (A) Minimal change disease. (B) Focal segmental glo- merulosclerosis. (C) Membranous nephropathy. Data are approximated from Nachman et al. (1) and Cameron (2). Clin J Am Soc Nephrol 12: ccc–ccc, February, 2016 Minimal Change Disease, Vivarelli et al. 3 Figure 2. | Time-to-response to prednisone is much shorter in children than in adults with minimal change disease. Data are extrapolated from references Waldman et al. (12) and Vivarelli et al. (82) for children (A and B), from references (83) and Chen et al. (84) for adults (C and D). after a first episode, approximately 50% of children achieve re- histologic picture that has been defined as IgA nephro- mission within 8 days of steroid treatment and most patients pathy with MCD (15). Focal segmental distribution of IgM who are going to respond to steroids do so within 4 weeks. and C3 staining should strongly suggest FSGS even when In contrast, in adults the median time to remission exceeds no sclerotic lesions are captured by LM, whereas more 2 months. In both populations, MCD has a high tendency to than trace amounts of IgG and IgA suggest alternative di- relapse, and relapses tend to be more rapid in children. agnoses. By electron microscopy, foot process effacement In Table 1, a further classification of MCD in clinical is the only morphologic feature of MCD. Other pathologic subgroups on the basis of response to therapy, separating features automatically exclude the diagnosis of MCD, children and adults, is reviewed. This classification for which is therefore a diagnosis of exclusion. children was initially proposed by the International Study of Kidney Disease in Children (ISKDC) (9) and has been adapted over the years (10). Clinical Features Rarely, nephrotic-range proteinuria is discovered on a routine urinalysis, but most frequently the presenting Pathology symptom of MCD is nephrotic syndrome, characterized by MCD accounts for the vast majority of idiopathic NS in edema, periorbital (often misinterpreted as allergic), of the children, particularly for the steroid-sensitive forms (3,11). scrotum or labia, and of the lower extremities (11). Anasarca In adults the picture is much more varied, with MCD be- may develop with ascites and pleural and pericardial effu- ing the third cause of idiopathic NS, after FSGS and mem- sion, leading to abdominal pain because of hypoperfusion branous nephropathy (1,2,4). Thus, whereas in adults early and/or thrombosis, dyspnea (rarely), and cold extremities kidney biopsy is crucial in driving the therapeutic ap- with low BP (11). Especially in children, severe infections proach (12), the indications for renal biopsy in children such as sepsis, pneumonia, and peritonitis may be present at are limited to features that may suggest alternative diag- disease onset or during disease course because of Ig de- noses: at onset, age ,1or.12 years, gross hematuria, low pletion and altered T cell function (16). Moreover, onset is serum C3, marked hypertension, renal failure without frequently preceded by upper respiratory tract infection. severe hypovolemia, and positive history for secondary Intravascular volume depletion and oliguria are also pres- cause; subsequently, steroid resistance or therapy with cal- ent, and concomitant factors (sepsis, diarrhea, diuretics) can cineurin inhibitors. By LM, no glomerular lesions or only lead to AKI, which is more frequently seen in adults (12), mild focal mesangial prominence not exceeding three or although it occurs in about half of children hospitalized for four cells per segment are seen. The presence of more than nephrotic syndrome (17). Rarely, AKI with gross hematuria four mesangial cells per mesangial region affecting at least followed by anuria can also be secondary to bilateral renal 80% of the glomeruli defines the diffuse mesangial hyper- vein thrombosis. Gross hematuria is rare, occurring in 3% of cellularity variant of MCD. These patients can present patients (11). with hematuria and hypertension, unlike children with Laboratory analyses begin with urinalysis, with urinary classic MCD (13). Immunofluorescence is usually negative. dipstick showing 31/41 proteinuria (corresponding to However, low-intensity mesangial IgM (sometimes accom- $300 mg/dl on urinalysis) and, in about 20% of pa- panied by C3 or C1q) staining can be found (14) and MCD tients, microhematuria, which may resolve during remission is occasionally accompanied by glomerular IgA deposits, a of proteinuria. Urine collection shows nephrotic-range 4 Clinical Journal of the American Society of Nephrology Table 1.
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