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Article Iga Nephropathy with Minimal Change Disease Article IgA Nephropathy with Minimal Change Disease Leal C. Herlitz,* Andrew S. Bomback,† Michael B. Stokes,* Jai Radhakrishnan,† Vivette D. D’Agati,* and Glen S. Markowitz* Abstract Background and objectives Patients with IgA nephropathy typically present with hematuria and subnephrotic proteinuria. Nephrotic syndrome is uncommon in IgA nephropathy, and when present, it is usually associated with severe histologic features, such as endocapillary proliferation, segmental sclerosis, and crescent formation. Rarely, patients with IgA nephropathy present with nephrotic syndrome and only mild mesangial disease. This *Department of Pathology and Cell study sought to better characterize these patients. Biology, Division of Renal Pathology, and Design, setting, participants, & measurements A retrospective review of cases of IgA nephropathy diagnosed †Department of from 2004 to 2011 identified patients with nephrotic range proteinuria and histologically mild IgA nephropathy. Medicine, Division of fi fi fi Nephrology, Speci cally, using the Oxford Classi cation of IgA Nephropathy, we identi ed cases that lacked endocapillary Columbia University proliferation or segmental sclerosis. Medical Center and the New York Results The cohort consisted of 17 patients, including 10 men and 15 adults. The median serum creatinine was Presbyterian Hospital, New York, New York 0.9 mg/dl (range=0.7–3.1), median 24-hour urine protein was 8.0 g/d (3.0–18.0 g), and 14 patients were fully fi nephrotic, whereas the remaining 3 patients ful lled two of three criteria for nephrotic syndrome. Biopsies Correspondence: revealed IgA-dominant or codominant deposits accompanied by mesangial proliferation in 14 patients (82.4%). Dr. Leal C. Herlitz, Electron microscopy showed mesangial deposits and extensive foot process effacement (median=90%). Initial Department of treatment consisted of corticosteroids, although many patients required additional agents to maintain remission Pathology, 630 West status. Over a median follow-up of 20 months (2.2–82 months), 14 patients experienced a complete response, 168th Street, VC14- – 238, New York, NY and 3 patients showed a partial response, with a median response time of 2 months (0.5 27 months). At least 10032. Email: one relapse of nephrotic syndrome occurred in nine patients (53%). All patients exhibited stable or improved [email protected] renal function over the follow-up period. Conclusions The findings in this cohort and previous studies suggest that rare cases of mild IgA nephropathy with nephrotic range proteinuria exhibit a clinical presentation, biopsy findings, treatment response, and outcome more typical of IgA nephropathy with superimposed minimal change disease. This study favors the view that such cases represent a dual glomerulopathy. Clin J Am Soc Nephrol 9: 1033–1039, 2014. doi: 10.2215/CJN.11951113 Introduction proliferative IgAN. In these patients, electron micros- IgA nephropathy (IgAN) is the most common GN copy typically reveals diffuse foot process effacement worldwide (1). IgAN is typically an indolent disease without peripheral capillary wall immune deposits, with a slow progression to end stage kidney disease reminiscent of minimal change disease (MCD). Many in approximately 30% of patients over 20 years (2). of the patients are rapidly responsive to treatment Clinical risk factors for progression of IgAN include with corticosteroids. Such cases are uncommon but proteinuria.1.0 g/d, hypertension, and persistent have been presented as case reports and in small series microscopic hematuria (1). Clinical presentations of over the last 30 years (4–17). Some of these reports IgAN vary widely, but the most common presenta- have suggested that the clinical and pathologic find- tion is that of hematuria with subnephrotic protein- ings in these unusual cases of IgAN (4–17), as well as uria. Nephrotic syndrome (NS) is an unusual the clinical outcomes (4–17), are more typical of mild presentation occurring in approximately 5% of cases IgAN with superimposed MCD. (3). Heavy proteinuria and/or NS in patients with The literature on treating MCD and IgAN as separate IgAN are often accompanied by renal insufficiency entities is abundant. In recent years, first-line treatment and biopsy findings of more aggressive disease, of MCD (i.e., corticosteroids) (18) also has been estab- with endocapillary proliferative and sclerosing glo- lished as a standard therapy for patients with IgAN merular lesions typically associated with subendothe- with significant proteinuria (19–21), generally consid- lial IgA deposits. ered to be .1 g/d despite effective use of renin angio- A subset of patients with IgAN presents with NS tensin system-blocking drugs. Thus, the patient who and has histologic findings of only mild mesangial presents with NS and is found to have findings www.cjasn.org Vol 9 June, 2014 Copyright © 2014 by the American Society of Nephrology 1033 1034 Clinical Journal of the American Society of Nephrology suggestive of both MCD and mild IgAN should be a straight- urinary sediment. NS was defined as NRP, hypoalbumin- forward treatment decision. Still, the prognosis for these pa- emia, and peripheral edema. Hypertension was defined as tients remain uncertain (specifically, whether they will have systolic BP.140 mmHg, diastolic BP.90 mmHg, or use of the often benign long-term course of mild IgAN or the fre- antihypertension medication. For children, NRP was defined quently relapse-ridden course of MCD). as .40 mg/m2 per hour (24), hypoalbuminemia was defined We present a series of 17 patients from the United States as serum albumin,2.5 g/dl (24), renal insufficiency was de- who developed nephrotic range proteinuria (NRP) and, in fined as calculated creatinine clearance,90 ml/min normal- most cases, full NS and were found to have mild mesangial ized for body surface area (25), and hypertension was based proliferative IgAN with diffuse podocyte foot process on the clinical assessment of the referring nephrologist. effacement. The mild degree of immune complex deposi- For the purpose of outcomes analysis, the following tion and mesangial proliferation, in the absence of endo- definitions were applied. Stable renal function was defined capillary proliferation or subendothelial deposits, seemed as a change in SCr of #25% of the initial value. Complete insufficient to explain the severe foot process effacement. remission (CR) was 24-hour urine protein of #500 mg/d at We believe that these cases represent mild IgAN with last follow-up. Partial remission (PR) was 24-hour urine pro- superimposed podocytopathy, which is most consistent tein of #3 g/d and a reduction in proteinuria of at least 50%. with MCD. Clinical presentation, biopsy findings, treat- CRs were further subdivided into patients who achieved re- ment, and prolonged follow-up are presented to better mission but were still on therapy at the last follow-up (CR characterize these unusual cases of mild IgAN with severe on therapy) and patients in remission who were no longer proteinuria. on therapy (CR off therapy). If a patient achieved remission for 6 months or longer while off therapy, subsequent epi- sodes of NS were considered to be relapses. If remission Materials and Methods status was lost within 6 months of therapy withdrawal, All native renal biopsies received and processed at the return of proteinuria was considered to be evidence of Columbia University Medical Center from 2004 to 2011 therapy dependence and not categorized as a relapse. Data were reviewed retrospectively (Columbia University In- are presented descriptively, because the small number of stitutional Review Board AAAI3051) for the diagnosis of subjects precludes formal statistical analysis. IgAN occurring in the setting of NS or NRP with edema or hypoalbuminemia. IgAN was defined by the presence of dominant or codominant glomerular staining for IgA in the Results absence of systemic lupus. Biopsies were graded according The cohort of 17 patients included 10 men and 7 women: 15 to the Oxford Classification of IgAN (22,23). Specifically, adults between the ages of 22 and 72 years and 2 children the degree of mesangial hypercellularity (M), the presence (ages 5 and 6 years). Eleven patients were Caucasian, three of endocapillary proliferation (E) and segmental glomeru- patients were Hispanic, two patients were Asian, and one losclerosis (S), and the degree of tubulointerstitial scarring patient was African American (Table 1). Past medical history (T) were assessed, producing an Oxford-MEST score. Cases was significant for obesity in four patients, diabetes in two with endocapillary proliferation, necrosis, or cellular cres- patients, and coronary artery disease and chronic obstructive cents were excluded, and an Oxford-MEST score of either pulmonary disease in one patient each. No secondary causes M1E0 or M0E0 was required. We identified a total of 17 of IgAN were identified (e.g., chronic liver disease, HIV in- cases, which originated from multiple nephrology centers fection, and inflammatory bowel disease). in the northeastern United States. All cases were processed Laboratory findings at the time of biopsy are summarized for light microscopy, immunofluorescence, and electron in Table 1. Fourteen patients (82.4%) had preserved renal microscopy according to standard techniques. For immu- function (SCr#1.2) at the time of biopsy. The median 24- nofluorescence, 3-mm cryostat sections were stained with hour urine protein was 8.0 g/d (range=3.0–18.0 g), and me- FITC-conjugated rabbit anti-human IgG, IgM, IgA, C3, dian serum
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