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2014 ; 9
(6
): 1033-9
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IgA nephropathy with minimal change disease
#MMPMID24721885
Clin J Am Soc Nephrol
2014[Jun]; 9
(6
): 1033-9
PMID24721885
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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 study sought to better characterize these
patients. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: A retrospective review
of cases of IgA nephropathy diagnosed from 2004 to 2011 identified patients with
nephrotic range proteinuria and histologically mild IgA nephropathy.
Specifically, using the Oxford Classification of IgA Nephropathy, we identified
cases that lacked endocapillary proliferation or segmental sclerosis. RESULTS:
The cohort consisted of 17 patients, including 10 men and 15 adults. The median
serum creatinine was 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 nephrotic, whereas the remaining
3 patients fulfilled two of three criteria for nephrotic syndrome. Biopsies
revealed IgA-dominant or codominant deposits accompanied by mesangial
proliferation in 14 patients (82.4%). Electron microscopy showed mesangial
deposits and extensive foot process effacement (median=90%). Initial treatment
consisted of corticosteroids, although many patients required additional agents
to maintain remission status. Over a median follow-up of 20 months (2.2-82
months), 14 patients experienced a complete response, and 3 patients showed a
partial response, with a median response time of 2 months (0.5-27 months). At
least one relapse of nephrotic syndrome occurred in nine patients (53%). All
patients exhibited stable or improved 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.
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