A case of minimal change nephrotic syndrome with immunoglobulin A nephropathy transitioned to focal segmental glomerulosclerosis

Abstract

A 50-year-old woman with a 1-month history of lower extremity edema and a 5 kg weight increase was admitted to our hospital with suspected nephrotic syndrome in October 1999. Urine protein level was 3.5 g per day, 10-15 erythrocytes in urine per high-power field, and serum albumin level 2.5 g/dl. Furthermore, an accumulation of pleural effusion was confirmed by chest X-ray. The results of a renal biopsy indicated slight mesangial proliferation in the glomeruli by light microscopy, and an immunofluorescence study confirmed the deposition of immunoglobulin (Ig) A and C3 in the mesangial area. Diffuse attenuation of foot processes and dense deposits in the mesangial area were observed by electron microscopy. Treatment with 40 mg/day of prednisolone was effective, and proteinuria was negative 1 month later. Because of this course, we diagnosed minimal change nephrotic syndrome complicated by mild-proliferative IgA nephropathy. In November 2000, there was a relapse of nephrotic syndrome, which was believed to be induced by an influenza vaccination, but response to increased steroid treatment was favorable, and proteinuria disappeared on day 13 of steroid increase. A second relapse in May 2001, showed steroid resistance with renal insufficiency, and an increase in the selectivity index to 0.195. Light microscopy revealed focal sclerotic lesions of the glomeruli, and an immunofluorescence study revealed attenuation of mesangial IgA and C3 deposition. These findings led to the diagnosis that minimal change nephrotic syndrome had transitioned to focal segmental glomerulosclerosis, whereby mesangial IgA deposition was reduced by immunosuppressive treatment. Subsequently, her renal function gradually worsened to the point of end-stage renal failure by 27 months after the second relapse of nephrotic syndrome.

IgA Nephropathy with Minimal Change Disease

“…The single pathologic finding that correlated with the presentation of full NS was extensive (median=90%) foot process effacement. The clinical and biopsy findings in these 17 patients, as well as similar cases that have been previously reported (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17), are most consistent with mild IgAN with superimposed MCD.…”

“…Many of the patients are rapidly responsive to treatment with corticosteroids. Such cases are uncommon but have been presented as case reports and in small series over the last 30 years (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). Some of these reports have suggested that the clinical and pathologic findings in these unusual cases of IgAN (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17), as well as the clinical outcomes (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17), are more typical of mild IgAN with superimposed MCD.…”

Estimated glomerular filtration rate and daily amount of urinary protein predict the clinical remission rate of tonsillectomy plus steroid pulse therapy for IgA nephropathy

“…IgA nephropathy patients without hematuria may have nephrosclerosis or hereditary nephritis with concomitant glomerular IgA deposition, because 4 % of normal persons without urinary abnormalities are reported to have glomerular IgA deposition on postmortem examination after accidental death [7]. Concomitant glomerular IgA deposition has been reported in hereditary nephritis, including thin basement membrane disease [8–10], mild Alport syndrome [11], focal segmental glomerulosclerosis [12], and complement factor abnormalities [13]. Moreover, the CR rate in patients without proteinuria (mainly hematuria alone) is relatively low, 60.8 % compared to approximately 73.0 % in patients with 0.3–0.69 g/day of urinary protein.…”