Plasma exchange combined with immunosuppressive treatment in a child with rapidly progressive IgA nephropathy

Abstract

Although diffuse crescentic formation in immunoglobulin A (IgA) nephropathy, histologically characterized by extensive extracapillary proliferation, is assumed to have a poor prognosis, there has still been no established treatment because of the low prevalence of the condition, especially in pediatric patients. This paper reports on a 5-year-old boy with rapidly progressive IgA nephropathy requiring dialysis for 1 month. He had been treated with plasma exchange (PE) combined with immunosuppressive treatment, including steroids and mizoribine, because renal function deteriorated rapidly despite initial treatment with intravenous methylprednisolone pulse. The histological findings at that time revealed IgA nephropathy, with large circumferential cellular crescent formation in approximately 80% of the glomeruli. Three weeks after PE initiation, serum levels of creatinine and IgA-containing immune complexes returned to normal, and urinary protein excretion gradually decreased. The second renal biopsy taken 7 months later demonstrated mild IgA nephropathy with small fibrocellular crescents. This case report indicates that PE combined with immunosuppressive treatment may benefit children with rapidly progressive IgA nephropathy, even when extensive crescent formations are present.

Plasma Exchange as an Adjunctive Therapy for Crescentic IgA Nephropathy

“…One anecdotal report indicated a benefit of PE in combination with immunosuppressive therapies in 5 IgAN patients [9] . Furthermore, this has been confirmed in other case reports [10][11][12][13] .…”

“…Coppo et al [9] studied the effects of PE in 5 patients with progressive IgAN and found that PE delayed disease progression in the 3 patients with crescent formation, but did not delay progression in the patients with advanced sclerotic lesions [13] . In another 2 reports, PE treatment successfully restored kidney function in a child with CreIgAN and rapid renal function deterioration despite intravenous methylprednisolone therapy [10,22] . However, this benefit has not been observed in other reports [21] , while some reports have found that PE treatment only slowed renal function deterioration and delayed the timeto-dialysis dependence [11,13,17,20] .…”

“…Case reports and case series from previous decades have addressed the PE treatment of IgAN patients with rapidly progressive kidney failure and crescent formation on renal biopsy (online suppl. table S2) [9][10][11][12][13][17][18][19][20][21] . Coppo et al [9] studied the effects of PE in 5 patients with progressive IgAN and found that PE delayed disease progression in the 3 patients with crescent formation, but did not delay progression in the patients with advanced sclerotic lesions [13] .…”

Clinical and Pathological Studies of IgA Nephropathy Presenting as a Rapidly Progressive Form of Glomerulonephritis

“…However, a number of optimistic case series have recently been published indicating good preservation of the renal function obtained with the use of treatment regimens similar to those recommended for renal vasculitis; high-dose corticosteroids and cyclophosphamide, and in some cases, plasma exchange (6,17). Anecdotal reports claim variable degrees of benefit from plasma exchange in patients with active, severe IgAN (18,19). However, there have still not been any RCTs of these treatments in patients with crescentic IgAN, and the response to treatment is not uniform.…”

Altered P-selectin and CD44 expression in the renal tissues and peripheral blood of children with IgA nephropathy

“…Occurring in all age groups, with a peak incidence in the second and third decades, and in both sexes, with higher frequency in males, IgAN shows regional variations in frequency as a result of genetic differences or differences in renal biopsy practices [1][2][3][4]. For diagnosis of IgAN, many predictors of progression of IgAN, including arterial hypertension, episodic macroscopic hematuria, abnormal urine sediment and proteinuria, elevated serum creatinine level, hyperlipoproteinemia, hyperuricemia, and histological signs of glomerular sclerosis and interstitial fibrosis, are used clinically; all of these are associated with poor prognosis [5,6].…”