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2013 ; 2
(2
): 204-208
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Granulomatosis with polyangiitis associated with IgA nephropathy
#MMPMID28509292
Fukuhara D
; Kurayama R
; Ito Y
; Komagata Y
; Arimura Y
; Yan K
CEN Case Rep
2013[Nov]; 2
(2
): 204-208
PMID28509292
show ga
Granulomatosis with polyangiitis (GPA), previously referred to as Wegener's
granulomatosis, is a rare necrotizing granulomatous vasculitis, especially in
children. GPA affects small- to medium-sized vessels, leading to involvement of
multiple organs, including the upper and lower respiratory tracts and kidneys.
Glomerular lesions associated with GPA typically present as crescentic
glomerulonephritis with necrotizing lesions, with little or no staining for
immunoglobulins and complement proteins. We report a unique pediatric case of GPA
associated with IgA nephropathy, a representative immune-mediated glomerular
disease. The initial renal biopsy specimen revealed fibrous sclerosis and mild
mesangial proliferation without deposition of IgA. However, after clinical
remission of GPA by treatment, the serum IgA level continued to be significantly
higher than normal, and her paranasal sinusitis was poorly controlled. An acute
upper respiratory infection resulted in worsened urinary findings without any
systemic signs of GPA. The second renal biopsy specimen revealed deposition of
IgA and C3 in the mesangium. The patient was treated with oral prednisolone
alone, which led to complete remission of proteinuria within 1 month. IgA
nephropathy is possibly associated with GPA during remission stage, and serum IgA
level may be a valuable indicator to predict its association.