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Case Reports
. 2022 Jun 9:10:886371.
doi: 10.3389/fped.2022.886371. eCollection 2022.

Hypocomplementemic Atypical IgA Vasculitis: A Case Report

Melvin Chan  1 , Melisha Gayle Hanna  1 , Nicholas Willard  2 , Amy Treece  2 , Bradley Patton Dixon  1
Affiliations
Case Reports

Hypocomplementemic Atypical IgA Vasculitis: A Case Report

Melvin Chan et al. Front Pediatr. .

Abstract

IgA vasculitis (IgAV, also known as Henoch-Schönlein purpura or HSP) is a vasculitis of small vessels involving multiple organs, particularly of the joints, gastrointestinal tract, skin, and kidneys. Growing laboratory evidence has shown that complement plays a key role in the pathogenesis of IgAV, although direct evidence of this association in patients is lacking. We report a child with IgAV associated with clinical features of hypertension, nephrotic range proteinuria, acute kidney injury, and low serum C3, with histopathologic findings on renal biopsy of membranoproliferative glomerulonephritis with C3 and IgA co-dominance, and extensive complement derangements. This case report suggests that complement modifies the pathogenesis of IgAV, and further investigation into complement-targeted therapy in cases of refractory IgAV may be beneficial.

Keywords: HSP; IgA vasculitis; case report; hypocomplementemia; membranoproliferative glomerulonephritis.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Light microscopy showing prominent lobularization of glomeruli with both mesangial hypercellularity and endocapillary proliferation. Frequent glomerular inflammatory cells are also seen.
Figure 2
Figure 2
Electron microscopy showing sub endothelial deposits (black arrows) as well as basement membrane duplication or “tram-tracking” (clear arrows).
Figure 3
Figure 3
Immunofluorescence showing 2+ IgA deposits (left) and 2+ C3 deposits (right) with granular capillary loop and mesangial staining.
See this image and copyright information in PMC

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