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. 2020 Oct-Dec;24(4):191-193.

Dense deposit disease in an adolescent male mimicking acute post-streptococcal glomerulonephritis

E Siomou  1 , G Liapis  2 , A Zisi  1 , D Csuka  3 , Z Prohászka  3
Affiliations

Dense deposit disease in an adolescent male mimicking acute post-streptococcal glomerulonephritis

E Siomou et al. Hippokratia. 2020 Oct-Dec.

Abstract

Background: Dense deposit disease (DDD), a subtype of complement factor 3 glomerulopathy (C3G), is a rare entity associated with dysregulation of the alternative complement pathway. It usually affects children, with a 50% likelihood of progression to end-stage renal disease within ten years of diagnosis. Description of the case: We report the case of an adolescent male with acute nephritic syndrome and nephrotic range proteinuria, initially diagnosed as acute post-streptococcal glomerulonephritis (APSGN). Despite his spontaneous improvement, renal biopsy, performed due to a persistently low C3 level for over 18 weeks, confirmed the diagnosis of DDD. Complement and genetic studies showed high levels of C3-nephritic factor and risk polymorphisms for developing the disease. He was treated with prednisolone and mycophenolate mofetil (MMF). At the last follow-up, 15 months from onset, the serum creatinine level and 24h-hour total protein excretion were normal.

Conclusion: C3G (including the DDD subtype) should be suspected in apparent APSGN with atypical clinical features at presentation/follow-up, even in the case of spontaneous improvement. Timely and accurate diagnosis, based on histopathological, complement, and genetic studies, is important to initiate the appropriate treatment aimed at preventing or slowing the disease progression. HIPPOKRATIA 2020, 24(4): 191-193.

Keywords: Complement factor 3 glomerulopathy; adolescent; child; dense deposit disease; post-streptococcal glomerulonephritis.

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Conflict of interest statement

The authors declare that they have no conflict of interest relevant to the material discussed in this article.

Figures

Figure 1
Figure 1. Light microscopy image (hematoxylin and eosin stain, x 400) showing a glomerulus with endocapillary proliferation and exudative features, with numerous neutrophils in the glomerular lumen and thickening of the glomerular basement membrane, and segmental lobular architecture (a sign of membranoproliferative glomerulonephritis).
Figure 2
Figure 2. Immunofluorescence image (C3, x 400) showing staining of the glomerular capillary walls and the mesangium in a discontinuous pattern, with some spherical structures.
Figure 3
Figure 3. Electron microscopy image (Uranyl acetate and lead citrate, x 2200) showing a segment of a large glomerulus with intramembranous and mesangial electron-dense deposits, unevenly distributed in a ribbon-like fashion, among the glomerular base membranes, with mild to moderate glomerular involvement.
See this image and copyright information in PMC

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