Perimacular drusenoid deposits in a child with IgA nephropathy

Cetın, Nuran; Başmak, Hikmet; Gencler, Aylin; Açıkalın, Mustafa Fuat

doi:10.1016/j.jcjo.2017.07.014

Cited by 10 publications

(8 citation statements)

References 13 publications

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“…Indeed, drusen have been described in other forms of GN such as C3 glomerulopathy, dense deposit disease, and IgA GN, 11 , 28 , 29 , 30 , 36 , 37 but these diseases had already been excluded by renal biopsy. Retinal abnormalities with a similar appearance including hard exudates, inherited retinal dystrophies (Sorsby’s macular dystrophy, Malattia Leventinese, pattern macular dystrophy), the Alport fleck retinopathy, and epiretinal membranes were excluded by a retinal expert.…”

Section: Discussionmentioning

confidence: 99%

Retinal Drusen Are More Common and Larger in Systemic Lupus Erythematosus With Renal Impairment

Ham

Nicklason

Wightman

et al. 2022

Kidney International Reports

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Section: Discussionmentioning

confidence: 99%

Retinal Drusen Are More Common and Larger in Systemic Lupus Erythematosus With Renal Impairment

Ham

Nicklason

Wightman

et al. 2022

Kidney International Reports

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“…The demonstration of drusen in individuals with glomerulonephritis provides further evidence for a role for complement activation in glomerular disease. Drusen have been described in dense deposit disease 9 , 10 , lupus nephropathy 11 and IgA glomerulonephritis 12 , 13 , as well as case reports in membranous and post-streptococcal glomerulonephritis 14 . There is, however, no common complement activation pathway for all these diseases.…”

Section: Introductionmentioning

confidence: 99%

Retinal drusen in glomerulonephritis with or without immune deposits suggest systemic complement activation in disease pathogenesis

Harraka

Mack

Colville

et al. 2022

Sci Rep

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Retinal drusen are characteristic of macular degeneration and complement activation, but also occur in C3, lupus and IgA nephropathy. This cross-sectional observational study compared drusen counts in different forms of glomerulonephritis. Consecutive individuals with glomerulonephritis attending a general renal or transplant clinic underwent retinal imaging with a non-mydriatic camera. Drusen were counted in deidentified images by trained graders, compared with matched hospital patients, and correlated with clinical features. Eighty-four individuals with glomerulonephritis had a mean drusen count of 10 ± 27 compared with 3 ± 8 in hospital controls (p = 0.007). Fourteen individuals with glomerulonephritis (17%) and 4 hospital controls (4/49, 8%) had increased drusen counts (≥ 10) (p = 0.20). Increased drusen counts ≥ 10 were present in 13 (13/63, 21%) of those with glomerulonephritis and immune deposits [membranous (n = 8), antiglomerular basement membrane nephritis (n = 6), FSGS (n = 49)], and one of the 21 (5%) with glomerulonephritis without immune deposits [ANCA-associated (n = 15), minimal change disease (n = 6)]. In antibody-mediated glomerulonephritis (n = 14), mean drusen counts were 2 ± 3 in individuals with normal kidney function, 16 ± 41 with impaired function and 5 ± 7 with kidney failure . Mean counts were 24 ± 56 in individuals with glomerular IgG deposits and 1 ± 1 in those without (p = 0.76), and 23 ± 60 with complement deposits and 4 ± 8 in those without. Drusen counts were also less in immunosuppressed individuals (p = 0.049). The demonstration of retinal drusen in some forms of glomerulonephritis is consistent with systemic complement activation, and suggests that treatment targeting the complement pathways is worthwhile.

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“…IgA glomerulonephritis has been reported previously in both ulcerative colitis and Crohn’s disease 41 , 42 and was excluded in other study participants based on their normal urinary sediments. Drusen have been described previously in only occasional cases of IgA glomerulonephritis 21 , 43 but drusen and glomerular immune deposits in other forms of glomerulonephritis share their composition of complement, immunoglobulins, glycoproteins, and extracellular matrix 44 , 45 . Complement activation and complement risk alleles are important in both the pathogenesis of macular degeneration and Crohn’s disease, but also IgA glomerulonephritis.…”

Section: Discussionmentioning

confidence: 98%

Retinal drusen counts are increased in inflammatory bowel disease, and with longer disease duration, more complications and associated IgA glomerulonephritis

Nicklason

Ham

et al. 2022

Sci Rep

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Retinal drusen are deposits of inflammatory proteins that are found in macular degeneration and glomerulonephritis and result, in part, from complement activation. This was a cross-sectional observational study of individuals with inflammatory bowel disease (IBD) recruited from a Gastroenterology clinic who underwent non-mydriatic retinal photography. Deidentified images were examined for drusen, and drusen counts and size were compared with matched controls, and examined for clinical associations. The cohort with IBD comprised 19 individuals with ulcerative colitis, 41 with Crohn’s disease and three with indeterminate colitis, including 34 males (54%) and an overall median age of 48 (IQR 23) years. Their median IBD duration was 7 (IQR 10) years, median CRP level was 7 (IQR 14) mg/L, and 28 (44%) had complications (fistula, stricture, bowel resection etc.), while 28 with Crohn’s disease (68%) had colonic involvement. Drusen counts were higher in IBD than controls (12 ± 34, 3 ± 8 respectively, p = 0.04). Counts ≥ 10 were also more common (14, 22%, and 4, 6%, p = 0.02, OR 4.21, 95%CI 1.30 to 13.63), and associated with longer disease duration (p = 0.01, OR 1.06, 95%CI 1.00 to 1.13), an increased likelihood of complications (p = 0.003, OR 6.90, 95%CI 1.69 to 28.15) and higher CRP levels at recruitment (p = 0.008, OR1.02, 95%CI 1.00 to 1.05). Increased retinal drusen were found in all four individuals with Crohn’s disease and IgA glomerulonephritis. IBD and drusen may share pathogenetic mechanisms and underlying risk factors such as complement activation.

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Perimacular drusenoid deposits in a child with IgA nephropathy

Cited by 10 publications

References 13 publications

Retinal Drusen Are More Common and Larger in Systemic Lupus Erythematosus With Renal Impairment

Retinal Drusen Are More Common and Larger in Systemic Lupus Erythematosus With Renal Impairment

Retinal drusen in glomerulonephritis with or without immune deposits suggest systemic complement activation in disease pathogenesis

Retinal drusen counts are increased in inflammatory bowel disease, and with longer disease duration, more complications and associated IgA glomerulonephritis

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