C3 glomerulopathy in cystic fibrosis: a case report

C3 glomerulonephritis is a rare glomerulopathy characterized at renal biopsy by C3 deposition, alone or with scanty immunoglobulins, as well as by an electron-dense material in mesangium, subendothelial and subepithelial space. An abnormal systemic activation of the alternative pathway of the comple...

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Bibliographic Details
Published in:BMC nephrology 2018-03, Vol.19 (1), p.73-73, Article 73
Main Authors: Santoro, Domenico, Siligato, Rossella, Vadalà, Carmela, Lucanto, Mariacristina, Cristadoro, Simona, Conti, Giovanni, Buemi, Michele, Costa, Stefano, Sabadini, Ettore, Magazzù, Giuseppe
Format: Article
Language:English
Subjects:
C3 glomerulopathy
Case Report
Cystic fibrosis
Inflammation
Renal biopsy
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Summary:C3 glomerulonephritis is a rare glomerulopathy characterized at renal biopsy by C3 deposition, alone or with scanty immunoglobulins, as well as by an electron-dense material in mesangium, subendothelial and subepithelial space. An abnormal systemic activation of the alternative pathway of the complement cascade is responsible for the development of the disease if triggered by several possible environmental conditions. We report the first case in literature of a patient affected by cystic fibrosis and C3GN. Our case involves a young woman with cystic fibrosis, who had persistent microscopic hematuria, proteinuria and hypocomplementemia C3 for over three months. Renal biopsy confirmed the diagnosis of C3 glomerulopathy. Complement system dysregulation was tested and resulted in a strong terminal pathway activation proved by high levels of sC5b-9 complex, amounting to 1588 ng/ml (normal value
ISSN:1471-2369
1471-2369
DOI:10.1186/s12882-018-0880-y