title: Dense Deposit Disease Mimicking a Renal Small Vessel Vasculitis.
abstract: Dense deposit disease is caused by fluid-phase dysregulation of the alternative complement pathway and frequently deviates from the classic membranoproliferative pattern of injury on light microscopy. Other patterns of injury described for dense deposit disease include mesangioproliferative, acute proliferative/exudative, and crescentic GN. Regardless of the histologic pattern, C3 glomerulopathy, which includes dense deposit disease and C3 GN, is defined by immunofluorescence intensity of C3c two or more orders of magnitude greater than any other immune reactant (on a 0-3 scale). Ultrastructural appearances distinguish dense deposit disease and C3 GN. Focal and segmental necrotizing glomerular lesions with crescents, mimicking a small vessel vasculitis such as ANCA-associated GN, are a very rare manifestation of dense deposit disease. We describe our experience with this unusual histologic presentation and distinct clinical course of dense deposit disease, discuss the pitfalls in diagnosis, examine differential diagnoses, and review the relevant literature.
authors.affiliation: Department of Pathology and.
authors.forename: Lavleen
authors.initials: L
authors.lastname: Singh
authors.affiliation: Department of Pathology and ggmed22@gmail.com.
authors.forename: Geetika
authors.initials: G
authors.lastname: Singh
authors.affiliation: Division of Pediatric Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, Delhi, India.
authors.forename: Swati
authors.initials: S
authors.lastname: Bhardwaj
authors.affiliation: Division of Pediatric Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, Delhi, India.
authors.forename: Aditi
authors.initials: A
authors.lastname: Sinha
authors.affiliation: Division of Pediatric Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, Delhi, India.
authors.forename: Arvind
authors.initials: A
authors.lastname: Bagga
authors.affiliation: Department of Pathology and.
authors.forename: Amit
authors.initials: A
authors.lastname: Dinda
mesh_terms: D002648:Child; D003937:Diagnosis, Differential; D015432:Glomerulonephritis, Membranoproliferative / Q000175:diagnosis*; D006801:Humans; D007668:Kidney / Q000098:blood supply*; D008297:Male; D014657:Vasculitis / Q000175:diagnosis*
keywords: C3 glomerulopathy; C3GN; dense deposit disease; necrotizing crescentic GN