title: C1q Nephropathy: The Unique Underrecognized Pathological Entity.
abstract: C1q nephropathy is a rare glomerular disease with characteristic mesangial C1q deposition noted on immunofluorescence microscopy. It is histologically defined and poorly understood. Light microscopic features are heterogeneous and comprise minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), and proliferative glomerulonephritis. Clinical presentation is also diverse, and ranges from asymptomatic hematuria or proteinuria to frank nephritic or nephrotic syndrome in both children and adults. Hypertension and renal insufficiency at the time of diagnosis are common findings. Optimal treatment is not clear and is usually guided by the underlying light microscopic lesion. Corticosteroids are the mainstay of treatment, with immunosuppressive agents reserved for steroid resistant cases. The presence of nephrotic syndrome and FSGS appear to predict adverse outcomes as opposed to favorable outcomes in those with MCD. Further research is needed to establish C1q nephropathy as a universally recognized distinct clinical entity. In this paper, we discuss the current understanding of pathogenesis, histopathology, clinical features, therapeutic options, and outcomes of C1q nephropathy.
authors.affiliation: University of MO, 1 Hospital Drive, Columbia, MO 65201, USA.
authors.forename: Joe
authors.initials: J
authors.lastname: Devasahayam
authors.affiliation: South West Acute Hospital, Enniskillen BT74 4RT, UK.
authors.forename: Gowrishankar
authors.initials: G
authors.lastname: Erode-Singaravelu
authors.affiliation: Wayne State University, 42 W. Warren Avenue, Detroit, MI 48202, USA.
authors.forename: Zeenat
authors.initials: Z
authors.lastname: Bhat
authors.affiliation: Sanford University, 1305 W. 18th Street, Sioux Falls, SD 57105, USA.
authors.forename: Tony
authors.initials: T
authors.lastname: Oliver
authors.affiliation: University of MO, 1 Hospital Drive, Columbia, MO 65201, USA.
authors.forename: Arul
authors.initials: A
authors.lastname: Chandran
authors.affiliation: Temple University, 1801 N. Broad Street, Philadelphia, PA 19122, USA.
authors.forename: Xu
authors.initials: X
authors.lastname: Zeng
authors.affiliation: Presence Covenant Medical Center, 1400 W. Park Street, Urbana, IL 61801, USA.
authors.forename: Paramesh
authors.initials: P
authors.lastname: Dakshinesh
authors.affiliation: Ball Memorial Hospital, 2401 W. University Avenue, Muncie, IN 47303, USA.
authors.forename: Unni
authors.initials: U
authors.lastname: Pillai
mesh_terms: D003167:Complement Activation / Q000276:immunology; D015922:Complement C1q / Q000276:immunology*; D006801:Humans; D007668:Kidney / Q000473:pathology / Q000648:ultrastructure; D007674:Kidney Diseases / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D015922:Complement C1q