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2015 ; 2015
(ä): 490413
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C1q Nephropathy: The Unique Underrecognized Pathological Entity
#MMPMID26640759
Devasahayam J
; Erode-Singaravelu G
; Bhat Z
; Oliver T
; Chandran A
; Zeng X
; Dakshinesh P
; Pillai U
Anal Cell Pathol (Amst)
2015[]; 2015
(ä): 490413
PMID26640759
show ga
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.