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2013 ; 2
(2
): 134-138
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AP-VAS 2012 case report: a case of lupus nephritis with predominant synchronous
cellular crescent formation and myeloperoxidase-antineutrophil cytoplasmic
antibody seropositivity
#MMPMID28509285
Makiishi T
; Shirase T
; Yamamoto S
; Maeda S
CEN Case Rep
2013[Nov]; 2
(2
): 134-138
PMID28509285
show ga
Several cases with an overlap of antineutrophil cytoplasmic antibody
(ANCA)-associated glomerulonephritis (GN) and lupus nephritis (LN) features have
been reported in recent years. However, the clinical and the pathologic features
of this condition, including mode of development, histology, and response to
treatment, are not fully understood. We report a 77-year-old woman who was
diagnosed with Sjögren syndrome 15 years previously. The patient presented with
acute worsening of renal function and was diagnosed with new-onset systemic lupus
erythematosus. A renal biopsy specimen revealed proliferative LN with synchronous
cellular crescents. She was also seropositive for myeloperoxidase-ANCA. Together
with the positive staining for immunoglobulins and complement factors on
immunofluorescence microscopy and scant subendothelial deposits by electron
microscopy, we reached a diagnosis of ANCA-associated crescentic GN overlapping
with LN. Although immunosuppressive treatment with methylprednisolone pulse
therapy and intravenous cyclophosphamide followed by oral predonisolone was
initiated, along with intermittent hemodialysis, these treatments did not induce
remission of her GN. Therefore, she continued regular intermittent hemodialysis.
However, she died because of candida pneumonia 4 months after admission.
Generally, the glomeruli of patients with ANCA-associated GN exhibit different
stages of crescents, namely cellular, fibrocellular, or fibrous. The
histologically synchronous crescents in this case indicate that ANCA-associated
GN overlapping with LN can progress more rapidly than that without LN. This
overlapping type of GN may be resistant to conventional immunosuppressive
therapies.