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2013 ; 2
(2
): 154-157
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AP-VAS 2012 case report: anti-glomerular basement membrane disease with high
titer of myeloperoxidase anti-neutrophil cytoplasmic antibody-an autopsy case
report
#MMPMID28509301
Otani M
; Shoji H
; Tomioka H
; Kaneda T
; Kida Y
; Kaneko M
; Fujii H
; Nakajima Y
; Katsuyama E
CEN Case Rep
2013[Nov]; 2
(2
): 154-157
PMID28509301
show ga
It has been reported that patients who are positive for both myeloperoxidase
anti-neutrophil cytoplasmic antibody (MPO-ANCA) and anti-glomerular basement
membrane (GBM) antibody have a poor prognosis. We present an autopsy case of
anti-GBM disease with a high titer of MPO-ANCA. The patient was a 77-year-old
woman with a medical history of idiopathic interstitial pneumonia. After being
treated for bacterial pneumonia, she was referred to our hospital for evaluation
of non-nephrotic range proteinuria, hematuria, and a course of rapidly
progressive glomerulonephritis. Results of urinalysis were 2+ for protein and 3+
for blood, with many dysmorphic red blood cells observed in the urinary sediment.
A sample of a 24-h urine collection contained 0.3 g protein. The serum creatinine
concentration was 5.0 mg/dl on admission. The patient tested positive for
MPO-ANCA at a titer of >640 EU and for anti-GBM antibody at a titer of 14 EU.
Renal biopsy revealed glomerulonephritis with crescent formation, and
immunofluorescence studies showed that the glomeruli had a generalized linear
fluorescence and anti-immunoglobulin G (IgG) and C(3) along the peripheral
glomerular capillaries. She was diagnosed with anti-GBM disease. Treatment was
started with intravenous prednisolone and oral cyclophosphamide, followed by
plasma exchange. Despite improved renal function, she died of pulmonary
hemorrhage. Autopsy revealed deposits of IgG and C(3) in the basement membranes
of lung alveoli.