MPO-ANCA-Positive Anti-glomerular Basement Membrane Antibody Disease Successfully Treated by Plasma Exchange and Immunosuppressive Therapy

Figures & data

Table 1. Laboratory findings on admission.

Peripheral blood		Blood chemistry		Serology
WBC	11,800/mL	TP	6.0 g/dL	CRP	8.98 mg/dL
Band	0.5 %	Alb	2.4 g/dL	IgG	906 mg/dL
Seg	80.0 %	AST	14 U/L	IgA	416 mh/dL
Lymphocyte	11.0 %	ALT	7 U/L	IgM	110 mg/dL
Monocyte	8.0 %	LDH	355 U/L	ASO	43 U/mL
Eosinophil	0.5 %	CK	86 U/L	RF	1 U/mL negative
RBC	395 × 10^4mL	ALP	129 U/L	ANA
Hb	9.3 g/dL	T-Bil	0.6 mg/dL	C3	40 mg/dL
Ht	27.0 %	Amylase	192 U/L	C4	14 mg/dL
Plt	39.5 × 10^4 mL	Uric acid	6.7 mg/dL	CH50	52.7 U/mL
		BUN	51 mg/dL	PR3-ANCA	<10 EU
		Cr	5.96 mg/dL	MPO-ANCA	65 EU
		Na	131 mEq/L	Anti-GBM	38 EU
				antibody
		K	3.3 mEq/L
Urinalysis		Cl	97 mEq/L
Protein	2+	Ca	7.7 mg/dL
Occult blood	3+	IP	4.3 mg/dL
Sugar sediment	—	Blood sugar	92 mg/dL
RBC	>100HPF dysmorphic
WBC	10–19HPF
RBC cast	2LPF
Granular cast	2LPF
Urine chemistry
NAG	17.9 U/L
β2MG	0.25 m/ml
Creatinine clearance	5.5 ml/min/1.74 m^2

Note: MPO-ANCA, myeloperoxidase antineutrophil cytoplasmic antibody; GBM, glomerular basement membrane; NAG, N-acetyl-β-D-glucosaminidase; β2MG, β2 microglobulin; TP, total protein; AST, aspartate aminotransferase; ALT, alanine aminotransferase; LDH, lactate dehydrogenase; CK, creatine Kinase; ALP, alkaline phosphatase; BUN, blood urea nitrogen; IP, inorganic phosphorus; CRP, C-reactive protein; ASO, antistreptolysin O; ANA, anti-nuclear antibody; PR3, proteinase-3; RF, rheumatoid factor.

Figure 1. (A) Chest radiograph showing a few small obscure nodular shadows in bilateral lung (arrows). (B) Chest computed tomography examination showing multiple nodules (arrow heads). (C) Macrophages phagocytizing hemosiderin by cytology of broncho-alveolar lavage.

Figure 2. Clinical course.

Note: MPO-ANCA, myeloperoxidase antineutrophil cytoplasmic antibody; GBM, glomerular basement membrane; PSL, predonisolone; Cy, cyclophosphamide; Aza, azathioprine.

Figure 3. (A) Periodic acid–methenamine silver stain, glomeruli showing cellular crescent. (B) Periodic acid–methenamine silver stain, glomerulus showing destruction of Bowman’s capsule (arrows) and GBM (arrow head).

Figure 4. Immunofluorescence with anti-IgG antibody and anti-C3 antibody: capillary linear pattern. IgA, IgM, C4, and C1q were negative.

Note: Scale bars = 50 μm.