Diagnostic Performance and Prognostic Value of Extravascular Retention of 123I-Labeled Serum Amyloid P Component in Systemic Amyloidosis

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FIGURE 1.
PSDI and EVR₂₄ of all 167 patients with systemic amyloidosis (AA, AL, and ATTR types). Patients are subdivided according to intensity of scintigraphic uptake, represented by × (negative, n = 28), ○ (score 1+, n = 45), (score 2+, n = 32), and • (score 3+, n = 62). Dotted lines are reference limits of controls (>0.51 for PSDI and <50% for EVR₂₄), resulting in reference area in upper left quadrant.
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FIGURE 2.
Relationship between EVR₂₄, serum alkaline phosphatase (A), and endogenous creatinine clearance (B) in patients with AL amyloidosis. Solid lines are linear regression lines.
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FIGURE 3.
Five-year survival of 80 patients with AL amyloidosis. (A) Patients with (dotted line) and without (solid line) clinical involvement of heart. (B) Patients with high (>50%) (•) and with low (<50%) (○) EVR₂₄. (C) Patients stratified according to high (>50%) (•) and low (<50%) (○) EVR₂₄ and presence (dotted line) or absence (solid line) of clinical involvement of heart.

TABLE 1

Patient Characteristics and Organ Involvement

Parameter	Control	AA	AL	ATTR	Localized amyloidosis
Number of patients	20	60	80	27	22
Male:female	14:6	19:41	46:34	13:14	12:10
Age (y)	53.3 (17.0)	56.5 (14.7)	61.8 (10.2)	51.8 (10.5)	50.6 (12.1)
I. Heart
a. Mean thickness^* > 12 mm	1 (5%)	7 (12%)	36 (45%)	9 (33%)	0 (0%)
b. Heart failure^†	1 (5%)	3 (5%)	24 (30%)	2 (7%)	0 (0%)
c. Low-voltage electrocardiography	0 (0%)	4 (7%)	23 (29%)	1 (4%)	0 (0%)
Heart involvement (a, b, or c)	2 (10%)	11 (18%)	44 (55%)	9 (33%)	0 (0%)
II. Kidney
d. Proteinuria > 0.5 g/24 h	8 (40%)	39 (65%)	51 (64%)	1 (4%)	0 (0%)
e. Creatinine clearance < 60 mL/min	10 (50%)	41 (68%)	38 (48%)	3 (11%)	1 (5%)
Kidney involvement (d or e)	11 (55%)	54 (90%)	66 (83%)	4 (15%)	1 (5%)
III. Liver
f. Liver span > 16 cm	0 (0%)	0 (0%)	15 (19%)	0 (0%)	0 (0%)
g. Alkaline phosphatase > 180 kU/L	0 (0%)	3 (5%)	18 (23%)	0 (0%)	0 (0%)
Liver involvement (f or g)	0 (0%)	3 (5%)	24 (30%)	0 (0%)	0 (0%)
IV. Nervous tissue
h. Peripheral polyneuropathy	2 (10%)	2 (3%)	26 (33%)	21 (78%)	1 (5%)
i. Autonomic neuropathy	1 (5%)	7 (12%)	33 (41%)	20 (74%)	1 (5%)
Nerve involvement (h or i)	2 (10%)	8 (13%)	40 (50%)	23 (85%)	1 (5%)
Number of organs involved:
0	6 (30%)	4 (7%)	2 (3%)	2 (7%)	20 (91%)
1	12 (60%)	41 (68%)	20 (25%)	15 (56%)	2 (9%)
2	2 (10%)	10 (17%)	27 (34%)	9 (33%)	0 (0%)
3	0 (0%)	5 (8%)	24 (30%)	1 (4%)	0 (0%)
4	0 (0%)	0 (0%)	7 (9%)	0 (0%)	0 (0%)

↵* Of cardiac septum and left ventricular wall.
↵† According to New York Heart Association grades 3 or 4.
Data are mean, with SD in parentheses, or number, with percentage in parentheses.

TABLE 2

Retention Figures of ¹²³I-SAP (Percentage of Dose) at Different Intervals After Injection in Patients

Parameter	Control	AA	AL	ATTR	Localized amyloidosis
Plasma retention
10 min	105 (21)	95 (23)	90 (28)^*	99 (22)	105 (18)
4 h	72 (19)	56 (23)^*	52 (28)^†	68 (19)	74 (17)
PSDI	0.69 (0.13)	0.57 (0.17)^*	0.55 (0.19)^†	0.68 (0.11)	0.71 (0.11)
24 h	40 (13)	32 (16)	29 (17)^*	40 (14)	41 (12)
48 h	20 (10)	18 (9)	16 (10)	21 (7)	22 (8)
Body retention
TBR 24 h	76 (13)	86 (11)^†	85 (11)^†	75 (10)	69 (14)
TBR 48 h	56 (17)	71 (16)^†	71 (18)^†	53 (15)	47 (17)
EVR₂₄	36 (15)	54 (19)^†	56 (22)^†	35 (17)	29 (10)
EVR₄₈	36 (18)	53 (18)^†	55 (22)^†	32 (15)	26 (12)

↵* P < 0.05 vs. controls.
↵† P < 0.01 vs. controls.
TBR = total body retention.
Retention was measured in plasma, total body, and extravascular compartment of body. Data are mean, with SD in parentheses.

TABLE 3

Predictors of Survival of Patients with AL Amyloidosis During First 5 Years After Inclusion

Predictor	Hazard ratio	P
Univariate analysis
EVR₂₄ > 50%	2.3 (1.2–4.5)	0.01
The male sex	1.2 (0.7–2.1)	0.6
Age > 60	0.9 (0.5–1.5)	0.6
Multiple myeloma	2.2 (1.2–4.0)	0.01
I. Heart
a. Mean thickness^* > 12 mm	3.3 (1.8–5.9)	<0.001
b. Heart failure^†	5.1 (2.8–9.3)	<0.001
c. Low voltage electrocardiography	2.5 (1.4–4.5)	0.002
Heart clinical (a, b, or c)	4.2 (2.1–8.3)	<0.001
II. Kidney
d. Proteinuria > 0.5 g/24 h	1.2 (0.7–2.3)	0.5
e. Creatinine clearance < 60 mL/min	0.8 (0.4–1.3)	0.3
Kidney clinical (d or e)	1.6 (0.7–3.5)	0.3
III. Liver
f. Liver span > 16 cm	0.8 (0.4–1.8)	0.6
g. Alkaline phosphatase > 180 kU/L	1.4 (0.7–2.7)	0.3
Liver clinical (f or g)	1.2 (0.7–2.2)	0.6
IV. Nerves
h. Peripheral polyneuropathy	0.9 (0.5–1.7)	0.8
i. Autonomic neuropathy	1.2 (0.7–2.2)	0.5
Neuropathy clinical (h or i)	1.3 (0.7–2.3)	0.3
Number of organs involved (I–IV) > 2	2.4 (1.4–4.3)	0.002
Multivariate analysis (1)
Heart clinical	3.9 (2.0–7.8)	<0.001
EVR₂₄ > 50%	2.0 (1.1–3.9)	0.04
Multivariate analysis (2)
Heart failure^†	4.9 (2.6–9.1)	<0.001
EVR₂₄ > 50%	2.1 (1.1–4.0)	0.03