Fig. 1(A) Plasma adrenocorticotropic hormone (ACTH) and (B) serum dehydroepiandrosterone sulfate (DHEA-S) levels. Percentage suppression of (C) serum cortisol and (D) urine free cortisol (UFC) after high-dose dexamethasone suppression test in patients with Cushing disease (CD) and adrenal Cushing syndrome (CS).
Fig. 2Receiver operating characteristic (ROC) curves for plasma adrenocorticotropic hormone (ACTH), serum dehydroepiandrosterone sulfate (DHEA-S), percentage suppression of serum cortisol, and urine free cortisol (UFC) after the high-dose dexamethasone suppression test. The black line represents the results equivalent to chance.
Table 1Baseline Characteristics and Laboratory Results of Patients with Adrenal Cushing Syndrome and Cushing Disease
Characteristic |
Adrenal Cushing syndrome (n=57) |
Cushing disease (n=35) |
P value |
Age at diagnosis, yr |
40 (31-54) |
40 (28-54) |
0.834 |
Female sex |
51 (89.5) |
28 (80.0) |
0.230 |
Body mass index, kg/m2
|
24.1 (21.6-27.0) |
23.6 (22.2-26.4) |
0.816 |
24-Hour UFC, nmol/day |
1,535 (1,004-3,022) |
1,199 (611-1,961) |
0.136 |
Serum cortisol, nmol/L |
499 (417-624) |
593 (397-712) |
<0.001 |
ACTH, pmol/L |
3.1 (0.2-4.7) |
22.2 (12.1-29.9) |
<0.001 |
DHEA-S, nmol/L |
677 (453-1,982) |
6,321 (3,610-8,850) |
0.001 |
Serum cortisol after LDST, nmol/L |
535 (430-601) |
290 (212-444) |
0.001 |
UFC after LDST, nmol/day |
1,311 (806-2,324) |
312 (146-624) |
0.001 |
Percentage suppression of serum cortisol after HDST, % |
-0.7 (-13.3 to 17.5) |
88.5 (62.6-92.8) |
<0.001 |
Percentage suppression of UFC after HDST, % |
-3.1 (-78.2 to 24.0) |
94.4 (88.9-97.4) |
<0.001 |
Patients with repeated ACTH measurement |
29 (50.9) |
18 (51.4) |
0.959 |
Mass size (adrenal or pituitary), mm |
18.5 (10.0-30.0) |
2.2 (0.1-5.0) |
|
Patients with adrenal carcinoma |
1 (1.8) |
- |
|
Patients with pituitary adenoma of >10 mm |
- |
3 (8.6) |
|
Table 2Comparison of Clinical and Biochemical Characteristics in Patients with Adrenal Cushing Syndrome Based upon Suppression of Plasma ACTH
Characteristic |
Suppressed ACTH (n=21) |
Normal/high ACTH (n=36) |
P value |
Age at diagnosis, yr |
39 (32-54) |
42 (30-56) |
0.785 |
Female sex |
20 (95.2) |
31 (86.1) |
0.397 |
Body mass index, kg/m2
|
22.8 (20.7-27.1) |
24.1 (22.2-27.1) |
0.457 |
Adrenal mass size, mm |
30.0 (25.0-33.5) |
30.0 (25.0-34.8) |
0.784 |
24-Hour UFC, nmol/day |
1,120 (639-1,997) |
1,348 (593-1,886) |
0.832 |
Serum cortisol, nmol/L |
505 (436-668) |
499 (414-624) |
0.841 |
ACTH, pmol/L |
1.98 (2.11-2.18) |
4.44 (3.37-6.45) |
<0.001 |
DHEA-S, nmol/L |
478 (236-570) |
1,048 (627-2,589) |
0.017 |
Serum cortisol after LDST, nmol/L |
535 (430-706) |
532 (372-582) |
0.571 |
UFC after LDST, nmol/day |
1,018 (847-2,672) |
1,098 (599-2,139) |
0.415 |
Percentage suppression of serum cortisol after HDST, % |
4.6 (-17.9 to 18.8) |
-4.2 (-13.2 to 14.9) |
0.580 |
Percentage suppression of UFC after HDST, % |
-2.2 (-66.1 to 16.4) |
-7.3 (-91.1 to 24.7) |
0.878 |
Table 3Efficacy of Biochemical Parameters Used for Diagnosing Adrenal Cushing Syndrome
Parameter |
Cut-off value |
AUC |
95% CI |
Sensitivity, % |
Specificity, % |
PPV, % |
NPV, % |
ACTH, pmol/L |
5.3 |
0.954 |
0.889-0.987 |
84 |
94 |
96 |
79 |
DHEA-S, nmol/L |
2,464 |
0.810 |
0.693-0.936 |
86 |
85 |
92 |
73 |
Suppression of serum cortisol, % |
-33.3 |
0.950 |
0.877-0.986 |
96 |
91 |
94 |
94 |
Suppression of UFC, % |
-61.6 |
0.997 |
0.949-1.000 |
98 |
94 |
96 |
97 |
Table 4Characteristics of Patients with Cushing Disease with Adrenocortical Adenoma
Patient no. |
Sex |
Age, yr |
ACTH, pmol/L |
HDST, % |
Pituitary mass, mma
|
Adrenal mass, mma
|
Serum cortisol |
UFC |
1 |
F |
69 |
38.3 |
99 |
99 |
10 |
10 |
2 |
M |
66 |
3.5 |
89 |
97 |
5 |
10 |
3 |
F |
73 |
22.2 |
90 |
- |
5 |
10 |
Table 5Characteristics of Patients with Adrenal Cushing Syndrome with Pituitary Adenoma
Patient no. |
Sex |
Age, yr |
ACTH, pmol/L |
HDST, % |
DHEA-S, nmol/L |
Adrenal mass, mma
|
Pituitary mass, mma
|
Serum cortisol |
UFC |
1 |
F |
24 |
8.4 |
29.0 |
14 |
665 |
37 |
4 |
2 |
F |
33 |
3.5 |
19.0 |
-152 |
510 |
27 |
7 |
3 |
F |
21 |
8.4 |
-29.0 |
10 |
8,717 |
10 |
4 |
4 |
F |
27 |
2.0 |
-64.0 |
-467 |
185 |
27 |
3 |
5 |
F |
51 |
4.8 |
-111.0 |
11 |
- |
32 |
4 |
6 |
M |
40 |
5.4 |
-1.3 |
-10 |
1,878 |
30 |
3 |
7 |
F |
40 |
4.4 |
- |
- |
- |
10 |
2 |
8 |
M |
53 |
1.8 |
- |
-17 |
2,464 |
10 |
3 |
9 |
M |
57 |
3.7 |
6.7 |
-36 |
- |
20 |
5 |