Table 1.
Baseline characteristics of study participants.
| MACS (n = 59) | Adrenal CS (n = 12) | Pituitary CS (n = 51) | Ectopic CS (n = 7) | All participants (n = 129) | |
|---|---|---|---|---|---|
| Women, n (%) | 43 (72.9) | 9 (75.0) | 44 (86.3) | 5 (71.4) | 101 (78.3) |
| Caucasian, n (%) | 56 (94.9) | 12 (100.0) | 45 (88.2) | 7 (100.0) | 120 (93.0) |
| Age at diagnosis, median (IQR), years | 54 (48-65) | 56 (35-61) | 46 (37-59) | 42 (40-62) | 51 (41-62) |
| Body mass index, median (IQR), kg/m2 | 31.2 (27.4-38.0) | 31.6 (29.7-35.3) | 35.6 (30.5-43.7) | 31.5 (27.1-39.3) | 32.9 (28.5-40.6) |
| Duration of symptoms before diagnosis, median (IQR), months | - | 12.0 (12.0-29.2) | 36.0 (12.0-82.0) | 2.0 (2.0-9.0) | 12.0 (5.0-36.0) |
| Clinical characteristics | |||||
| Hypertension, n (%) | 47 (79.7) | 10 (83.3) | 41 (80.4) | 7 (100.0) | 105 (81.4) |
| Therapy with ≥3 antihypertensive medications, n (%) | 18 (30.5) | 4 (33.3) | 13 (25.5) | 2 (28.6) | 37 (28.7) |
| Hyperglycemia | |||||
| Prediabetes, n (%) | 19 (32.2) | 2 (16.7) | 16 (31.4) | 1 (14.3) | 38 (29.5) |
| Type 2 diabetes mellitus, n (%) | 18 (30.5) | 2 (16.7) | 21 (41.2) | 5 (71.4) | 46 (35.7) |
| Insulin therapy, n (%) | 10 (16.9) | 1 (8.3) | 10 (19.6) | 3 (42.9) | 24 (18.6) |
| Bone disease | |||||
| Osteopenia, n (%) | 15 (25.4) | 7 (58.3) | 17 (33.3) | 2 (28.6) | 41 (31.8) |
| Osteoporosis, n (%) | 10 (16.9) | 0 (0.0) | 9 (17.6) | 1 (14.3) | 20 (15.5) |
| Fragility fracture within 12 months, n (%) | 1 (1.7) | 0 (0.0) | 5 (9.8) | 2 (28.6) | 8 (6.2) |
| Dyslipidemia, n (%) | 38 (64.4) | 7 (58.3) | 33 (71.7) | 3 (60.0) | 81 (66.4) |
| Atherosclerotic cardiovascular disease, n (%) | 9 (15.3) | 0 (0.0) | 1 (2.0) | 2 (28.6) | 12 (9.3) |
| Venous thromboembolism within 12 months, n (%) | 0 (0.0) | 1 (8.3) | 4 (7.8) | 0 (0.0) | 5 (3.9) |
| Weight gain, n (%) | 28 (47.5) | 12 (100.0) | 48 (94.1) | 5 (71.4) | 93 (72.1) |
| Physical exam findings | |||||
| Central obesity, n (%) | 23 (39.0) | 10 (83.3) | 45 (88.2) | 7 (100.0) | 85 (65.9) |
| Supraclavicular and/or dorsocervical fat accumulation, n (%) | 15 (25.4) | 9 (75.0) | 42 (82.4) | 7 (100.0) | 73 (56.6) |
| Rounding of the face ± plethora, n (%) | 11 (18.6) | 9 (75.0) | 44 (86.3) | 7 (100.0) | 71 (55.0) |
| Skin changes (ie, violaceous striae, skin thinning, and/or bruising), n (%) | 17 (28.8) | 10 (83.3) | 39 (76.5) | 5 (71.4) | 71 (55.0) |
| Proximal muscle weaknessa, n (%) | 28 (47.5) | 9 (75.0) | 30 (58.8) | 6 (85.7) | 73 (56.6) |
| Biochemical assessment | |||||
| 1 mg DST, median (IQR), µg/dL | |||||
| Missing, n = 26 | 3.4 | 14.0 | 7.6 | - | 5.4 |
| Reference range: <1.8 µg/dL | (2.6-5.7) | (12.3-20.7) | (5.0-14.5) | (2.9-11.6) | |
| 8 mg DST, median (IQR), µg/dL | |||||
| Missing, n = 108 | 2.5 | 16.0 | 2.7 | 4.0 | 2.7 |
| Reference range: <1.0 µg/dL | (2.2-6.6) | (9.1-18.8) | (2.5-3.0) | (4.0-4.0) | (2.2-6.2) |
| 24-h urine cortisol, median (IQR), µg/24 h | |||||
| Missing, n = 34 | 23.0 | 197.0 | 103.5 | 803.0 | 70.0 |
| Reference range: <45 µg/24h | (15.5-38.0) | (113.0-273.1) | (49.8-216.5) | (440.0-1880.5) | (33.0-215.0) |
| Late night salivary cortisol, median (IQR), ng/dL | |||||
| Missing, n = 68 | 85.5 | 254.0 | 230.0 | 1540.0 | 230 |
| Reference range: < 100 ng/dL | (54.8-145.5) | (213.0-348.5) | (147.0-391.0) | (1185.5-1550.0) | (147.0-428.0) |
| ACTH, median (IQR), pg/mL | |||||
| Missing, n = 3 | 7.2 | <5.0 | 74.0 | 164.0 | 21.0 |
| Reference range: 5-63 pg/mL | (5.1-12.0) | (<5.0-<5.0) | (47.0-97.0) | (141.0-242.0) | (6.3-74.5) |
| DHEA-S, median (IQR), µg/dL | |||||
| Missing, n = 7 | 36.5 | 40.0 | 145.5 | 214.0 | 58.0 |
| Reference range age- and sex-dependent | (20.0-51.5) | (15.0-66.5) | (85.8-231.0) | (203.0-327.0) | (33.2-141.8) |
| CS disease severity score | |||||
| Clinical severity score, median (IQR) | 6.0 (3.0-9.5) | 15.5 (11.0-16.2) | 14.0 (11.0-17.5) | 17.0 (15.5-17.0) | 11.0 (6.0-16.0) |
| Clinical severity category, n (%) | |||||
| Mild, sum score 1-8 points | 39 (66.1) | 2 (16.7) | 6 (11.8) | 0 (0.0) | 47 (36.4) |
| Moderate, sum score 9-14 points | 14 (23.7) | 3 (25.0) | 20 (39.2) | 2 (28.6) | 39 (30.2) |
| Severe, sum score 15-22 points | 6 (10.2) | 7 (58.3) | 25 (49.0) | 5 (71.4) | 43 (33.3) |
| Biochemical severity score, median (IQR) | 3.0 (2.0-4.0) | 7.0 (6.0-8.0) | 6.0 (5.0-8.0) | 7.0 (7.0-10.0) | 5.0 (3.0-7.0) |
| Biochemical severity category, n (%) | |||||
| Mild, sum score 0-3 points | 37 (62.7) | 0 (0.0) | 4 (7.8) | 0 (0.0) | 41 (31.8) |
| Moderate, sum score 4-6 points | 21 (35.6) | 5 (41.7) | 22 (43.1) | 1 (14.3) | 49 (38.0) |
| Severe, sum score 7-11 points | 1 (1.7) | 7 (58.3) | 25 (49.0) | 6 (85.7) | 39 (30.2) |
Abbreviations: ACTH, corticotropin; CS, Cushing syndrome; DHEA-S, dehydroepiandrosterone sulfate; DST, dexamethasone suppression test; IQR, interquartile range; MACS, mild autonomous cortisol secretion.
a
Based on patient report and/or documented clinical physical exam.