Table 1.
Baseline characteristics of 40 patients with IgG4-related aortitis/periaortitis and periarteritis
| Location of vascular lesion | Luminal dilatation before Tx | Risk factor of arteriosclerosis | Initial PSL Tx (mg/day) | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Follow-up | IgG | IgG4 | IgE | CRP | Extravascular lesions | ||||||
| Patient | (mo) | (mg/dl) | (mg/dl) | (IU/ml) | (mg/dl) | Symptoms | |||||
| 1 | 204 | 1,894 | 128 | 543 | 6.2 | AA | (−) | La, Sa | abdo P, fever | (−) | 30 |
| 2 | 120 | 2,840 | 693 | 468 | 0.1 | AA | (−) | Sa | (−) | DM, Sm | 30 |
| 3 | 96 | 5,970 | 3,100 | 259 | 1.56 | AA | (−) | La, Sa, Hy, Pa | malaise | DM, HT | 40 |
| 4 | 78 | 2,140 | 557 | 266 | <0.10 | AA, IA | (+) | La, Sa | (−) | DL, Sm | 50 |
| 5 | 70 | 1,500 | 173 | 151 | 0.3 | IA, SMA | (−) | Pa, RF | abdo P | DM, HT, DL, Sm | 20 |
| 6 | 63 | 2,970 | 1,330 | 419 | <0.10 | AA | (−) | La, Sa, Pa, Ki, RF | malaise | DM, HT, DL, Sm | 40 |
| 7 | 63 | 2,130 | 715 | 253 | <0.10 | IA | (−) | La, Sa | (−) | DM, DL, Sm | 40 |
| 8 | 63 | 2,731 | 269 | 975 | 0.6 | AA | (−) | Sa, RF | (−) | DM, HT, DL, Sm | 30 |
| 9 | 57 | 1,790b | 105b | 212b | 1.5b | AA, IA | (+) | Hy, Lu, Pa, Ly | (−) | DL | 40 |
| 10 | 54 | 2,570 | 1,420 | 345 | 0.3 | AA, IA | (+) | Pa | (−) | DM, HT | 20 |
| 11 | 48 | 2,950 | 1,540 | 7.9 | <0.1 | AA | (−) | Sa, Bi, Pa, Pr | (−) | DM | 30 |
| 12 | 43 | 1,487 | 196 | 447 | 0.6 | AA, IA | (−) | RF | abdo P | DM, HT, DL, Sm | 0 |
| 13 | 37 | 2,563 | 1,330 | 283 | 0.09 | AA | (−) | Sa, Ki | pollakiuria | DL, Sm | 45 |
| 14 | 35 | 2,319 | 734 | 542 | 1.19 | TA, AA | (−) | Sa, Pa, Ki | (−) | DM, DL | 40 |
| 15 | 34 | 1,458 | 158 | 452 | 0.22 | AA | (−) | Sa, Ly | (−) | DM, HT, DL, Sm | 30 |
| 16 | 27 | 2,081 | 870 | 1,285 | 0.0 | AA, IA | (−) | Sa, RF, Pr | (−) | DM, HT | 20 |
| 17 | 27 | 1,756 | 408 | 513 | 0.2 | AA, IA | (−) | Sa, Pa, Ki | (−) | DM, HT, DL, Sm | 20 |
| 18 | 27 | 1,762 | 144 | 24 | 0.32 | AA, IA | (+) | Pa | (−) | DL, Sm | 20 |
| 19 | 25 | 2,024 | 292 | 1,400 | 0.14 | AA | (−) | Pa, RF | (−) | DM, HT, Sm | 40 |
| 20 | 24 | 2,262 | 299 | 443 | <0.05 | AA | (−) | (−) | (−) | HT | 0 |
| 21 | 24 | 2,184 | 236 | 365 | 0.3 | AA | (+) | La, Sa | fever | DM, HT, DL | 30 |
| 22 | 22 | 3,484 | 1,896 | 247 | 0.0 | AA | (−) | La, Sa, Pa, Ki, Ly | (−) | Sm | 35 |
| 23 | 15 | 4,171 | 2,120 | <20 | 0.32 | AA, IA | (−) | La, Sa, Ki | (−) | DM, HT, Sm | 40 |
| 24 | 13 | 1,837 | 261 | 687 | 0.06 | IA | (−) | RF | (−) | HT, DL | 30 |
| 25 | 13 | 1,454 | 196 | 350 | 0.13 | AA, IA | (−) | (−) | abdo P | Sm | 15 |
| 26 | 10 | 2,213 | 455 | NA | 0.56 | AA, IA | (−) | Sa, Ki | arthralgia | HT, DL, Sm | 40 |
| 27 | 10 | 3,120 | 1,020 | 1,760 | 1.5 | AA, IA, IMA | (−) | La, Sa | hoarseness, fever | Sm | 30 |
| 28 | 9 | 2,936 | 1,070 | 17 | 0.0 | TA, AA, IA | (−) | La, Sa, Pa, Ki | thirst | DM, DL, Sm | 40 |
| 29 | 9 | 10,121 | 2,500 | <20 | 0.37 | IA | (+) | Pa, Ki, Ly | malaise | DM | 50 |
| 30 | 9 | 1,200 | 147 | NA | 2.94 | AA, IA | (−) | (−) | fever, malaise | DM, HT | 30 |
| 31 | 8 | 1,475 | 210 | 111 | 0.3 | IA | (−) | Sa | (−) | (−) | 40 |
| 32 | 4 | 2,938 | 1,520 | 48 | 0.1 | AA | (−) | La, Sa, Ki, RF | (−) | Sm | 30 |
| 33 | 4 | 1,463 | 672 | 216 | 0.13 | AA | (−) | Sa, Pl, Ca | (−) | Sm | 0 |
| 34 | 3 | 2,439 | 782 | 703 | 0.2 | AA, IA | (−) | La, Ki, RF | (−) | DM, DL, Sm | 20 |
| 35 | 3 | 2,244 | 503 | 311 | 0.0 | AA, IA | (−) | Ki | edema | (−) | 30 |
| 36 | 2 | 1,950 | 711 | 737 | 0.0 | AA | (−) | La, Sa, Lu, Ki | (−) | Sm | 35 |
| 37 | 2 | 1,328 | 106 | 19 | 0.28 | AA, IA | (+) | RF | (−) | DL | 0 |
| 38 | 1 | 4,420 | 2,680 | 174 | 0.1 | IA | (−) | La, Sa, Bi, Pa, Ki, Ne | diarrhea | Sm | 50 |
| 39 | 1 | 2,276 | 835 | <20 | 0.91 | IA | (−) | Sa | (−) | HT | 15 |
| 40 | 1 | 1,600 | 206 | 212 | 0.38 | AA | (−) | (−) | abdo P, malaise | Sm | 30 |
AA, Abdominal aorta; abdo, Abdominal; Bi, Bile tract; Ca, Pericarditis; CRP, C-reactive protein; DL, Dyslipidemia; DM, Diabetes mellitus; F, Female; HT, Hypertension; Hy, Hypophysitis; IA, Iliac artery; IgE, Serum immunoglobulin E at diagnosis; IgG, Serum immunoglobulin G at diagnosis; IgG4, Serum immunoglobulin G4 at diagnosis; IMA, Inferior mesenteric artery; Ki, IgG4-related kidney disease; La, Lacrimal gland, Lu, Lung; Ly, Lymph node; M, Male; Mo, month; NA, Not available; Ne, Nerve; P, Pain; Pa, Pancreas; Pl, Pleuritis; Pr, Prostate; PSL, Prednisolone; RF, Retroperitoneal fibrosis; Sa, Salivary gland; Sm, Past or current smoking; SMA, Superior mesenteric artery; TA, Thoracic aorta; Tx, Treatment. bValue under corticosteroid therapy.