| Case No. | Sex/Age at onset | Clinical symptoms other than fever | Applicable number to EULAR-PRINTO-PRES criteria/presence of bruit | Laboratory data at diagnosis | Duration from onset to diagnosis (days) | ITAS2010 at diagnosis | Treatment | Follow-up status at most recent US date | ||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| CRP (mg/dL) | ESR (mm/h) | Induction therapy | Present therapy | ITAS2010 | VDI | Follow-up period (months) | ||||||
| 1 | F/12 years | Knee pain | 3/Yes | 7.8 | 129 | 25 | 9 | MPT | PSL/AZP/MTX | 0 | 1 | 53 |
| 2 | M/9 years | Abdominal pain | 1/No | 8.2 | 132 | 21 | 1 | MPT | PSL/AZP | 0 | 1 | 55 |
| 3 | M/12 years | Abdominal pain | 2/Yes | 10.4 | 106 | 15 | 3 | MPT/IVCY | MMF | 0 | 2 | 70 |
| 4 | F/11 years | Lymphadenopathy | 4/Yes | 3.0 | 113 | 35 | 11 | MPT/IVCY | None | 0 | 1 | 91 |
| 5 | F/5 months | Cough | 2/No | 11.5 | 132 | 37 | 0 | MPT/IVCY | None | 0 | 0 | 60 |
| Case No. | Abdominal arteries (US/CECT) | Cervical arteries (US/CECT) | US undetectable lesions detected by CECT | |||||
|---|---|---|---|---|---|---|---|---|
| AAo | CA | Right RA | Left RA | SMA | Right CCA | Left CCA | ||
| 1 | −/＋ | −/− | −/＋ | −/− | ＋/＋ | −/− | ＋/＋ | Wall thickening of the left subclavian artery and trunk of right renal artery |
| 2 | −/－ | −/− | −/− | −/− | −/− | −/＋ | ＋/＋ | None |
| 3 | −/− | ＋/＋ | −/− | −/− | ＋/＋ | −/− | ＋/＋ | None |
| 4 | −/＋ | −/＋ | −/− | −/− | ＋/＋ | ＋/− | −/＋ | None |
| 5 | ＋/＋ | ＋/＋ | −/− | −/− | ＋/− | NA | NA | None |
| Study | Sex/Age at onset (years) | Clinical symptoms other than fever | Applicable number to EULAR-PRINTO-PRES criteria/Presence of a bruit | Laboratory data at diagnosis | Duration from onset to diagnosis (months) | US/Definitive image | Modality for definitive diagnosis | Follow-up period (months) | Present therapy | Relapse | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| CRP (mg/dL) | ESR (mm/h) | Abdominal | Cervical/Thoracic | |||||||||
| Tachibana et al | F/20 | Pain throughout entire body | 1/No | 7.5 | 99 | 4 | NA/+ | +/+ | CTA | 6 | PSL/MTX | Yes |
| Goel et al | M/18 | Dyspnea | 1/No | 6.0 | 35 | 6 | +/+ | +/+ | CTA | 3 | PSL/MTX | No |
| Sasae et al | F/20 | Periumbilical pain | 2/No | 9.1 | 119 | 3 | +/+ | +/+ | CTA | 4 | PSL | No |
| Sada et al | F/19 | Malaise | 1/No | 31.2 | 145 | 6 | NA/NA | +/+ | CTA | 24 | PSL/MTX | No |
| Agostinis et al | M/75 | Cough and neck pain | 1/No | 15.1 | 85 | 3 | NA/+ | +/+ | FDG-PET | 6 | PSL/MTX | No |
| Gupta et al | F/20 | Painful nodular skin lesions | 2/No | 15.2 | 77 | 12 | +/+ | NA/+ | MRA | NA | PSL | No |
| Ahasan et al | F/20 | Pain in all four limbs | 2/No | 1.2 | 62 | 2 | +/NA | NA/NA | NA | NA | PSL | NA |
| Thai et al | F/14 | Pain in left upper extremity | 4/Yes | 21000.0 | 154 | 4 | NA/NA | +/NA | MRA | NA | PSL | NA |
| Skoura et al | F/54 | None | 1/No | 2.0 | 80 | NA | +/NA | NA/+ | FDG-PET | 6 | PSL | NA |
| Uthman et al | F/28 | None | 1/No | NA | 150 | 18 | NA/NA | +/+ | DSA | 5 | PSL/MTX | NA |
| KiŞla Ekİncİ et al | F/12 | Painful lesion on the face | 1/No | 14.8 | 116 | 2 | +/+ | +/+ | MRA | 18 | PSL/MTX/MM F | Yes |
| Schmidt et al | F/38 | Joint pain | 1/No | 3.6 | 92 | 12 | NA/NA | +/+ | DSA | 14 | PSL/AZP | No |
| F/27 | Joint pain | 1/No | 24.6 | 120 | 36 | NA/NA | +/+ | DSA | 8 | PSL/MTX | Yes | |
