Table 1.
Time to diagnosis, disease activity measures and outcomes in a cohort of 11 patients with childhood Takayasu arteritis a
| Patient number | Time to diagnosis (mo) | PVAS/ITAS2010/DEI.Tak at diagnosis | Surgical/endovascular intervention(s) | Follow-up (mo) | Status at last follow-up |
|---|---|---|---|---|---|
| 1 | 42 | 1/6/5 | Repair of LV aneurysm (twice), ascending aorta aneurysm repair, AMPLATZER Septal Occluder device (AGA Medical, Golden Valley, MN, USA) insertion, stent insertion within ascending aorta, coil embolisation of right CA aneurysm | 11 | Remission on treatment |
| 2 | 2 | 10/15/12 | Mitral valvuloplasty and aortic root replacement, subsequent LIMA bypass graft for stenotic left CA | 32 | Remission on treatment |
| 3 | 4 | 5/16/7 | – | 16 | Relapse on treatment |
| 4 | 7 | 10/15/9 | – | 36 | Remission on treatment |
| 5 | 132 | 2/6/2 | – | 24 | Remission off treatment |
| 6 | 0 | 2/10/6 | Angioplasty of both renal arteries, SMA and external iliac arteries; coil embolisation of a cerebral artery aneurysm | 168 | Deceased |
| 7 | 120 | 5/9/7 | – | 11 | Deceased |
| 8 | 17 | 3/8/8 | – | 6 | Remission on treatment |
| 9 | 18 | 1/6/5 | – | 6 | Remission off treatment |
| 10 | 0 | 3/10/5 | Angioplasty of the renal arteries (twice) | 26 | Remission on treatment |
| 11 | 60 | 12/9/12 | Angioplasty of abdominal aorta; required subsequent surgical repair | 14 | Deceased |
aCA, Coronary artery; DEI.Tak, Disease Extent Index-Takayasu; ITAS2010, Indian Takayasu Arteritis Activity Score; LIMA, Left internal mammary artery; LV, Left ventricle; PVAS, Paediatric Vasculitis Activity Score; SMA, Superior mesenteric artery. Total possible scores range from 0 to 63 for PVAS, from 0 to 57 for ITAS2010 and from 0 to 81 for DEI.Tak (0–81). Higher scores reflect higher disease activity for all tools [16-18].