Table 1 Characteristic features of the patients with cTA and dissection
From: Arterial dissection in childhood Takayasu Arteritis: not as rare as thought
Patient 1 | Patient 2 | Patient 3 | |
|---|---|---|---|
Age at diagnosis | 14.6 years | 12.4 years | 10.3 years |
Age at symptom onset | 14.4 years | ? | 10.0 years |
Gender | Female | Female | Female |
Ethnicity | First Nations | African | East Indian |
Presenting symptoms | Constitutional symptoms, abdominal pain, headache, cough, chest pain, dyspnea | Constitutional symptoms, bilateral cervical lymphadenopathy | Constitutional symptoms, vomiting, left focal seizures with secondary generalization |
Clinical findings | Arterial hypertension, weak femoral and pedal pulses, BP discrepancy of 50 mmHg, cardio-respiratory failure | Decreased left radial and peripheral pedal pulses | Arterial hypertension, midline abdominal bruits, BP discrepancy, pulsatile right cervical mass |
Laboratory findings | WBC 13.6 × 109/L Neutrophils 12.3 × 109/L Hb 83 g/l ESR 31 mm/h CRP 14.5 mg/dL Albumin 25 g/L Creatinine 123 umol/l | WBC 5.17 × 109/L Neutrophils not done Hb 144 g/l ESR 32 mm/h CRP not done Albumin 36 g/L | WBC 22.3 × 109/L Neutrophils 19.1 × 109/L Hb 115 g/l ESR 40 mm/h CRP 24.2 mg/dL Albumin 36 g/L |
Radiological findings | CTA and MRI/A: Thickening of entire abdominal aorta with dilatation and post-contrast enhancement of the wall. Dissection of the abdominal aorta with intramural hematoma. Severe narrowing of SMA, celiac artery and both renal arteries. | MRI/A: Extensive arteritis involving aortic arch, entire descending aorta and upper abdominal aorta with lumen irregularity, vessel wall thickening and post-contrast enhancement. Descending aorta with two areas of dilatation, in between narrowing with evidence of dissection. L subclavian artery severely narrowed. | MRI/A: Thickening and post-contrast enhancement of the abdominal aorta with evidence of an aneurysm. R carotid artery aneurysm with perivascular edema and vessel inflammation, intramural hematoma and suspected dissection flap. Bilateral renal artery stenosis; dilatation of the origin of the celiac trunk and SMA. |
PVAS (max. score 63) | 21 | 8 | 25 |
Treatment | Induction: Pulse Methylprednisolone, then high-dose Prednisone Methotrexate orally Infliximab every 4 weeks | Induction: High dose Prednisone | Induction: High-dose Prednisone Methotrexate subcutaneously |
Other: Quadruple anti-hypertensive therapy ASA | Other: Anti-tuberculosis therapy | Other: Quintuple anti-hypertensive therapy ASA | |
Follow-up duration | 26 months | 6 years | 36 months |