Background
Findings
Hypothesis
Methods
Results
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 |
Patient 1
Patient 2
Patient 3
cTA with dissection (N = 3) | cTA without dissection (N = 24) |
P-value | |
---|---|---|---|
Age at diagnosis in years, mean (SD) | 12.4 (2.2) | 11.5 (3.7) | 0.37 |
Female (%) | 3 (100) | 17 (71) | 0.55 |
Tuberculosis (%) | 1 (33) | 2 (8) | 0.29 |
Arterial hypertension (%) | 2 (66) | 13 (54) | 1 |
Constitutional symptoms (%) | 3 (100) | 14 (58) | 0.27 |
PVAS, median (IQR) | 21 (8–25) | 10 (6–35) | 0.26 |
ESR in mm/h, median (IQR) | 32 (31–40) | 35 (1–109) | 0.89 |
Albumin in g/L, mean (SD) | 32.3 (6.4) | 41.1 (6.6) | 0.05 |
Hemoglobin in g/L, mean (SD) | 113.7 (31.0) | 112.8 (18.4) | 0.95 |
WBC in x109, mean (SD) | 13.7 (8.6) | 9.3 (3.0) | 0.07 |
Neutrophils in x109, mean (SD) | 15.7 (4.8) | 5.8 (2.3) | <0.0001 |
Platelets in x109, mean (SD) | 349 (183) | 401 (155) | 0.60 |
Imaging at diagnosis (%) | |||
MRI/A | 3 (100) | 5 (21) | 0.02 |
Conventional angiography | 0 | 6 (25) | 1 |
Combined (MRI/A, CTA, angiography) | 0 | 13 (54) | 0.22 |