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Table 3 Pediatric Cases of ANCA-Vasculitis Associated with Cardiac Involvement

From: ANCA-associated vasculitis with cardiac valve vegetations in two teenage males: two case reports and a literature review

Author

Patient Age, Sex

Onset of Cardiac Involvement

Description of Cardiac Involvement

Other organ involvement

ANCA-titer at time cardiac involvement found (pattern and antigen)

Treatment

Outcome

N/A

15yo, M

Diagnosis

thickened aortic valve leaflets with perforation within the right coronary leaflet as well as 1–2 small areas with vegetation on the aortic valve with mild aortic regurgitation, as well as thickening of the anterior leaflet of the mitral valve

Upper and lower respiratory tract, renal, cutaneous, CNS (thromboembolic stroke)

C-ANCA pattern 1:640, PR3 812 AU/mL

Pulse glucocorticoids (1GMx3 days), 5 days TPE, followed by methylprednisolone 2 mg/kg/day, Rituximab 1000 mg week 0 and week 2, followed by 1000 mg Rituximab every 6 months

Normalization of renal function, resolution of inflammatory markers and ANCA titers, recovery of symptoms; ultimately remission on treatment

N/A

16yo, M

Diagnosis

small mitral valve vegetation, likely perforation of the anterior leaflet near the medial annulus

Lower respiratory tract, cutaneous, renal

c-ANCA pattern 1:5120, PR3 1242 AU/mL

Pulse glucocorticoids (1GMx3 days), 5 days TPE, followed by methylprednisolone 2 mg/kg/day, Rituximab 375 mg/m2 once weekly for 4 doses, followed by 1000 mg Rituximab every 6 months

Improvement in renal function, resolution of inflammatory markers and ANCA titers, recovery of symptoms; ultimately remission on treatment

Harris et al. [19]

14yo, F

Diagnosis

Ovoid, homogenous pedunculated mass in apex of left ventricle with EF 40%

Upper and lower respiratory tract, cutaneous, arthritis, renal

C-ANCA (titer not available);

PR3 148 EU/mL

Pulse glucocorticoids (30 mg/kg) followed by methylprednisolone 2 mg/kg/day, cyclophosphamide 1 mg/kg/day, 7 days TPE; mass resection

Unknown

Kosovsky et al. [20]

16yo, M

Diagnosis

Ventricular tachycardia, mass involving full thickness of right anterior ventricular wall extending into base of papillary muscles of the anterior leaflet of the tricuspid valve; biopsy revealed acute and organizing granulomatous vasculitis

Upper and Lower respiratory tract

ANCA studies negative

Cyclophosphamide (exact dosing not provided), Prednisone (exact dosing not provided)

Unknown

Varnier et al. [21]

16yo, M

Diagnosis

Vegetation adjacent to tricuspid valve

Cutaneous, upper and lower respiratory tract, renal, genital

C-ANCA (titer not available); PR3 194 EU/mL

Methylprednisolone pulse 1 g daily for 3 consecutive days followed by high dose oral prednisolone; 10 days TPE; Rituximab 1 g at week 0 and 2; Cyclophosphamide 500 mg/m2 every 3 weeks for 4 doses

Normalization of renal function, resolution of inflammatory markers, recovery of symptoms; ultimately remission on treatment with prednisolone 3 mg daily, Azathioprine 125 mg daily, Amlodipine 5 mg daily

Leff et al. [22]

17yo, M

Diagnosed 1 year after treatment

Mild LV enlargement, AI, AV perforation

Cutaneous, upper and lower respiratory tract, arthritis

C-ANCA 1:512; PR3 (exact unavailable)

Oral cyclophosphamide 150 mg/day, prednisone 40 mg/day

AV replacement due to progressive insufficiency; flare -- > re-initiation of cyclophosphamide, oral prednisone, Bactrim

Moghadam et al. [23]

10yo, M

Diagnosis

Diffuse ectasia and dilation with large aneurysm in the left anterior descending artery (14 mm)

Perforated otitis media with effusion, saddle nose deformity, mastoiditis, sinusitis, deep vein thrombosis

P-ANCA (titer not available); antigen not provided

Methylprednisolone pulse (30 mg/kg/day for 3 days/monthly), cyclophosphamide 750 mg/m2/monthly for 6 months. Oral prednisolone 1 mg/kg/day, mycophenolate mofetil 1200 mg/m2/day. Aspirin 5 mg/kg, warfarin 0.1 mg/kg.

Maintenance therapy with low-dose prednisolone, mycophenolate mofetil, antithrombotic therapy. No flare 1.5 years after diagnosis.

  1. Abbreviations: EF Ejection Fraction, LV Left Ventricular, AI Aortic Insufficiency, AV Aortic Valve, TPE Therapeutic Plasma Exchange