Table 2 Clinical features at presentation in patients with childhood ANCA-associated vasculitis

Systemic

21 (100%)

12 (100%)

9 (100%)

1

Cutaneous

7 (33%)

3 (25%)

4 (44%)

0.4

Petechiae/purpura

4

1

3

Rash

3

2

1

ENT

9 (43%)

1 (8%)

8 (89%)

< 0.01

Epistaxis

3

0

3

Sinusitis

8

1

7

Chronic nasal discharge

5

0

5

Subglottic involvement

1

0

1

Otitis/mastoiditis

2

0

2

ENT Biopsy

3 (14%)

0 (0%)

3 (33%)

0.06

Pulmonary

16 (76%)

8 (67%)

8 (89%)

0.33

Alveolar hemorrhage/hemoptysis

6

3

3

Respiratory failure

3

2

1

Pleural effusion

4

2

2

Pulmonary nodules

6

0

6

Pulmonary infiltrates

11

7

4

Cavitary lesions

4

0

4

Renal impairment

19 (90%)

12 (100%)

7 (78%)

0.17

Hematuria

16

10

6

Proteinuria

16

9

7

Hypertension

8

6

2

Creatinine at presentation, mg/dL (median, IQR)

1.56 (0.75–11.5)

6.6 (1.1–14.1)

1 (0.6–2.9)

0.16

Peak creatinine, mg/dL (median, IQR)

2.93 (0.75–11.5)

7.4 (1.3–14.1)

2.4 (0.6–6.2)

0.2

Renal Biopsy

16 (76%)

10 (83%)

6 (67%)

0.6

GI

6 (29%)

2 (17%)

4(44%)

0.33

Abdominal pain

6

2

4

Cardiovascular

4 (19%)

3 (25%)

1 (11%)

0.60

Pericarditis

4

3

1

Cardiomegaly

1

1

0

Nervous

4 (19%)

1 (8%)

3 (33%)

0.27

Facial paralysis

1

0

1

Mononeuritis

2

0

2

Headache

1

1

0

Anti-MPO positive (n, %)

12 (57%)

10 (83%)

2 (22%)

< 0.01

Anti-PR-3 positive (n, %)

10 (48%)

1 (8%)

9 (100%)

< 0.0001

Hemoglobin, g/dL

(median, IQR)

8.9 (5.8–10.4)

6.9 (5.3-9)

10.4 (9-11.2)

0.03