Skip to main content

Table 1 Prevalence of PIDs among the 117 patients with various juvenile-onset autoimmune rheumatologic diseases

From: Primary complement and antibody deficiencies in autoimmune rheumatologic diseases with juvenile onset: a prospective study at two centers

 

Total (n = 117)

JIA (n = 84)

SLE (n = 21)

Vasculitis (n = 6)

MCTD (n = 1)

Scleroderma/SLE overlap syndrome

n (%)

n (%)

n (%)

n (%)

 

(n = 1)

PIDs

16 (13.7)

6 (7.2)

6 (28.6)

2 (33.3)a

1

1

C4D

7 (6)

3 (3.6)

3 (14.3)

1 (16.6)

0

0

C2D

3 (2.6)

1 (1.2)

2 (9.5)

0

0

0

SIgAD

5 (4.3)

2 (2.4)

1 (4.8)

0

1

1

Unclassified hypogamma globulinaemia

2 (1.7)

0

0

2 (33.3)

0

0

  1. PID primary immunodeficiency, C4D C4 complement deficiency, C2D C2 complement deficiency, SIgAD selective immunoglobulin A deficiency
  2. aOne patient with vasculitis has been diagnosed with hypogammaglobulinemia, also complete C4B deficiency