Table 6.
Type of granuloma, USIDNET Registry
| Type of Granuloma | Persons with PIDD and granulomas N=177 |
Ataxia telangiectasia N=2 |
Chronic Granulomatous Disease (CGD)a N=54 |
Common variable immunodeficiency disorders (CVID)b N=96 |
||||
|---|---|---|---|---|---|---|---|---|
| # | % | # | % | # | % | # | % | |
| Autoimmunity | 7 | 4% | 0 | 0% | 1 | 2% | 6 | 6% |
| Cardiovascular | 1 | 1% | 0 | 0% | 0 | 0% | 1 | 1% |
| Constitutional | 1 | 1% | 0 | 0% | 0 | 0% | 1 | 1% |
| Oral/dental | 1 | 1% | 0 | 0% | 0 | 0% | 0 | 0% |
| Endocrine - Metabolic | 1 | 1% | 0 | 0% | 0 | 0% | 1 | 1% |
| Gastrointestinal | 17 | 10% | 0 | 0% | 11 | 20% | 5 | 5% |
| Genitourinary | 10 | 6% | 0 | 0% | 8 | 15% | 1 | 1% |
| Hematologic-Lymphoid | 58 | 33% | 0 | 0% | 14 | 26% | 39 | 41% |
| Musculoskeletal | 1 | 1% | 0 | 0% | 1 | 2% | 0 | 0% |
| Neoplastic | 3 | 2% | 0 | 0% | 0 | 0% | 1 | 1% |
| Neurologic | 1 | 1% | 0 | 0% | 0 | 0% | 1 | 1% |
| Sino-Pulmonary | 88 | 50% | 0 | 0% | 19 | 35% | 56 | 58% |
| Skin | 29 | 16% | 2 | 100% | 11 | 20% | 9 | 9% |
| Classified as prominent granuloma | 75 | 42% | 2 | 100% | 24 | 44% | 34 | 35% |
a
Chronic Granulomatous Disease (CGD) defined as diagnosis of “CGD, uncertain genetic cause”, “Autosomal recessive CGD - p22 phox deficiency (CYBA)”, “Autosomal recessive CGD - p47 phox deficiency (NCF1)”, “Autosomal recessive CGD - p67 phox deficiency (NCF2)”, “Autosomal recessive CGD – p22 phox deficiency (CYBA)”, “Autosomal recessive CGD – p47 phox deficiency (NCF1)”, “X-linked chronic granulomatous disease (CYBB)”
b
Common variable immunodeficiency disorders (CVID) defined as diagnosis of “Common variable immunodeficiency disorders with unknown genetic basis”