Noninfectious complications associated with predominantly antibody deficiencies
| Comorbidities . | Number . | % (N = 28) . | DEFI study4 . | USIDNET5 . | Ho H en, Cunningham-Rundles C6 (N = 623) . | ||
|---|---|---|---|---|---|---|---|
| With autoimmune cytopenia (n = 55) . | Total (N = 311) . | With autoimmune cytopenia (n = 99) . | Total (N = 990) . | ||||
| Immune thrombocytopenia | 28 | 100% | 81.8% | 13.2% | 73.7% | 7.4% | 16.2% |
| AIHA | 11 | 39.3% | 30.1% | 5.5% | 11.1% | 1.1% | 7.7% |
| AIN | 5 | 17.9% | 18.2% | 3.2% | 10.1% | 1% | NS |
| Bronchechiasties | 6 | 21.4% | NS | NS | 12.9% | 11.2% | 6% |
| Gastrointestinal disease | 5 | 17.9% | NS | NS | 12.9% | 10.4% | 17.3% |
| Liver disease | 3 | 10.7% | NS | NS | 12.9% | 6.2% | 12.7% |
| Splenomegaly | 14 | 50% | 65.4% | 34.1% | 27.7% | 10.5% | 15.6% |
| Lymphadenopathy | 13 | 46.4% | NS | NS | 35.6% | 13.7% | 9% |
| Granulomas | 10 | 35.7%∗ | NS | NS | 14.9% | 6.8% | 9.3% |
| Lymphoma | 3 | 10.7% | NS | NS | 6.9% | 3.1% | 6.7% |
| Comorbidities . | Number . | % (N = 28) . | DEFI study4 . | USIDNET5 . | Ho H en, Cunningham-Rundles C6 (N = 623) . | ||
|---|---|---|---|---|---|---|---|
| With autoimmune cytopenia (n = 55) . | Total (N = 311) . | With autoimmune cytopenia (n = 99) . | Total (N = 990) . | ||||
| Immune thrombocytopenia | 28 | 100% | 81.8% | 13.2% | 73.7% | 7.4% | 16.2% |
| AIHA | 11 | 39.3% | 30.1% | 5.5% | 11.1% | 1.1% | 7.7% |
| AIN | 5 | 17.9% | 18.2% | 3.2% | 10.1% | 1% | NS |
| Bronchechiasties | 6 | 21.4% | NS | NS | 12.9% | 11.2% | 6% |
| Gastrointestinal disease | 5 | 17.9% | NS | NS | 12.9% | 10.4% | 17.3% |
| Liver disease | 3 | 10.7% | NS | NS | 12.9% | 6.2% | 12.7% |
| Splenomegaly | 14 | 50% | 65.4% | 34.1% | 27.7% | 10.5% | 15.6% |
| Lymphadenopathy | 13 | 46.4% | NS | NS | 35.6% | 13.7% | 9% |
| Granulomas | 10 | 35.7%∗ | NS | NS | 14.9% | 6.8% | 9.3% |
| Lymphoma | 3 | 10.7% | NS | NS | 6.9% | 3.1% | 6.7% |
Quantitative variables are presented as median (range, minimum to maximum); and qualitative variables as frequency (percentage).
Median age at PID diagnosis was similar across these 4 populations.
NS, not specified.
Overestimation of granulomas is possible because GLILD was classified as granulomas in our population.