Table 1.
Demographic characteristics, features at CVID onset and diagnosis are detailed.
| Overall CVID cohort (N = 73) | Pediatric-onset (N = 44) | Adult-onset (N = 29) | |||||
|---|---|---|---|---|---|---|---|
| Demographic Characteristics | Mean | SD | Mean | SD | Mean | SD | p |
| Age at the latest follow-up | 38.4 | 16.59 | 42.9 | 13.45 | 47.4 | 12.89 | 0.213 |
| Age at onset | 17.3 | 13.84 | 10.3 | 6.68 | 30.8 | 11.29 | <0.001 |
| Age at diagnosis | 28.4 | 16.00 | 31.6 | 12.17 | 38.9 | 12.59 | 0.041 |
| Diagnostic delay | 11.5 | 11.71 | 21.7 | 13.76 | 8.2 | 7.76 | <0.001 |
| Follow up time | 10.0 | 8.17 | 11.2 | 9.45 | 8.6 | 7.67 | 0.279 |
| Time of disease | 21.5 | 13.86 | 33.0 | 15.38 | 16.8 | 10.23 | <0.001 |
| Characteristics at CVID onset | N | % | N | % | N | % | p |
| Immune Dysregulation | 31.0 | 42.47 | 16.0 | 36.36 | 15.0 | 51.72 | 0.231 |
| Infections | 65.0 | 89.04 | 38.0 | 86.36 | 27.0 | 93.10 | 0.465 |
| Malignancy | 0.0 | 0.00 | 0.0 | 0.00 | 0.0 | 0.00 | NA |
| Syndromic Features | 3.0 | 4.11 | 3.0 | 6.82 | 0.0 | 0.00 | 0.272 |
| Other Symptoms | 5.0 | 6.85 | 3.0 | 6.82 | 2.0 | 6.90 | 1 |
| No Symptoms | 2.0 | 2.74 | 2.0 | 4.55 | 0.0 | 0.00 | 0.514 |
| Hypogammaglobulinemia | 2.0 | 2.74 | 2.0 | 4.55 | 0.0 | 0.00 | 0.514 |
| Characteristics at CVID diagnosis | N | % | N | % | N | % | p |
| Recurrent respiratory infections | 65 | 89.0 | 42 | 95.5 | 24 | 82.8 | 0.252 |
| Failure to thrive (infancy) | 5 | 6.8 | 5 | 11.4 | 0 | 0.0 | 0.51 |
| Recurrent pyogenic infections | 6 | 8.2 | 2 | 4.5 | 4 | 13.8 | 0.207 |
| Unusual infections | 7 | 9.6 | 4 | 9.1 | 3 | 10.3 | ns |
| Same pathogen recurrent infections | 7 | 9.6 | 5 | 11.4 | 2 | 6.9 | 0.696 |
| Autoimmunity, autoinflammation, and lymphoid proliferation | 30 | 41.1 | 16 | 36.4 | 14 | 48.3 | 0.34 |
| Pneumonia | 44 | 60.3 | 26 | 59.1 | 18 | 62.1 | ns |
| Meningitis | 6 | 8.2 | 3 | 6.8 | 3 | 10.3 | 0.676 |
| Upper respiratory airways recurrent infections | 64 | 87.7 | 41 | 93.2 | 23 | 79.3 | 0.142 |
| Lower respiratory airways recurrent infections | 40 | 54.8 | 22 | 50.0 | 18 | 62.1 | 0.345 |
| Gastrointestinal recurrent infections | 25 | 34.2 | 16 | 36.4 | 9 | 31.0 | 0.802 |
| Urinary tract infections | 14 | 19.2 | 6 | 13.6 | 8 | 27.6 | 0.223 |
| Skin recurrent infections | 6 | 8.2 | 3 | 6.8 | 3 | 10.3 | 0.676 |
| Other recurrent infections | 4 | 5.5 | 2 | 4.5 | 2 | 6.9 | ns |
| Unusual viral infections | 11 | 15.1 | 7 | 15.9 | 4 | 13.8 | ns |
| Unusual opportunistic infections | 4 | 5.5 | 2 | 4.5 | 2 | 6.9 | ns |
| Unusual parasitic infections | 1 | 1.4 | 0 | 0.0 | 1 | 3.4 | 0.394 |
| Bronchiectasis | 31 | 43.1 | 18 | 41.9 | 13 | 44.8 | 0.813 |
| Enteropathy | 9 | 12.3 | 4 | 9.1 | 5 | 17.2 | 0.469 |
| Lymphocytic colitis | 9 | 12.3 | 4 | 9.1 | 5 | 17.2 | 0.469 |
| Hepatomegaly | 16 | 21.9 | 8 | 18.2 | 8 | 27.6 | 0.394 |
| Splenomegaly | 38 | 52.1 | 21 | 47.7 | 17 | 58.6 | 0.474 |
| Liver disease | 5 | 6.8 | 3 | 6.8 | 2 | 6.9 | ns |
| Pulmonary disease | 29 | 39.7 | 18 | 40.9 | 11 | 37.9 | ns |
| GLILD | 1 | 1.4 | 0 | 0.0 | 1 | 3.4 | 0.397 |
| Other organ diseases | 10 | 13.7 | 7 | 15.9 | 3 | 10.3 | 0.73 |
| Hypothyroidism | 3 | 4.1 | 2 | 4.5 | 1 | 3.4 | ns |
| Chronic diarrhea | 5 | 6.8 | 4 | 9.1 | 1 | 3.4 | 0.642 |
| Autoimmune cytopenia | 17 | 23.3 | 10 | 22.7 | 7 | 24.1 | ns |
| Autoimmune hemolytic anemia | 8 | 11.0 | 6 | 13.6 | 2 | 6.9 | 0.465 |
| Autoimmune neutropenia | 3 | 4.1 | 1 | 2.3 | 2 | 6.9 | 0.559 |
| Immune Thrombocytopenia | 17 | 23.3 | 10 | 22.7 | 7 | 24.1 | ns |
| Evans syndrome | 9 | 12.3 | 6 | 13.6 | 3 | 10.3 | ns |
| Other organ autoimmunity | 13 | 17.8 | 6 | 13.6 | 7 | 24.1 | 0.35 |
| Thyroiditis | 7 | 9.6 | 3 | 6.8 | 4 | 13.8 | 0.425 |
| Atrophic gastritis | 1 | 1.4 | 1 | 2.3 | 0 | 0.0 | ns |
| Vitiligo | 2 | 2.7 | 2 | 4.5 | 0 | 0.0 | 0.514 |
| Psoriasis | 3 | 4.1 | 1 | 2.3 | 2 | 6.9 | 0.559 |
| History of malignancy | 1 | 1.4 | 0 | 0.0 | 1 | 3.4 | 0.397 |
| Lymphoid Nodular Hyperplasia | 7 | 9.6 | 4 | 9.1 | 3 | 10.3 | ns |
| Granulomas | 3 | 4.1 | 1 | 2.3 | 2 | 6.9 | 0.559 |
| Celiac disease | 5 | 6.8 | 2 | 4.5 | 3 | 10.3 | 0.38 |
| Allergy | 16 | 22.2 | 12 | 27.9 | 4 | 13.8 | 0.248 |
| Persistent HCV viremia | 0 | 0.0 | 0 | 0.0 | 0 | 0.0 | na |
| Persistent HIV viremia | 0 | 0.0 | 0 | 0.0 | 0 | 0.0 | na |
| Persistent EBV viremia | 3 | 4.8 | 1 | 2.6 | 2 | 8.0 | 0.557 |
| Persistent CMV viremia | 1 | 1.6 | 1 | 2.8 | 0 | 0.0 | ns |
| Pathologic HRCT | 51 | 79.7 | 32 | 82.1 | 19 | 76.0 | 0.751 |
| Pathologic endoscopy | 23 | 63.9 | 14 | 77.8 | 9 | 50.0 | 0.164 |