Respiratory system evaluation of adult primary immunodeficiency patients: a tertiary care center experience
Saltuk Buğra Kaya
İnönü University School of Medicine Department of Chest Diseases, Division of Allergy and Clinical ImmunologyMalatya, Turkey
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, Mehmet Erdem Çakmak
Department of Allergy and Clinical Immunology, Başakşehir Çam ve Sakura City HospitalIstanbul, Turkey
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, Özge Can Bostan
Hacettepe University School of Medicine Department of Chest Diseases, Division of Allergy and Clinical ImmunologyAnkara, Turkey
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, Ebru Damadoğlu
Hacettepe University School of Medicine Department of Chest Diseases, Division of Allergy and Clinical ImmunologyAnkara, Turkey
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, Gül Karakaya
Hacettepe University School of Medicine Department of Chest Diseases, Division of Allergy and Clinical ImmunologyAnkara, Turkey
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, Ali Fuat Kalyoncu
Hacettepe University School of Medicine Department of Chest Diseases, Division of Allergy and Clinical ImmunologyAnkara, Turkey
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, Saliha Esenboğa
Hacettepe University School of Medicine Department of Pediatrics, Division of ImmunologyAnkara, Turkey
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, Deniz Çağdaş Ayvaz
Hacettepe University School of Medicine Department of Pediatrics, Division of ImmunologyAnkara, Turkey
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and İlhan Tezcan
Hacettepe University School of Medicine Department of Pediatrics, Division of ImmunologyAnkara, Turkey
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Feb 02, 2025
About this article
Article Category: Original Study
Published Online: Feb 02, 2025
Page range: 1 - 6
Received: Sep 24, 2024
Accepted: Dec 20, 2024
DOI: https://doi.org/10.2478/ahem-2024-0018
Keywords
© 2025 Saltuk Buğra Kaya et al., published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
Demographic and clinical characteristics of the patients
| CVID(N=89) | CGD(N=16) | SCID(N=42) | SIgAD(N=20) | XLA(N=19) | Total(N=186) | |
|---|---|---|---|---|---|---|
| Age (years) | 53 (28–70) | 24.81±11.11 | 49 (28–70) | 26.45± 8.76 | 25.53 ± 6.69 | 38 (19–70) |
| Age at diagnosis (years) | 44 (14–62) | 11.12 ± 13.63 | 14.26 ± 15.09 | 22.40 ±8.36 | 5.42 ± 3.39 | 30 (1–70) |
| Gender (male) N(%) | 45 (50.6) | 10 (62.5) | 29 (69) | 11 (55) | 19 (100) | 114 (61.3) |
| Smoking (yes) N(%) | 9 (10.1) | 1 (6.3) | 4 (9.5) | 6 (30) | 4 (21.1) | 24 (12.9) |
| Smoking (pack/year) | 17.89 ± 11.62 | 35 | 15.25 ± 20.12 | 6.33 ± 3.77 | 3.50 ± 1.29 | 12.88 ± 12.71 |
| Comorbid diseases | ||||||
| Chronic kidney disease N(%) | - | - | 3 (7.1) | - | - | 3 (1.6) |
| Autoimmune disease N(%) | 24 (27) | 1 (6.3) | 8 (19) | 3 (15) | 3 (15.8) | 39 (21) |
| Hematological diseases N(%) | 6 (6.7) | - | 7 (16.7) | - | - | 13 (7) |
| Neurological diseases N(%) | 2 (2.2) | - | 3 (7.1) | - | 1 (5.3) | 6 (3.2) |
| Gastrointestinal system diseases N(%) | 7 (7.9) | - | 7 (16.7) | 1 (5) | 2 (10.5) | 17 (9.1) |
| Thyroid diseases N(%) | 1 (1.1) | - | 4 (9.5) | - | - | 5 (2.7) |
| Malignancy N(%) | 7 (7.9) | 1 (6.3) | 8 (19) | - | - | 16 (8.6) |
| Allergy (drug, food, bee, pollen etc.) N(%) | 8 (9) | 5 (31.3) | - | - | 3 (15.8) | 16 (8.6) |
| Treatment of immunodeficiency | ||||||
| IVIg or SCIg N(%) | 76 (85.4) | 1 (6.3) | 25 (59.5) | - | 19 (100) | 121 (65.1) |
| Prophylactic antibiotic N(%) | 23 (25.8) | 16 (100) | 16 (38.1) | 4 (20) | 4 (21.1) | 63 (33.9) |
| Prophylactic antifungal N (%) | 1 (1.1) | 16 (100) | 4 (9.5) | - | - | 21 (11.3) |
| Prophylactic antiviral N(%) | - | - | 4 (9.5) | - | - | 4 (2.2) |
| Systemic steroid N(%) | 5 (5.6) | - | 4 (9.5) | - | 1 (5.3) | 10 (5.4) |
| Immuno-suppressive therapy N(%) | 8 (9) | 4 (25) | 9 (21.4) | - | 1 (5.3) | 22 (11.8) |
| GM-CSF N(%) | - | - | 5 (11.9) | - | - | 5 (2.7) |
| SCT N(%) | 1 (1.1) | 2 (12.5) | 10 (23.8) | - | - | 13 (7) |
Lung function test results of the patients
| FEV1(%) | 78.45 ± 23.15 | 48.12 ± 21.18 | 68.37 ± 17.45 | 86.33 ± 14.32 | 81.50 ± 25.89 | 74.76 ± 23.23 | 0.002 |
| FVC(%) | 84.20 ± 18.94 | 56.88 ± 17.91 | 76.32 ± 15.69 | 87.89 ± 13.04 | 85.70 ± 20.71 | 80.64 ± 19.40 | 0.002 |
| FEV1/FVC (%) | 78.55 ± 15.26 | 71.88 ± 16.47 | 72.32 ± 11.41 | 81.89 ± 8.11 | 77.80 ± 13.66 | 76.89 ± 13.99 | 0.305 |
| Smoking (pack/year ) | 17.89 ± 11.62 | 35 | 15.25 ± 20.12 | 6.33 ± 3.77 | 3.50 ± 1.29 | 12.88 ± 12.71 | 0.062 |
Accompanying lung diseases of the patients
| Comorbid lung disease | CVID (N=89) | CGD (N=16) | SCID (N=42) | SIgAD (N=20) | XLA (N=19) | Total (N=186) | P |
|---|---|---|---|---|---|---|---|
| Asthma N(%) | 24 (27) | 1 (6.3) | 7 (16.7) | 5 (25) | 4 (21.1) | 41 (22) | 0.355 |
| COPD N(%) | 1 (1.1) | 2 (12.5) | 3 (7.1) | - | - | 6 (3.2) | 0.06 |
| Bronchiectasis N(%) | 30 (33.7) | 8 (50) | 16 (38.1) | 5 (25) | 10 (52.6) | 69 (37.1) | 0.307 |
| Tuberculosis history N (%) | 5 (5.6) | 7 (43.8) | 6 (14.3) | - | - | 18 (9.7) | 0.000 |
| Respiratory failure |
1 (1.1) | - | 1 (2.4) | - | - | 2 (1.1) | 0.866 |
| PTE N(%) | - | 1 (6.3) | - | - | - | 1 (0.5) | 1.000 |
| Lung cancer N(%) | 1 (1.1) | 1 (6.3) | - | - | - | 2 (1.1) | 0.297 |
| GLILD N(%) | 7 (7.9) | - | - | - | - | 7 (3.8) | 0.005 |
| BOOP N(%) | 4 (4.5) | - | 1 (2.4) | - | - | 5 (2.7) | 0.619 |
Radiological findings of the patients
| Thoracic radiological findings N(%) | CVID (N=89) | CGD (N=16) | SCID (N=42) | SIgAD (N=20) | XLA (N=19) | Total (N=186) | P |
|---|---|---|---|---|---|---|---|
| Atelectasis | 16 (18) | 6 (37.5) | 11 (26.2) | 1 (5) | 5 (26.3) | 39 (21) | 0.125 |
| Bronchiectasis | 30 (33.7) | 8 (50) | 16 (38.1) | 5 (25) | 10 (52.6) | 69 (37.1) | 0.307 |
| Consolidation | 12 (13.5) | 9 (56.3) | 11 (26.2) | 1 (5) | 5 (26.3) | 38 (20.4) | 0.001 |
| Ground glass opacity | 25 (28.1) | 9 (56.3) | 15 (35.7) | 3 (15) | 6 (31.6) | 58 (31.2) | 0.094 |
| Lymphadenopathy (> 1 centimeter) | 19 (21.3) | 8 (50) | 14 (33.3) | 1 (5) | 4 (21.1) | 46 (24.7) | 0.017 |
| Tracheal deviation | 3 (3.4) | 3 (18.8) | 7 (16.7) | - | 5 (26.3) | 18 (9.7) | 0.003 |
| Pleural thickening | 10 (11.2) | 6 (37.5) | 8 (19) | - | 4 (21.1) | 28 (15.1) | 0.018 |
| Pleural effusion | 9 (10.1) | 4 (25) | 5 (11.9) | - | 2 (10.5) | 20 (10.8) | 0.207 |
| Thickening of the bronchial wall | 21 (23.6) | 4 (25) | 10 (23.8) | 2 (10) | 6 (31.6) | 43 (23.1) | 0.559 |
| Enlargement of the bronchial wall | 15 (16.9) | 3 (18.8) | 9 (21.4) | 2 (10) | 4 (21.1) | 33 (17.7) | 0.842 |
| Parenchymal nodule (< 3cm) | 26 (29.2) | 9 (56.3) | 17 (40.5) | 2 (10) | 7 (36.8) | 61 (32.8) | 0.033 |
| Parenchymal mass (> 3 cm) | 2 (2.2) | 1 (6.3) | 3 (7.1) | - | - | 6 (3.2) | 0.392 |
| Emphysema | 1 (1.1) | 4 (25) | 7 (16.7) | - | - | 12 (6.5) | 0.000 |
| Thymic enlargement | 1 (1.1) | - | - | - | - | 1 (0.5) | 0.478 |
| Cavitation or air cyst | 7 (7.9) | 5 (31.3) | 13 (31) | - | 4 (21.1) | 29 (15.6) | 0.001 |
| Reticular opacities | 19 (21.3) | 8 (50) | 11 (26.1) | 1 (5) | 5 (26.3) | 44 (23.7) | 0.033 |
| Centrolobular opacity | 19 (21.3) | 6 (37.5) | 10 (23.8) | - | 3 (15.8) | 38 (20.4) | 0.072 |
| Fibrotic changes | 21 (23.6) | 8 (50) | 10 (23.8) | - | 6 (31.6) | 45 (24.8) | 0.012 |
| Pulmonary artery enlargement | 3 (3.4) | 2 (12.5) | 7 (16.7) | - | 3 (15.8) | 15 (8.1) | 0.032 |
| Mosaic perfusion or air trapping | 10 (11.2) | 7 (43.8) | 10 (23.8) | - | 5 (26.3) | 32 (17.2) | 0.002 |