CRP8 CASE SERIES OF PRIMARY IMMUNODEFICIENCY-ASSOCIATED BRONCHIECTASIS

Zhi Qi Teoh, Yi Cheau Chua, Nicholas Chang Lee Wen
Respiratory Unit, Paediatric Department, Hospital Pulau Pinang, Malaysia

Introduction
Primary immunodeficiency disorders (PIDs) are a heterogeneous group of inherited immune defects that predispose individuals to recurrent infections and chronic inflammation. One of the most significant long-term complications is bronchiectasis, resulting from repeated pulmonary infections. Early recognition and immunoglobulin replacement therapy are key to reducing morbidity.

Methodology
We reviewed eight patients aged 5–25 years with bronchiectasis secondary to PID. Composition of patients included X-linked agammaglobulinemia (n=2), common variable immunodeficiency (n=2), hypogammaglobulinemia (n=2, siblings), Hyper-IgE syndrome (n=1), and X-linked lymphoproliferative disease type 1 (XLP1) (n=1).

Results
The median age of symptom onset was 2 years, with diagnostic delays ranging from 2 months to 10 years. All patients presented with recurrent lower respiratory tract infections; several required PICU admission for respiratory support. One patient underwent multiple thoracic surgeries for complications including pneumatoceles and bronchopleural fistulae.

Radiological features of bronchiectasis were seen in most patients on high-resolution CT or CT thorax. Two patients were diagnosed early (within 1.5 years of symptom onset) and commenced on intravenous immunoglobulin (IVIG) promptly. These individuals had significantly milder disease, fewer infections, and fewer hospitalizations compared to those with delayed diagnosis.
All patients received regular IVIG (three-weekly or monthly), which led to a marked reduction in infection frequency and severity across the cohort.

Conclusion
This case series underscores the importance of early diagnosis and timely initiation of IVIG therapy in children with PID. Prompt treatment is associated with reduced infection burden and may help prevent the onset or progression of bronchiectasis.