A CHILD WITH CHRONIC LUNG DISEASE A CASE REPORT OF COMMON VARIABLE IMMUNODEFICIENCY
Wella Yurisa,Nia Kurniati,Dina Muktiarti
Department of Child Health Faculty of Medicine Universitas Indonesia Cipto Mangunkusumo National General Hospital Jakarta Indonesia
Background : Common variable immunodeficiency (CVID) is a heterogeneous disorder involving immune dysfunction of B and T cells and dendritic cells. The characteristic immunologic defect is the inability of B cells to differentiate into plasma cells capable of secreting all immunoglobulin types. While CVID is often regarded as a disease of adults, it does affect children, and diagnosis can be challenging.
Case Presentation Summary : A 7-year-old boy was referred to Cipto Mangunkusumo Hospital with suspected of primary immunodeficiency. Patient began to have chronic diarrhea since 2 months old and hospitalized several times. Patient started to have recurrent respiratory tract infection since 10 months old. He has been hospitalized several times due to pneumonia. His chronic cough considered as tuberculosis and treated with anti-tuberculosis therapy in 3 different times but the condition didn’t improve. There was no complaint about oral thrush. His growth was faltering. He also suffered from bronchiectasis as a complication of the chronic respiratory tract infection. Diagnostic work up showed reduction of IgG (19 mg/dl; normal range 633-1280 mg/dl), IgA (<23,3 mg/dl; normal range 33-202 mg/dl) and IgM (<18,6 mg/dl; normal range 48-207 mg/dl) with normal B cell count (402 cell/uL, 6%). Anti-HIV screening was non-reactive. He then diagnosed as CVID and treated with intravenous immunoglobulin therapy every month 400-600 mg/kg/dose. This dose adjusted depend on his trough serum immunoglobulin level (target more than 600 mg/dL).
Learning Points/Discussion : Respiratory tract infections are the most common presentation of antibody associated primary immunodeficiency. Delayed diagnosis will lead to chronic lung disease. The clinician should be aware of this presentation to prevent the severe complication. The diagnosis of CVID is based upon the findings of hypo-gammaglobulin, defective antibody formation and exclusion of other causes of antibody deficiency. The management of CVID includes immunoglobulin replacement therapy, prevention of infection and monitoring of pulmonary status.