The aim is to study the immune response to conjugated pneumococcal vaccination (Prevenar13) in Immunoglobulin G-deficient patients and healthy controls. Our hypothesis is that the antibody response will be impaired in patients as compared with controls. In contrast, we postulate that the cellular immune response will be intact.

Ten patients with Immunoglobulin G1 and/or Immunoglobulin G2 deficiency will be enrolled together with 10 age and sex- matched controls. Blood-samples will be drawn at base-line and after 1, 2 and 4 weeks post-vaccination. Serum will be analyzed for opsonophagocytic antibodies. Peripheral blood monocytic cells (PBMCs) will be isolated, frozen and later analyzed for all time-points simultaneously. PBMCs will be stimulated with vaccine-antigens for 3-5 days and cellular activation markers will be measured together with cytokines (Interferon-gamma, Interleukin (IL)-5, IL-13, IL-10, IL-17, IL-22). In addition, levels of antimicrobial peptides in nasal fluid will be measured at baseline and after 4 weeks post-vaccination.

Inclusion Criteria:

• Immunoglobulin gamma-deficiency: Immunoglobulin gamma 1-deficiency < 2,8 g/L and/or Immunoglobulin gamma 2-deficiency < 1,15 g/L
• Age 18-65

Exclusion Criteria:

• Immunoglobulin gamma-substitution for 6 months prior to study inclusion
• Prevenar7 or Prevenar13 given prior to study inclusion.
• Pneumovax given during a 2 year period prior to study inclusion
• Pregnancy or planning to become pregnant during the study period (4 weeks)
• Taking part in another clinical study involving drugs or vaccinations during the study period (4 weeks).
• Taking systemic steroids, Non steroidal anti inflammatory drugs (NSAID) or immunosuppressive drugs.
• Being allergic to any substance in the vaccine.
• Acute and ongoing disease with high fever.
• Thrombocytopenia (< 100x109/L).
• Coagulopathy
• Breastfeeding a baby
• The healthy controls should not have any disease with effects on the immune-system