Immunodeficiency with predominantly antibody defects
Description
obsolete_sarcoidosis: ['An idiopathic inflammatory disorder characterized by the formation of non-necrotizing epithelioid granulomas which contain giant cells. It usually affects the lungs, lymph nodes, liver, and skin.', 'An idiopathic systemic inflammatory granulomatous disorder comprised of epithelioid and multinucleated giant cells with little necrosis. It usually invades the lungs with fibrosis and may also involve lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands.']
Data source
FinnGen phenocode | D3_IMMUDEFANTIBODY |
Hospital Discharge registry | ICD-10: D80 |
Cause of Death registry | ICD-10: D80 |
Include | D3_HEREDHYPOGAMMA, D3_NONHEREDHYPOGAMMA, D3_DEF_IIGA, D3_DEF_IGG, D3_DEF_IGM, D3_IMMUDEF_INCR_IGM, D3_AB_DEF_NORMHYPERIG, D3_TRANSHYPOGAMMAINFANT, D3_IMMUDEFANTIBODYNAS |
Level in the ICD-hierarchy | 3 |
First defined in version | DF2 |
Key figures
Sex | All | Female | Male |
Number of individuals | 208 | 144 | 64 |
Unadjusted prevalence (%) | 0.16 | 0.19 | 0.11 |
Mean age at first event (years) | 47.07 | 47.01 | 47.2 |
Median number of events / individual | 4 | 5 | 3 |
Clinical metrics
Sex | All | Female | Male |
Recurrence within 6 months (%) | 65.38 | 69.44 | 56.25 |
Case fatality at 5-years (%) | 4.81 | 4.17 | 6.25 |