Hereditary C2 deficiency in Sweden: frequent occurrence of invasive infection, atherosclerosis, and rheumatic disease

G Jönsson, L Truedsson, G Sturfelt, VA Oxelius… - Medicine, 2005 - journals.lww.com
Although frequently asymptomatic, homozygous C2 deficiency (C2D) is known to be
associated with severe infections and rheumatic disease. We describe the clinical findings in
40 persons with C2D from 33 families identified in Sweden over 25 years. Medical records
covering 96% of the accumulated person-years were reviewed, giving a mean observation
time of 39 years (range, 1-77 yr). Severe infection was the predominant clinical
manifestation in the cohort: 23 patients had a past history of invasive infections, mainly …