Objective. To ascertain the clinical and serological associations of anti-K
u antibody.
Methods. Twenty-seven patients over a 7 year period (1987-1996) had anti-Ku
antibody detected by counterimmunoelectrophoresis (CIEP). Nineteen patient
s were available for clinical review. Five patients were assessed by chart
review. Serum was taken at review for repeat antibody analysis. Patients we
re assigned to diagnostic groups based on the American College of Rheumatol
ogy criteria.
Results. There were 22 women and 5 men. The duration of symptoms ranged fro
m one year to 28 years, Nine patients fulfilled criteria for systemic lupus
erythematosus (SLE), 4 scleroderma, 3 rheumatoid arthritis (RA), one disco
id lupus, and 7 had an undifferentiated connective tissue disease. There wa
s a low incidence of renal (2/24) and central nervous system involvement (1
/24); 19/24 had Raynaud's phenomenon, 15/24 had inflammatory arthritis but
only one had erosions on radiograph; 11/24 reported esophageal reflux sympt
oms. Three of 24 patients had myositis. All patients had anti-nuclear antib
ody using indirect immunofluorescence of > 640 titer with a speckled and nu
cleolar pattern. Anti-Ku antibody was detected on CIEP in 15/19 sera availa
ble for repeat testing. Three patients had anti-Re antibody, 2 had anti-U1R
NP antibody, one patient had anti-topoisomerase-1 and anti-lie.
Conclusion. Anti-Ku antibody is found in a wide variety of connective tissu
e syndromes. While several patients fulfilled diagnostic criteria for SLE,
scleroderma, and RA, their clinical features were usually mild and did not
form a distinctive clinical pattern. Common features associated with anti-g
u were Raynaud's phenomenon, arthralgia, skin thickening, and esophageal re
flux. Few patients had associated autoantibody specificities found in SLE o
r scleroderma.