High titers of autoantibodies in patients with sickle-cell disease.
OBJECTIVE
Frequency and titers of autoantibodies in patients with sickle-cell disease (SCD) have been reported as relatively high. In a prospective study of 88 patients, we examined this "hyper-autoreactivity" and its clinical consequences.


METHODS
For 1 year, patients with SCD were screened for the presence in their serum of antinuclear, anti-double-stranded DNA, antiextractible nuclear antigens, anticardiolipin antibodies, and rheumatoid factors. A population of 85 sex-matched individuals of similar ethnic origin served as controls.


RESULTS
Whereas prevalence of autoantibodies did not differ between the 2 groups, the type and rate of antinuclear antibodies were different. Autoantibodies from the SCD patients showed various immunofluorescence patterns, whereas only speckled patterns at low titers were present in controls. No antibody specificity was found in either group. SCD patients and controls displayed similar rates of anticardiolipin antibodies, but the SCD patients tended to be more frequently positive for rheumatoid factors. Six-year followup of the SCD patients did not provide any clinical evidence for onset of an autoimmune disease, except for 1 patient who developed rheumatoid arthritis, with increasing antinuclear antibodies followed by emergence of specific markers 5 years later.


CONCLUSIONS
Patients with SCD displayed high titers of autoantibodies. This observation may be due only to immune activation and/or dysfunction in SCD, as neither pathogenic specificity of autoantibodies nor autoimmune clinical signs appeared in the majority of cases in our study.
Department of Immunology, Laboratory of Hematology, Tenon Hospital, AP-HP, 4 rue de la Chine, 75020 Paris, France. cecile.toly-ndour@tnn.aphp.fr
Cécile
C
Toly-Ndour
Anne-Marie
AM
Rouquette
Stéphanie
S
Obadia
Pauline
P
M'bappe
François
F
Lionnet
Isabelle
I
Hagege
Faiza
F
Boussa-Khettab
Léon
L
Tshilolo
Robert
R
Girot
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood*; D001323:Autoantibodies / Q000097:blood*; D016009:Chi-Square Distribution; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies
D001323:Autoantibodies