Circulation, Vol 85, 249-258, Copyright © 1992 by American Heart Association
RJ Barst, ER Flaster, A Menon, M Fotino and JH Morse
BACKGROUND. A link between primary pulmonary hypertension (PPH) and
autoimmune disorders has been postulated. To investigate this relation, we
performed immunofluorescent antinuclear antibody tests (ANA) and
serological human leukocyte antigen (HLA)-A, B, C, DR, and DQ typing on two
groups of Caucasian children with unexplained pulmonary hypertension (PHT)
and their parents. METHODS AND RESULTS. Group 1 consisted of 17 children
with PPH including two patients with familial PPH and three patients with
trivial congenital pulmonary to systemic communications. Group 2 consisted
of 13 children with advanced PHT and anatomically large congenital
pulmonary to systemic communications (PHT + shunt). Both groups had
comparably severe pulmonary vascular disease documented by cardiac
catheterization. The following statistically significant data (p less than
0.05) were obtained when the study groups were compared with those
published for normal controls. Although positive ANAs and varying titers of
autoantibodies were found in both groups of children and mothers (not
fathers), +ANAs were only significant for the maternal groups. The PPH
(group 1) children had increased frequencies of HLA-DR3, DRw52, and DQw2
and decreased DR5, whereas the group 2 (PHT + shunt) children (also their
parents) had no statistically significant alterations in any of the DR or
DQ alleles. The PPH mothers had decreased DQw3, an allele in linkage
disequilibrium with DR5. CONCLUSIONS. These immunogenetic data suggest that
childhood PPH appears to be associated with the major histocompatibility
complex alleles HLA-DR3, DRw52, and DQw2. This newly found correlation of
juvenile PPH with these alleles adds this disease to the DR3+ group of
autoimmune diseases. Further studies are needed to determine whether there
is also an immunological or autoimmune component in some children with PHT
+ shunt lesions because this group lacked an HLA association.
ARTICLES
Evidence for the association of unexplained pulmonary hypertension in children with the major histocompatibility complex
Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY 10032.
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