Evidence for the association of unexplained pulmonary hypertension in children with the major histocompatibility complex
| Autor: | A Menon, Jane H. Morse, Robyn J. Barst, M. Fotino, E R Flaster |
|---|---|
| Rok vydání: | 1992 |
| Předmět: |
Adult
Male Pulmonary Circulation medicine.medical_specialty Anti-nuclear antibody Heart disease Hypertension Pulmonary medicine.medical_treatment Autoimmunity Human leukocyte antigen Gastroenterology Antigen-Antibody Reactions Major Histocompatibility Complex HLA Antigens Physiology (medical) Internal medicine Immunogenetics medicine Humans Vascular Diseases Child Autoantibodies Cardiac catheterization Autoimmune disease Vascular disease business.industry Hemodynamics Autoantibody medicine.disease Pulmonary hypertension Antibodies Antinuclear Child Preschool Immunology Female Cardiology and Cardiovascular Medicine business |
| Zdroj: | Circulation. 85:249-258 |
| ISSN: | 1524-4539 0009-7322 |
| DOI: | 10.1161/01.cir.85.1.249 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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