A study of the relationship of alpha1-antitrypsin phenotype to the occurrence and severity of juvenile rheumatoid arthritis

✍ Scribed by William E. Crowe; George Hug; Gail Chuck; David S. Knapp; Joseph E. Levinson

• Publisher: John Wiley and Sons
• Year: 1982
• Tongue: English
• Weight: 244 KB
• Volume: 25
• Category: Article
• ISSN: 0004-3591
• DOI: 10.1002/art.1780250816

✦ Synopsis

Alpha,-antitrypsin (a-AT) is a protease inhibitor system consisting of more than 30 phenotypic alleles, which can be differentiated by polyacrylamide gel-isoelectric focusing. The most common alleles are designated PiM, Pis, and PiZ. The phenotype MM is found in about 90% of the U.S. white population, whereas MS and MZ occur in 8 and 3%, respectively. The only well-documented disease association with a- AT phenotype is the Z homozygote (phenotype ZZ) with hepatic cirrhosis and pulmonary emphysema (1).

Since proteolytic enzymes seem to be involved in the breakdown of cartilage in the various forms of chronic arthritis, defects in the Pi system of protease inhibitors might contribute to the occurrence and severity of these diseases. Arnaud et a1 (2) tested this hypothesis in 96 white English children with juvenile chronic polyarthritis and found a statistically significant increase of a-AT phenotype MZ in these patients. No correlation existed between phenotype and clinical