Rheumatology 2000; 39: 1289-1290
© 2000 British Society for Rheumatology
Letters to the Editor |
Autoantibodies to neutrophilic proteases in a case of panniculitis by deficit of
1-antitrypsin
Department of Internal Medicine, University of Genoa, Italy
| The first 10% of the full text of this article appears below. |
SIR,
1-Antitrypsin is a serine protease inhibitor whose principal targets are neutrophil elastase, proteinase 3 and cathepsin G [1]. Several mutants of the gene encoding
1-antitrypsin have been identified; the non-functional Z variant is found in 12% of Caucasians of European descent [2]. ZZ homozygotes develop a disease leading to lung emphysema and/or liver cirrhosis [3]. Panniculitis is a rare manifestation of the disease [4]. The pathogenesis probably relates to the lack of inhibition of neutrophil proteases due to
1-antitrypsin