Title:Antisynthetase Syndrome: A Review of Etiopathogenesis, Diagnosis and Management
Volume: 22
Issue: 16
Author(s): Aibek E. Mirrakhimov
Affiliation:
Keywords:
Antisynthetase antibodies, antisynthetase syndrome, autoimmunity, cancer, glucocorticoids, inflammatory myopathy,
interstitial lung disease, Raynaud phenomenon.
Abstract: Antisynthetase syndrome is a group of closely related rare diseases which clinically manifest
with inflammatory myopathies, interstitial lung disease, inflammatory arthritis, skin hyperkeratosis
(mechanic’s hands) and Raynaud phenomenon. The pathophysiology of antisynthetase syndrome is
not entirely understood, but genetic predisposition, viral infections and medication use may play a
role. Certain antisynthetase antibodies are associated with various clinical presentations and a lower
burden of inflammatory myopathies. Patients with antisynthetase syndrome have a worse prognosis
than patients with pure inflammatory myopathies mainly because of interstitial lung disease. Future research
should further investigate the pathogenesis of antisynthetase syndrome which could identify new therapeutic targets.
It will be also important to study whether patients with AS are at increased risk of cancer and whether certain antisynthetase
antibodies have any association with the risk of malignancy.
