HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Anti-PL-7 (anti-threonyl-tRNA synthetase) antisynthetase syndrome: clinical manifestations in a series of patients from a European multicenter study (EUMYONET) and review of the literature.

Abstract
Autoantibodies against several aminoacyl-transfer-RNA synthetases have been described in patients with myositis; anti-threonyl-tRNA synthetase (anti-PL-7) is one of the rarest. We describe the clinical and laboratory characteristics of a cohort of European anti-PL-7 patients, and compare them with previously reported cases. This multicenter study of patients positive for anti-PL-7, identified between 1984 and 2011, derives from the EUMYONET cohort. Clinical and serologic data were obtained by retrospective laboratory and medical record review, and statistical analyses were performed with chi-squared and Fisher exact tests. Eighteen patients, 15 women, were anti-PL-7 antibody positive. Median follow-up was 5.25 years (interquartile range, 2.8-10.7 yr), and 4 patients died. All patients had myositis (12 polymyositis, 5 dermatomyositis, and 1 amyopathic dermatomyositis), 10 (55.6%) had interstitial lung disease, and 9 (50%) had pericardial effusion. Occupational exposure to organic/inorganic particles was more frequent in patients with interstitial lung disease than in the remaining patients (5 of 10 vs. 1 of 7; p = 0.152), although the difference was not significant. Concurrent autoantibodies against Ro60 and Ro52 were seen in 8 of 14 (57%) patients studied. In the literature review the most common manifestations of anti-PL-7 antisynthetase syndrome were interstitial lung disease (77%), myositis (75%), and arthritis (56%). As in other subsets of the antisynthetase syndrome, myositis and interstitial lung disease are common features of the anti-PL-7 antisynthetase syndrome. In addition, we can add pericarditis as a possible manifestation related to anti-PL-7 antibodies.
AuthorsAne Labirua-Iturburu, Albert Selva-O'Callaghan, Melinda Vincze, Katalin Dankó, Jiri Vencovsky, Benjamin Fisher, Peter Charles, Maryam Dastmalchi, Ingrid E Lundberg
JournalMedicine (Medicine (Baltimore)) Vol. 91 Issue 4 Pg. 206-211 (Jul 2012) ISSN: 1536-5964 [Electronic] United States
PMID22732951 (Publication Type: Comparative Study, Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't, Review)
Chemical References
  • Antibodies, Antinuclear
  • Autoantibodies
  • Threonine-tRNA Ligase
Topics
  • Adult
  • Antibodies, Antinuclear (analysis, immunology)
  • Autoantibodies (analysis, immunology)
  • Cohort Studies
  • Combined Modality Therapy
  • Dermatomyositis (epidemiology, immunology)
  • Europe (epidemiology)
  • Female
  • Humans
  • Incidence
  • Lung Diseases, Interstitial (epidemiology, immunology)
  • Male
  • Middle Aged
  • Myositis (diagnosis, epidemiology, immunology, therapy)
  • Polymyositis (epidemiology, immunology)
  • Prognosis
  • Retrospective Studies
  • Risk Assessment
  • Severity of Illness Index
  • Survival Analysis
  • Threonine-tRNA Ligase (immunology, metabolism)
  • Treatment Failure
  • Treatment Outcome

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: