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.
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.
From the Internal Medicine Department (AL-I, AS-O), Vall d'Hebrón General Hospital, Universitat Autònoma de Barcelona and Vall d'Hebrón Research Institute, Barcelona, Spain; 3rd Department of Internal Medicine (MV, KD), Division of Immunology, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary; Institute of Rheumatology (JV), Charles University, Prague, Czech Republic; Kennedy Institute of Rheumatology (BF, PC), Imperial College London, London, UK; Rheumatology Unit (MD, IEL), Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden.
Ane
A
Labirua-Iturburu
Albert
A
Selva-O'Callaghan
Melinda
M
Vincze
Katalin
K
Dankó
Jiri
J
Vencovsky
Benjamin
B
Fisher
Peter
P
Charles
Maryam
M
Dastmalchi
Ingrid E
IE
Lundberg
D000328:Adult; D000974:Antibodies, Antinuclear / Q000032:analysis / Q000276:immunology; D001323:Autoantibodies / Q000032:analysis / Q000276:immunology*; D015331:Cohort Studies; D003131:Combined Modality Therapy; D003882:Dermatomyositis / Q000453:epidemiology / Q000276:immunology; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D015994:Incidence; D017563:Lung Diseases, Interstitial / Q000453:epidemiology / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000175:diagnosis* / Q000453:epidemiology / Q000276:immunology* / Q000628:therapy; D017285:Polymyositis / Q000453:epidemiology / Q000276:immunology; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D016019:Survival Analysis; D013914:Threonine-tRNA Ligase / Q000276:immunology* / Q000378:metabolism; D017211:Treatment Failure; D016896:Treatment Outcome
D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D013914:Threonine-tRNA Ligase