Common and distinct clinical features in adult patients with anti-aminoacyl-tRNA synthetase antibodies: heterogeneity within the syndrome

• Published in: PloS one Vol. 8; no. 4; p. e60442
• Main Authors: Hamaguchi, Yasuhito, Fujimoto, Manabu, Matsushita, Takashi, Kaji, Kenzo, Komura, Kazuhiro, Hasegawa, Minoru, Kodera, Masanari, Muroi, Eiji, Fujikawa, Keita, Seishima, Mariko, Yamada, Hidehiro, Yamada, Ryo, Sato, Shinichi, Takehara, Kazuhiko, Kuwana, Masataka
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 03.04.2013; Public Library of Science (PLoS)
• Subjects: Adolescent; Adrenal Cortex Hormones - therapeutic use; Adult; Aged; Amino Acyl-tRNA Synthetases - immunology; Aminoacyl-tRNA ligase; Analysis; Antibodies; Arthritis; Autoantibodies - blood; Biology; Dermatology; Dermatomyositis; Dermatomyositis - blood; Dermatomyositis - drug therapy; Dermatomyositis - epidemiology; Dermatomyositis - immunology; Exanthema; Female; Health care facilities; Hospitals; Humans; Immunoglobulins; Immunoprecipitation; Inflammatory diseases; Internal medicine; Lung diseases; Lung diseases, Interstitial; Lung Diseases, Interstitial - blood; Lung Diseases, Interstitial - epidemiology; Lung Diseases, Interstitial - immunology; Lupus Erythematosus, Systemic - blood; Lupus Erythematosus, Systemic - epidemiology; Lupus Erythematosus, Systemic - immunology; Male; Medical diagnosis; Medicine; Middle Aged; Musculoskeletal diseases; Myositis; Pathogenesis; Patients; Polymyositis; Polymyositis - blood; Polymyositis - drug therapy; Polymyositis - epidemiology; Polymyositis - immunology; Prevalence; Rheumatology; Science; Scleroderma, Systemic - blood; Scleroderma, Systemic - epidemiology; Scleroderma, Systemic - immunology; Skin; Syndrome; Transfer RNA; tRNA; University graduates; Viral antibodies; Young Adult
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0060442

To identify similarities and differences in the clinical features of adult Japanese patients with individual anti-aminoacyl-tRNA synthetase antibodies (anti-ARS Abs). This was a retrospective analysis of 166 adult Japanese patients with anti-ARS Abs detected by immunoprecipitation assays. These patients had visited Kanazawa University Hospital or collaborating medical centers from 2003 to 2009. Anti-ARS Ab specificity included anti-Jo-1 (36%), anti-EJ (23%), anti-PL-7 (18%), anti-PL-12 (11%), anti-KS (8%), and anti-OJ (5%). These anti-ARS Abs were mutually exclusive, except for one serum Ab that had both anti-PL-7 and PL-12 reactivity. Myositis was closely associated with anti-Jo-1, anti-EJ, and anti-PL-7, while interstitial lung disease (ILD) was correlated with all 6 anti-ARS Abs. Dermatomyositis (DM)-specific skin manifestations (heliotrope rash and Gottron's sign) were frequently observed in patients with anti-Jo-1, anti-EJ, anti-PL-7, and anti-PL-12. Therefore, most clinical diagnoses were polymyositis or DM for anti-Jo-1, anti-EJ, and anti-PL-7; clinically amyopathic DM or ILD for anti-PL-12; and ILD for anti-KS and anti-OJ. Patients with anti-Jo-1, anti-EJ, and anti-PL-7 developed myositis later if they had ILD alone at the time of disease onset, and most patients with anti-ARS Abs eventually developed ILD if they did not have ILD at disease onset. Patients with anti-ARS Abs are relatively homogeneous. However, the distribution and timing of myositis, ILD, and rashes differ among patients with individual anti-ARS Abs. Thus, identification of individual anti-ARS Abs is beneficial to define this rather homogeneous subset and to predict clinical outcomes within the "anti-synthetase syndrome."