Abstract
Objectives: In the present study, we have sought to establish the clinical and immunological characteristics of Japanese patients with interstitial lung disease (ILD). Methods: Serum samples from 35 patients of ILD were screened for autoantibodies using RNA and protein immunoprecipitation assays. Patients with or without serum antibodies to aminoacyl tRNA synthetases (ARS) were assessed clinically and compared. Results: Sera from 12 of 35 (34%) patients with ILD (mean age at onset = 49.7 yrs; range 27 - 65 yrs) were found to contain anti-ARS antibodies (anti-EJ: 3 patients; anti-OJ: 2 patients; anti-PL-12: 3 patients; anti-KS: 4 patients). Nine of the 12 (75%) were female. Six (50%) had Raynaud’s phenomenon, 5 (42%) had arthralgia/arthritis and four (33%) had rheumatoid factor. Lung biopsy specimens of 8 patients with anti-ARS antibodies were examined histologically in detail. The following was determined: Two patients had usual interstitial pneumonia; 3 had non-specific interstitial pneumonia; one had organizing pneumonia; one had lymphocyte interstitial pneumonia and the remaining patient had desquamative interstitial pneumonia. Age at disease onset was significantly lower and the frequency of Raynaud’s phenomenon was significantly greater in these patients compared to anti-ARS-negative patients (49.7 yrs vs. 62.6 yrs, p = 0.004; 50% vs. 4%, p = 0.003, respectively). Conclusions: These results indicate that the presence of anti-ARS autoantibodies correlates with ILD without definite diagnosis of connective tissue diseases as well as polymyositis/dermatomyositis (PM/DM) with ILD in Japanese patients.
Highlights
In patients with idiopathic inflammatory myopathies, several autoantibodies to the 8 aminoacyl-tRNA synthetases histidyl, threonyl, alanyl, glycyl, isoleucyl, asparaginyl, tyrosyl and phenylalanyl tRNA synthetases have been identified [1]-[11]
These results indicate that the presence of anti-aminoacyl tRNA synthetases (ARS) autoantibodies correlates with interstitial lung disease (ILD) without definite diagnosis of connective tissue diseases as well as polymyositis/dermatomyositis (PM/DM) with ILD in Japanese patients
It has been known for some time that anti-Jo-1 antibodies are more common in patients with typical PM/DM, whereas anti-PL-12 and anti-KS antibodies are found in patients with ILD without any signs of myositis
Summary
In patients with idiopathic inflammatory myopathies, several autoantibodies to the 8 aminoacyl-tRNA synthetases (anti-ARS antibodies) histidyl (anti-Jo-1), threonyl (anti-PL-7), alanyl (anti-PL-12), glycyl (anti-EJ), isoleucyl (anti-OJ), asparaginyl (anti-KS), tyrosyl (anti-Ha) and phenylalanyl (anti-Zo) tRNA synthetases have been identified [1]-[11]. Patients with different anti-ARS antibodies manifest certain common clinical symptoms (fever, Raynaud’s phenomenon, arthritis and chronic interstitial lung disease (ILD)) together designated anti-synthetase syndrome [2]. Further observation and analysis suggests that each of the anti-ARS antibodies is to some degree associated with different clinical manifestations [2] [8] [12] [13]. It has been known for some time that anti-Jo-1 antibodies are more common in patients with typical PM/DM, whereas anti-PL-12 and anti-KS antibodies are found in patients with ILD without any signs of myositis. Anti-ARS antibodies have been reported in patients with idiopathic interstitial pneumonias (IIPs) [13]-[18]
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More From: Open Journal of Rheumatology and Autoimmune Diseases
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