ObjectiveTo identify similarities and differences in the clinical features of adult Japanese patients with individual anti-aminoacyl-tRNA synthetase antibodies (anti-ARS Abs).MethodsThis 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.ResultsAnti-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.ConclusionPatients 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.”

To clarify the association of clinical and prognostic features with dermatomyositis (DM)specific autoantibodies (Abs) in adult Japanese patients with DM.

Objective. To identify the 140-kd autoantigen recognized by anti-155/140 autoantibodies that are associated with adult cancer-associated dermatomyositis (DM) and juvenile DM and to determine the clinical relevance of anti-155/140 antibodies in a large cohort.Methods. Sera from 456 DM patients were assessed for the presence of anti-155/140 antibodies by immunoprecipitation using K562 cell extracts as substrate. Using immunoprecipitation and Western blotting, we then examined whether anti-155/140-positive sera recognized transcription intermediary factor 1␣ (TIF-1␣), TIF-1␤, and TIF-1␥. The clinical associations of antigen reactivity were also evaluated.Results. Anti-155/140-positive sera reacted with 140-kd TIF-1␣ in addition to 155-kd TIF-1␥. Among sera from 456 DM patients, 52 were reactive with both TIF-1␣ and TIF-1␥, while another 25 were reactive with TIF-1␥ alone. Additionally, 7 were reactive with TIF-1␤. Malignancy was more frequently found in adult patients with both anti-TIF-1␣ and anti-TIF-1␥ antibodies than in those with anti-TIF-1␥ antibodies alone (73% versus 50%; P < 0.05). In addition to juvenile DM patients and middle-aged and older DM patients with high percentages of malignancy, 8 "young adult" DM patients without malignancy had these autoantibodies.Conclusion. Anti-155/140 antibodies target TIF-1 family proteins, TIF-1␣ and TIF-1␤, in addition to TIF-1␥. Since TIF-1 proteins have significant roles in oncogenesis, these antibodies may be produced during misdirected antitumor immunity.Polymyositis (PM) and dermatomyositis (DM) are idiopathic inflammatory disorders that mainly affect the muscle and/or skin (1). Clinical manifestations of PM/DM are heterogeneous, with varying degrees of myositis, skin rash, and accompanying symptoms such as interstitial lung disease and internal malignancy. The association of malignancy with PM/DM, which is termed cancer-associated myositis, is well appreciated, particularly in patients with DM (2-6). Since malignant disease is one of the main causes of mortality in these patients, diagnosing occult cancer in them is important and challenging for clinicians.

Objective. To determine phenotypic and functional abnormalities of blood B cell subsets in patients with systemic sclerosis (SSc).Methods. Cell surface marker expression was determined by flow cytometry. Spontaneous apoptosis was evaluated by annexin V expression with flow cytometric analysis. IgG production by isolated IgD؊ memory B cells was examined by enzyme-linked immunosorbent assay.Results

This novel MSA is associated with cancer-associated DM and may serve as a diagnostic serological marker for this specific subset.