Clinical manifestations of skin, lung and muscle diseases in dermatomyositis positive for anti-aminoacyl tRNA synthetase antibodies.

Patients with dermatomyositis positive for anti-aminoacyl tRNA synthetase (ARS) antibodies, also known as antisynthetase syndrome (ASS), frequently present with mechanic's hand and interstitial lung disease (ILD). We first screened the antibody profiles of 59 patients with dermatomyositis, and then examined the cutaneous, muscular and pulmonary manifestations characteristic for patients with ASS. The anti-ARS antibodies Jo-1, PL-7, PL-12, EJ and KS, along with antibodies to TIF1-γ, MDA5 and Mi-2, were examined. Among the 59 patients, 20, 21, 15 and three patients were classified into the ASS, non-ASS, myositis-specific antibody-negative and unknown groups, respectively. Five of 16 patients (31%) with ASS had six relatives with a history of collagen diseases, within the second degree of relationship, including two cases of dermatomyositis (vs the non-ASS group, P = 0.018). Patients with ASS more frequently presented with fever and arthralgia, and had elevated levels of C-reactive protein. Nine of the 11 finger lesions (82%) clinically diagnosed as mechanic's hands showed a psoriasiform tissue reaction. ILD was observed in 19 of 20 patients (95%) with ASS, and eight of 21 patients (38%) in the non-ASS group, in which six patients possessed anti-MDA5 antibody. Patients with ASS showed higher serum levels of muscle enzymes, and four of 12 patients (33%) had fasciitis-dominant myopathy, while only one of 11 patients (9%) in the non-ASS group had fasciitis-dominant myopathy. Patients with ASS often present with a psoriasiform tissue reaction in the hand lesions and fasciitis-dominant myopathy, and the relatives of those with ASS are at high risk for collagen diseases.

Fukamatsu H ，Hirai Y ，Miyake T ，Kaji T ，Morizane S ，Yokoyama E ，Hamada T ，Oono T ，Koyama Y ，Norikane S ，Iwatsuki K ... -  《-》

Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti-aminoacyl-tRNA synthetase antibodies: A single center experience.

To identify the differences of clinical characteristics, laboratory findings, and the long-term outcomes in patients with anti-synthetase syndrome (ASS) of different anti-aminoacyl-transfer RNA synthetase antibodies. We retrospectively enrolled 119 patients with ASS, and the clinical characteristics and laboratory findings were collected. Additionally, multivariate COX regression analysis was performed to estimate the risk factors of prognosis in patients with ASS. The frequency of interstitial lung disease (ILD) reached 93.3% in our cohort, of 28 (23.5%) was classified as rapidly progressive (RP)-ILD. The highest incidence of RP-ILD was 36.4% in the PL12 group of ASS patients. The ILD group was characterized by an older age, a lower prevalence of V sign, and a higher prevalence of pulmonary symptoms when contrasted with the non-ILD group. There were statistical differences of clinical significance in arthritis, myositis, mechanic's hands, triad, shawl sign, V sign, and Raynaud's phenomenon among the four subgroups (all p < .05). Additionally, the prevalence rates of arthritis, myositis, mechanic's hands, triad, and V sign in the anti-Jo1 antibody-positive group were significantly higher than anti-Jo1 antibody-negative patients with ASS (all p < .05). Multivariate Cox regression analysis showed mechanic's hands (odds ratio [OR] = 6.47, p < .001), anti-nuclear antibodies (ANA) (OR = 2.13, p = .026), ILD (OR = 10.50, p < .001), and V sign (OR = 0.30, p = .007) were independent factors affecting the prognosis of patients with ASS. The incidences of RP-ILD, arthritis, myositis, triad, mechanic's hands, and shawl sign were more frequent in the anti-Ro52 antibody-positive group than the anti-Ro52 antibody-negative patients with ASS (all p < .05). Patients with ASS accompanied with ILD are highly prevalent. Mechanic's hands, ANA, and ILD may be a potential biomarker for predicting a poor prognosis in patients with ASS. Additionally, the detection of the anti-Ro52 antibody provides valuable insights for managing and predicting disease progression and long-term outcomes.

Zhang D ，Wang H ，Zhou X ，Yang J ，Liu Y ，Wang W ，Jiang P ，Fan B ... -  《-》

Clinical characteristics of interstitial lung diseases positive to different anti-synthetase antibodies.

To analyze the clinical, serological, and imaging characteristics of patients with interstitial lung diseases (ILD) positive to different anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies.The clinical data, serological indexes, pulmonary high-resolution computed tomography (HRCT) imaging features and pulmonary functions, and bronchoalveolar lavage fluid of 84 ILD patients with anti-ARS antibody positive in Beijing Chao-yang Hospital, Capital Medical University were reviewed.(1) Anti-ARS antibodies included anti-Jo-1 (42.86%), anti-PL-7 (26.19%), anti-PL-12 (10.71%), anti-EJ (14.29%), and anti-OJ (5.95%). (2) Nonspecific interstitial pneumonia was the main type of patients with ILD positive to antibodies of anti-Jo-1, anti-PL-7, and anti-EJ, organizing pneumonia was the main type of patients with ILD positive to anti-PL-12 antibody and usual interstitial pneumonia was the main type of patients with ILD positive to anti-OJ antibody. (3) Only 14.29% of the patients had typical "triad syndrome" (interstitial pneumonia, myositis, and non-erosive arthritis). Myositis mainly occurred in patients with ILD positive to antibodies of anti-PL-7, anti-Jo-1, and anti-EJ. The incidence of arthritis in ILD patients with anti-Jo-1 was higher than that in ILD patients with anti-PL-12 and anti-EJ (P < .05). The incidence of mechanic's hand in ILD patients with anti-Jo-1 was higher than that in ILD patients with anti-PL-12 (P < .05).ILD positive to anti-Jo-1 antibody is associated with multiple organ involvement, mainly manifested as myositis, mechanic's hand, and arthritis. As other clinical manifestations of some ILD patients are relatively hidden, ILD patients should pay attention to the screening of the anti-ARS antibodies and guard against anti-synthetase syndrome.

Jiang M ，Dong X ，Zheng Y 《-》

[Idiopathic Inflammatory Myopathy and Anti-aminoacyl-tRNA Synthetase Antibody].

Maeda MH 《-》

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

Hamaguchi Y ，Fujimoto M ，Matsushita T ，Kaji K ，Komura K ，Hasegawa M ，Kodera M ，Muroi E ，Fujikawa K ，Seishima M ，Yamada H ，Yamada R ，Sato S ，Takehara K ，Kuwana M ... -  《-》