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 ... -  《Immunity Inflammation and Disease》

[Analysis of clinical features of ruccrent interstitial lung disease in patients with anti-EJ positive antisynthetase syndrome].

Zhu Y ，Wang L ，Lyu C ，Tan W ，Zhang M ... -  《-》

Clinical features of anti-SAE1 antibody-positive myositis and interstitial lung disease: a multicenter, retrospective study in Taiwan.

The clinical characteristics of patients positive for anti-small ubiquitin-like modifier 1-activating enzyme subunit 1 (SAE1) antibodies and diagnosed with idiopathic inflammatory myopathies (IIMs) vary across different cohorts and ethnicities, particularly concerning interstitial lung disease (ILD). We aimed to assess the clinical utility of the line immunoblot assay (LIA) in detecting anti-SAE1 autoantibodies and evaluate the clinical relevance and chronology of ILD development in relation to SAE1 autoantibody positivity among Taiwanese patients. We retrospectively conducted a population-based cohort analysis involving 6,496 patients who visited Chang Gung Memorial Health System across Taiwan from May 2018 to December 2021. Patients were assayed for myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) using the LIA method, and the antinuclear antibody (ANA) indirect immunofluorescence (IIF) method was used to evaluate ANA patterns. Of these, 70 SAE1-positive patients (1.08%) were included and followed up until December 2023. Associations with clinical characteristics and final diagnosis, particularly ILD, were assessed. Among the 70 SAE1-positive patients, 10 (14.3%) were strongly positive and 60 (85.7%) were weakly positive. In the strong positive group, 70% (7/10) were diagnosed with IIM, with most (5/7) showing a concordant ANA IIF pattern (speckled type). Six patients presented ILD either before (1/6) or after (5/6) IIM diagnosis; the majority (4/6) were classified as organizing pneumonia. The remaining 30.0% (3/10) had connective tissue disease (CTD) other than IIM without detectable ILD during follow-up, and none demonstrated a concordant ANA IIF pattern. In the weakly positive group, only 5.0% (3/60) had IIM and 3.3% (2/60) had ILD. The positive predictive value for strong positive SAE1 autoantibodies in diagnosing IIM was significantly higher than for weak positives (70.0% vs. 5.0%; p < 0.001). The study suggests that strong positive SAE1 autoantibodies detected via LIA are more closely associated with IIM compared to weak positive results. A high prevalence of ILD was observed among strong positive Taiwanese patients, indicating the need for prompt screening. Patients with weak positive or discordant ANA IIF results may represent false positives with a lower ILD risk.

Hsiao CY ，Tseng SC ，Hsu CY ，Chiu LC ，Su LJ ，Chan TM ... -  《-》

Clinical features and prognosis of anti-MDA5-positive dermatomyositis with coexistent anti-aminoacyl-tRNA synthetase antibodies.

This study aimed to evaluate the incidence and clinical significance of coexistence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibody in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5 + DM). We assessed a cohort of 246 consecutive patients with anti-MDA5 + DM. Clinical characteristics and survival rates were compared between patients with and without anti-ARS antibodies. Of these 246 patients, 15 (15/246, 6.1%) were positive for anti-ARS antibodies. The anti-ARS-positive group had a higher proportion of mechanic's hands (53.3% vs 25.5%, P = 0.019), and the positivity rates for antinuclear antibody (ANA) (80.0% vs 50.6%, P = 0.033) and anti-Ro52 antibodies (93.3% vs 62.3%, P = 0.013) were also higher. However, the CRP levels were comparatively low [4.0 (0.5, 7.8) vs 6.0 (3.1, 12.2), P = 0.019]. In the overall population, no difference in survival rates was observed between anti-MDA5 + DM patients with or without anti-ARS antibodies (log-rank P = 0.339). The multivariate Cox regression analysis revealed that elevated lactate dehydrogenase (LDH) levels and the presence of rapidly progressive interstitial lung disease (RPILD) were associated with poor prognosis [hazard ratios of 1.002 (95% CI 1.001, 1.002, P < 0.001) and 11.096 (95% CI 5.006, 24.598, P < 0.001), respectively]. Among the 246 patients with anti-MDA5 + DM, only 15 patients (6.1%) tested positive for anti-ARS antibodies. The presence of anti-ARS antibodies is associated with the occurrence of mechanic's hands, as well as an increased positive rate of ANA and anti-Ro52 antibodies. Elevated LDH levels and the presence of RPILD are poor prognostic risk factors for patients with anti-MDA5 + DM. Key Points • The presence of anti-ARS antibodies is associated with the occurrence of mechanic's hands, as well as an increased positive rate of ANA and anti-Ro52 antibodies. • Elevated LDH levels and the presence of RPILD are poor prognostic risk factors for patients with anti-MDA5 + DM. • Understanding the clinical characteristics of these patients will help clinicians to develop individualised treatment plans for each patient.

Wang G ，Yan D ，Weng C ，Xue L ，Liu Z ... -  《-》

Clinical heterogeneity and prognostic factors of anti-synthetase syndrome: a multi-centered retrospective cohort study.

Tang HS ，Tang IYK ，Ho RTC ，Young JKY ，Lai BTL ，Chung JYK ，Yung AKM ，Cheung CCL ，Lee PML ，So H ... -  《-》