ArticleClinical rheumatology2026
Distinct phenotypes and prognosis between Jo-1 and non-Jo-1 subtypes in anti-synthetase syndrome: a retrospective cohort study from China.
Article in Clinical rheumatology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
objectiveAnti-synthetase syndrome (ASyS) is characterized by anti-aminoacyl-tRNA synthetase antibodies and heterogeneous multi-organ involvement. This study compared clinical phenotypes and outcomes between Jo-1-positive and non-Jo-1 ASyS patients in a Chinese cohort.
methodsWe retrospectively analyzed 184 ASyS patients treated between June 2017 and December 2020 at a tertiary academic center. Data included clinical records, laboratory results, CT imaging, and pulmonary function tests.
resultsAmong 184 patients, anti-aminoacyl-tRNA synthetase antibodies included Jo-1 (n = 76, 41.3%), EJ (n = 45, 24.5%), PL-7 (n = 35, 19.0%), PL-12 (n = 26, 14.1%), and OJ (n = 2, 1.1%). ILD prevalence was 94.0% (173/184), with 14 (7.6%) classified as RP-ILD. Malignancy was documented in 10 (5.4%) patients. Anti-Jo-1 patients showed significantly higher frequencies of myalgia (P < 0.001) and muscle weakness (P = 0.001), while non-Jo-1 patients exhibited more Gottron's sign (P = 0.005), malignancy (P = 0.039), RP-ILD (P = 0.034), and concurrent connective tissue diseases (P = 0.014). Laboratory analysis revealed higher CK, LDH, AST, ALT, and CD3 + CD8 + T-cell counts in Jo-1 patients (all P < 0.01), alongside lower complement C4 and ESR (P = 0.005 and P = 0.003). Survival analysis demonstrated a significantly better prognosis in Jo-1 patients (P = 0.002), with anti-PL-7 subgroup having the poorest outcome (P = 0.005). Multivariate logistic analysis identified RP-ILD and elevated serum ferritin as independent predictors of mortality.
conclusionJo-1 and non-Jo-1 ASyS demonstrate distinct clinical phenotypes. Non-Jo-1 antibodies are associated with higher risks of RP-ILD and malignancy, indicating a more severe disease course and worse prognosis. Key Points • Anti-Jo-1 syndrome and non-Jo-1 anti-synthetase syndrome represent distinct clinical phenotypes with significant differences in organ involvement. • Non-Jo-1 antibody positivity is associated with a more severe disease profile, including higher risks of rapidly progressive interstitial lung disease and malignancy. • The anti-PL-7 subtype identifies a patient subgroup with the poorest long-term prognosis. • Rapidly progressive ILD and elevated serum ferritin are independent prognostic factors for mortality in anti-synthetase syndrome.
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