Background Systemic lupus erythematosus (SLE) is a complex disease with heterogenous manifestations.1 Clinical subphenotypes have been reported, but limited studies have evaluated their long-term impact.2 This study utilized the data from the Asia Pacific Lupus Collaboration (APLC) cohort to evaluate subphenotypes in SLE and differences in long term outcome.3
Methods Patient data from the APLC cohort collected between 2013 and 2020 were included. Two-step cluster analysis was conducted based on serological features (anti-dsDNA, anti-Sm, anti-phospholipid antibodies, direct coomb’s test, and hypocomplementemia). Clinical characteristics, LLDAS-50 attainment, and risk of damage accrual were compared.
Results Three clusters with distinct clinical and serological characteristics were identified (table 1). Cluster one included 1051 patients (29.1%); all patients were positive for anti-dsDNA but negative for other autoantibodies. Patients in cluster one had earlier age of SLE diagnosis and more frequent renal involvement (55.5%). Cluster two included 1537 patients (42.5%); most patients were positive for anti-dsDNA (83.9%) and anti-phospholipid antibodies (57.3%), had more frequent haematological involvements (62.6%) and serositis (19.8%). Cluster three included 1026 patients (28.4%); anti-dsDNA was the only autoantibody found in 474 patients (46.2%) and mucocutaneous involvements were the predominant features (84.5%).
Over a median of 2.5 years, damage accrual occurred in 20.8% patients and the risk was highest in cluster one (cluster 1 vs cluster 2: OR 1.34, P=0.008; cluster 1 vs cluster 3: OR 1.28, P=0.041). LLDAS-50 was most frequently achieved in cluster three (cluster 1: 49.6%, cluster 2: 48.7%, cluster 3: 59.1%) (figure 1). LLDAS-50 was associated with reduced risk of damage accrual across three clusters (cluster 1: OR 0.71, P=0.032; cluster 2: OR 0.63, P<0.001; cluster 3: OR 0.58, P<0.001).
Conclusions Three distinct subphenotypes were confirmed and associated with different risks of damage accrual. LLDAS-50 was an attainable target and associated with reduced risk of damage accrual across three clusters.
Kaul A, Gordon C, Crow MK, Touma Z, Urowitz MB, van Vollenhoven R, et al. Systemic lupus erythematosus. Nat Rev Dis Primers. 2016;2:16039.
Li PH, Wong WH, Lee TL, Lau CS, Chan TM, Leung AM, et al. Relationship between autoantibody clustering and clinical subsets in SLE: cluster and association analyses in Hong Kong Chinese. Rheumatology (Oxford). 2013;52(2):337–45.
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