Study Addresses Autoimmune Connective Tissue Disease in At-Risk Patients
Autoimmune connective tissue diseases (AI-CTDs) are preceded by asymptomatic antinuclear antibody (ANA) positivity. In a recent study, researchers reported 3-year follow-up data of a cohort of ANA-positive referrals without AI-CTDs to provide more detailed analysis of the non-progressor group and predictors.
Patients were enrolled if they had ANA, did not meet criteria for AI-CTD, and had symptoms for less than 12 months. Diagnostic criteria for AI-CTDs and therapies were evaluated at baseline then yearly for 3 years. The researchers categorized progression as: absolute-non-progressors (no clinical criteria at all time points: 0-36 months), undifferentiated-CTD (³1 clinical criterion at baseline and/or at follow up but not meeting criteria), year-1-progressor (meeting criteria for AI-CTD within 12 months), and late-progressor (meeting criteria for AI-CTD later than 12 months). A 2-score analysis of interferon (INF) status was performed.
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Three-year follow-up data was available for 146 patients. Of the cohort, 23% (n=33) were categorized as absolute-non-responders, 59% (n=86) as undifferentiated-CTD, 14% (n=21) as year-1 progressors, and 3% (n=5) as late progressors. No patient progressed or required immunosuppression after 2 years. Six of 86 patients with undifferentiated-CTD required an immunosuppressant.
Of the 31 patients with no clinical criteria at baseline, one progressed to meet criteria within 1 year, two progressed at 1 to 2 years, three were prescribed hydroxychloroquine, and one was prescribed an immunosuppressant. Additionally, 108 patients with at least one criterion at baseline had the highest risk. The researchers reported that 20 patients progressed to meet criteria within 1 year, two progressed at 1 to 2 years, and the remaining cohort all had undifferentiated-CTD.
Researchers found no association between extractable nuclear antigen, C3 or C4, and clinical outcome. The association between IFN Score B and progression was more prevalent when comparing year 1 progessors with absolute-non-progressors (P=.007). IFN Scores did not predict late progression. They noted, however, that within undifferentiated-CTD, patients who required an immunosuppressant had higher expression of IFN Score A (P=.011) and IFN Score B (P<.001).
“Among ANA-positive referrals, no clinical feature or routine laboratory test could rule out development of clinically significant disease, which included AI-CTD or undifferentiated-CTD needing therapy. However, IFN Scores had a unique value in predicting these outcomes. At-risk individuals who ultimately developed clinically significant disease are therefore immunologically but not clinically distinctive,” concluded the researchers. “Future work will incorporate biomarkers into clinically applicable risk models to allow earlier exclusion of AI-CTD or trials of preventative treatment.”
—Eileen Koutnik-Fotopoulos
Reference
Ul-Hassan S, Dutton K, Wigston Z, Alase A, Yusof Y, Vital EM. O34 Predicting autoimmune connective tissue diseases: three year follow up of an at-risk cohort identifies late progression and predicts need for therapy. Rheumatology. 2020;59(suppl 2):ii16. https://doi.org/10.1093/rheumatology/keaa110.033
