Background Evolution from Undifferentiated Connective Tissue Disease (UCTD) to a defined Autoimmune Disease (AID) remains unexplained and usually occurs in the first 3 to 5 years.
Objectives To determine the frequency and predictors of differentiation from UCTD to AID in a selected cohort of patients originally labelled as UCTD within the first 3 years of follow-up.
Methods Demographic and clinical features were retrieved: (i) retrospectively, from the AID's Unit current database in December 2016 (n=195), including Systemic Lupus Erythematosus (SLE), incomplete SLE, Sjögren's Syndrome (SS), Systemic Sclerosis (SSc), VEDOSS and Mixed Connective Tissue Disease (MCTD); and (ii) from a prospectively UCTD database created in January 2012 (n=48). Inclusion criteria for the latter pertains to ANA positive patients, not fulfilling any of the existing classification criteria for AID and without severe organ involvement . Patients with cutaneous lesions suggestive of lupus, inflammatory myopathies, erosive arthritis, systemic sclerosis and certain auto-antibody profiles were excluded a priori from the definition of UCTD as these are thought to herald defined conditions, as previously defined . Definition of stable UCTD includes patients with at least one clinical manifestation of AID, positive ANA result and disease duration of at least 3 years . Comparisons between stable UCTD and progressing patients were made using Wilcoxon Rank Sum and Chi square tests, p values of <0.05 were considered statistically significant.
Results Each individual patient was analysed for differentiation into AID at yearly intervals. Overall, the main features of the prospective UCTD cohort were arthralgia (79%), rash (31%), arthritis (19%), sicca symptoms (19%), photosensitivity (17%) and Raynaud (13%). Prospective analysis in the UCTD cohort revealed differentiation in 4/48 patients (8.3%): into rheumatoid arthritis (n=2), psoriatic arthritis (n=1) and SLE (n=1). The main difference between stable UCTD and those that progressed to AID was the presence of arthritis (p=0.003). Median age of onset and symptom duration was similar between both groups. Retrospective analysis yielded very few patients presenting as UCTD (n=5/195): 2/106 SLE; 1/13 incomplete SLE; 1/43 SS; 1/19 SSc; 0/7 VEDOSS and 0/7 MCTD with no distinguishing features.
Conclusions Very few patients differentiated in the UCTD cohort after 3 years of follow-up and in our retrospectively studied cohort, in accordance to a previous study . Apart from arthritis, there were no other predicting factors for differentiation to AID. UCTD at disease onset seems to be a rare event.
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Disclosure of Interest None declared