Application of SLICC classification criteria in undifferentiated connective tissue disease and evolution in systemic lupus erythematosus: analysis of a large monocentric cohort with a long-term follow-up

Abstract

Objectives The objectives of this study were to analyse the performance of the Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria for systemic lupus erythematosus (SLE) in a large cohort of undifferentiated connective tissue disease (UCTD) population at onset of the disease and during a long-term follow-up of 15 years (1999-2013) and to evaluate the transition from UCTD to SLE, according to American College of Rheumatology (ACR) 1997 and SLICC 2012 classification criteria. Methods A cohort of patients who met the classification criteria proposed by Mosca et al. for UCTD, were analysed. The SLICC 2012 classification criteria for SLE were retrospectively applied to each patient at the time of the diagnosis (T0) and also periodically re-applied and compared to ACR 1997 criteria at three different time points in the follow-up. Results 329 patients were enrolled. According to inclusion criteria at T0 no patient met the SLE/ACR criteria, whilst, retrospectively applying the SLE/SLICC criteria, 44 patients already satisfied this set of criteria for SLE. During the follow-up 23 new patients reached the SLE/SLICC criteria and 14 patients met the ACR criteria with a stable rate of progression to SLE over time. Acute or subacute skin rash, antiphospholipid antibody (aPL) positivity and serositis were the variables correlated to the evolution to SLE. Conclusions In our UCTD population, the application of SLICC classification criteria for SLE at disease onset allowed identification of a proportion of otherwise missed SLE cases; during follow-up, and compared with ACR criteria, SLICC criteria expanded the number of patients classifiable as SLE otherwise classified as UCTD.

Incomplete Systemic Lupus Erythematosus: What Remains After Application of American College of Rheumatology and Systemic Lupus International Collaborating Clinics criteria?

“…Most publications on iSLE are based on patients with clinical features of SLE who do not fulfill ACR criteria (3), and only some studies use the SLICC criteria. Table shows an overview of these studies .…”

“…Bortoluzzi et al retrospectively selected 329 white patients with UCTD (defined as having signs and symptoms suggestive of a connective tissue disease), ANA positivity, and a disease duration of at least 1 year who did not fulfill ACR criteria (3). In retrospect, 44 patients (13%) already fulfilled the SLICC criteria (4) at baseline.…”

SLE: reconciling heterogeneity

“…The linear illness model posits that UAS, overlap, antibody-only and typical SLE reside within a continuum of a single pathogenic process. This model applies throughout a patient’s lifetime, during which the diagnosis name may change 41–49. The model’s strengths are that it suggests common pathogeneses and flexible treatment protocols for all lupus spectrum illnesses; it highlights potential causes for phenotype changes.…”

Systemic lupus erythematosus: an update

“…The more extended clinical and laboratory items that make up the SLICC classification criteria (see a summary in Box 1, and more details in the at http://mja.com.au) include conditions such as transverse myelitis, autoimmune haemolytic anaemia or immune‐mediated cytopenias that carry separate weighting. Whereas previously, patient's symptoms and test results may have been labelled as undifferentiated, the new criteria allow identification of otherwise missed SLE cases; in some cases this may result in earlier diagnosis 9 …”

“…Whereas previously, patient's symptoms and test results may have been labelled as undifferentiated, the new criteria allow identification of otherwise missed SLE cases; in some cases this may result in earlier diagnosis. 9 Autoantibody profiling can be useful in aiding the diagnosis of SLE. Antinuclear antibodies (ANA) are present in the vast majority of patients with SLE, but are not specific and can be seen at low to moderate titres in 5e13% of the normal population.…”