Performance of the Revised Classification Criteria for Systemic Autoimmune Rheumatic Diseases and Their Overlap Syndromes

Abstract

Objective We evaluated the performance of the revised classification criteria for assessing different systemic autoimmune rheumatic diseases and their overlap syndromes. Methods A total of 652 patients with or highly suspected of having systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositis (PM)/dermatomyositis (DM) or rheumatoid arthritis (RA)were included in this study. The 1997 revised American College of Rheumatology (ACR) and the 2019 European League Against Rheumatism (EULAR)/ACR criteria for SLE, the 1980 ACR and the 2013 ACR/EULAR criteria for SSc, the criteria by Bohan and Peter and the 2017 EULAR/ACR criteria for PM/DM, and the 1987 revised ACR and 2011 ACR/EULAR criteria for RA were used for disease classification. Results The old and new criteria and a clinical diagnosis were used to respectively classify 103, 106 and 105 SLE patients; 35, 47 and 58 SSc patients; 18, 23 and 33 PM/DM patients; and 297, 389 and 468 RA patients. Sensitivity increased from 82.9% to 92.4% in SLE, from 56.9% to 79.3% in SSc, from 54.5% to 66.7% in PM/DM, and from 62.6% to 80.8% in RA. SLE-SSc was the predominant type of clinical overlap syndrome, while SLE-RA was the most classifiable. Conclusion The revised classification criteria for all the diseases showed an improved sensitivity, and SLE-overlap syndrome was predominant, regardless of the criteria sets.

A Clinical Picture of Unselected Patients with Systemic Lupus Erythematosus in a Tertiary Hungarian Center—A Spectrum Ranging from Pure Lupus to Overlap Syndromes

“…Nevertheless, the existence of overlap syndromes was established based on the clinicians’ opinions and it was not investigated whether it fulfilled the classification criteria or not, which is another limitation to consider. In a recently published paper with 21% of the lupus patients studied having overlap syndromes, the authors found a very similar sensitivity, which was 82.9% for the 1997 ACR criteria and 92.4% for the 2019 EULAR/ACR criteria [ 8 ]. Overall, in SLE with overlap syndrome, we obtained results with a sensitivity comparable to the original publications, which was at the same time similar to the few results in the published literature to date in this area.…”

“…There was also one that looked at the same criteria in a narrower subgroup, e.g., SLE with a long disease duration [ 7 ]. One publication investigated the effect of revising criteria in different connective tissue diseases (CTDs), in which the newer criteria showed increasing sensitivity [ 8 ]. On the other hand, in the last few years, only a small number of publications have analyzed lupus overlap disease, and none of them have examined the impact of the presence of an overlap disease on the fulfillment of the criteria [ 9 , 10 , 11 ].…”

Inhibition of miRNA associated with a disease-specific signature and secreted via extracellular vesicles of systemic lupus erythematosus patients suppresses target organ inflammation in a humanized mouse model

“…Similarly, clinical tests to diagnose SLE or to predict flares have also lagged behind other scientific advances in medicine. In the “omics” era of biomarker discovery via DNA/RNA sequencing, epigenetics, and proteomics, SLE is still largely diagnosed and treated according to clinical classification criteria ( 43 , 44 ). Typical laboratory testing conventionally ordered in the clinic to aid in SLE diagnosis includes anti-nuclear antibodies (ANA), anti-double-stranded DNA antibodies (anti-dsDNA), and complement levels, but these tests have limited sensitivity and specificity ( 45 – 47 ).…”

Inhibition of extracellular vesicle-encapsulated miRNA produced by estrogen-mediated upregulation of cellular processing suppresses target organ inflammation in a humanized model of systemic lupus erythematosus

“…Similarly, clinical tests to diagnose SLE or to predict flares have also lagged behind other scientific advances in medicine. In the "omics" era of biomarker discovery via DNA/RNA sequencing, epigenetics, and proteomics, SLE is still largely diagnosed and treated according to clinical classification criteria, as defined by the American College of Rheumatology (ACR) and the Systemic Lupus International Collaborating Clinics (SLICC) [39,40]. Typical laboratory testing conventionally ordered in the clinic to aid in SLE diagnosis includes anti-nuclear antibodies (ANA), anti-double-stranded DNA antibodies (anti-dsDNA), and complement levels, but these tests have limited sensitivity and specificity [41][42][43].…”