50th Year of Publication: Exploring Advances in Rheumatology in the 2000s

In this edition of The Journal of Rheumatology, I will be bringing to your attention the following 3 articles which had, and continue to have, a significant impact in rheumatology. The first paper describes remission criteria for juvenile idiopathic arthritis (JIA),¹ the second has become a gold standard for studies in systemic lupus erythematosus (SLE),² and the third introduced the concept of classification of early systemic sclerosis (SSc).³

The first paper I wish to bring your attention to from the 2000s is the “Preliminary Criteria for Clinical Remission for Select Categories of Juvenile Idiopathic Arthritis” by Wallace et al.¹ It may seem hard to believe in 2023, but until the publication of this paper, there were no universally accepted criteria for remission in JIA. Using a Delphi or consensus technique that included pediatric rheumatologists worldwide, this paper defined 3 stages of quiescent disease: inactive disease, clinical remission on medication, and clinical remission off medication. Wallace et al suggested that these criteria could be used by clinicians in day-to-day practice, for evaluating new therapies, and even to study the disease process.¹ The authors felt that the draft criteria were a “work-in-progress and undoubtedly will go through considerable iteration.”¹ The criteria are still known as the Wallace criteria.

The authors, I am sure, are quite proud of the fact that their subsequent article, again from an international collaboration, only slightly modified the criteria and used the term clinically inactive disease.⁴ In fact, the changes did not alter the receiver-operating characteristic curves but increased face and content validity. The original Wallace criteria continue to be used in 2023 in outcome studies.^5,6

The next paper to review is “Systemic Lupus Erythematosus Disease Activity Index 2000” by Gladman et al.² In 1992, the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was developed and validated as a clinical index for the measurement of disease activity in SLE.⁷ Although the SLEDAI was used as a gold standard to measure disease activity, it did not reflect persistently active disease and therefore may not have reflected damage nor have been a good measure response in clinical trials.⁷ The major modifications to the original SLEDAI in the SLEDAI 2000 (SLEDAI-2K) allowed for persistently active disease in the items of alopecia, mucous membrane ulcers, rash, and proteinuria.²

Since this article,² there have been further modifications to the SLEDAI-2K to improve measurement of disease activity and response to therapy in clinical trials.^8,9 However, the SLEDAI-2K remains one of the 2 measures of disease activity used to determine efficacy of new therapies in SLE.^2,10

The final paper to bring your attention to is the “Criteria for the Classification of Early Systemic Sclerosis” by LeRoy and Medsger.³ This article is essentially an editorial or commentary on how to begin to classify patients with early SSc. The classification criteria proposed up until the publication of this manuscript was the 1980 preliminary criteria for the classification of SSc, which was designed intentionally to be specific rather than sensitive, to minimize false positive diagnoses.¹¹ In this article, the authors suggested that improvements in the accurate detection of patients with Raynaud phenomenon, use of nailfold microscopy, and better serologic testing since 1980 allowed for the identification of patients with features of SSc who do not fulfill the 1980 criteria for SSc.³

LeRoy and Medsger tested the proposed criteria in 2 cohorts and were able to show that although 134/639 patients in the Pittsburgh cohort and 2/3 of patients with limited SSc in the second criteria did not meet 1980 criteria,³ more than 90% of the above-mentioned patients did meet the proposed criteria for early SSc.¹² Early SSc is now recognized, as is very early SSc. The article by LeRoy and Medsger was the initial paper to recognize that it is important to identify patients with early and even very early SSc.^13,14

I could highlight other papers from the 2000s that continue to be important to rheumatology in 2023, but I made the philosophical decision to restrict myself to 3 papers from each decade. I hope that you will enjoy reading these 3 articles, which are still useful today.

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