Follow-up and management of serologically active clinically quiescent cases in pediatric systemic lupus erythematosus

Gabrielle Capone; Caroline Lojacono; Fatma Al-Bayitee; Shayan Makvandi; Teresa Hennon; Brian Wrotniak; Rabheh Abdul-Aziz

doi:10.5114/reum.2021.108353

Follow-up and management of serologically active clinically quiescent cases in pediatric systemic lupus erythematosus

Reumatologia. 2021;59(4):244-251. doi: 10.5114/reum.2021.108353. Epub 2021 Aug 13.

Authors

Gabrielle Capone¹, Caroline Lojacono¹, Fatma Al-Bayitee¹, Shayan Makvandi¹, Teresa Hennon¹, Brian Wrotniak¹, Rabheh Abdul-Aziz¹

Affiliation

¹ Department of Pediatric Rheumatology, University at Buffalo, United States.

Abstract

Objectives: Our aim is to identify the presence of serologically active clinically quiescent (SACQ) episodes in pediatric systemic lupus erythematosus (SLE) patients. We aim to identify serologic biomarkers associated with SACQ episodes and discuss risks and benefits of escalating treatments.

Material and methods: We evaluated 25 pediatric SLE patients, 13 of whom experienced SACQ episodes. Serologically active clinically quiescent was defined as two consecutive clinic visits without any clinical symptoms or clinical examination findings of a lupus flare with a clinical Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score of zero, but either elevated anti-ds-DNA antibodies or low complement (C3 and/or C4) levels.

Results: Among the 13 patients who experienced a SACQ episode, there were a total of 24 episodes, with each patient experiencing 1-4 SACQ episodes. Erythrocyte sedimentation rate (ESR) was the most commonly elevated laboratory marker in a SACQ episode, followed by low hemoglobin levels, and then elevated anti-dsDNA antibodies. Of the 17 episodes treated during a SACQ episode, 15 (88%) did not progress to a clinical flare within six months, while two did. Furthermore, of the 7 patients who were not treated during their SACQ episode, 2 (29%) continued to be SACQ without flare, whereas 5 led to a clinical flare within six months.

Conclusions: Serologically active clinically quiescent episodes were identified in pediatric SLE patients, suggesting that the presence of SACQ is not limited to adults with SLE. Serologic markers such as increased ESR, hemoglobin, and elevated anti-dsDNA antibodies are preliminarily associated with pediatric SACQ episodes. Treating these SACQ episodes in pediatric SLE patients was less likely to lead to a clinical flare within six months when compared to not treating (p < 0.05). More research with a larger sample size is needed to define SACQ episodes, determine the prevalence in pediatric SLE patients, and establish SACQ treatment guidelines.

Keywords: Systemic Lupus Erythematosus Disease Activity Index 2000; serologically active clinically quiescent; systemic lupus erythematosus.