The following is a summary of “Pediatric Sarcoidosis: Retrospective Analysis of Biopsy-Proven Patients,” published in the July 2023 issue of Rheumatology by Nott et al.

This study aims to characterize the phenotype, disease course, and treatment of a large cohort of children with sarcoidosis. This study included patients with biopsies consistent with sarcoidosis conducted between 2010 and 2020. The patient records were retrospectively evaluated. Children diagnosed with a disease before age five were compared to older children. A regression analysis was conducted to identify treatment outcome predictors. About 48 children with a mean age at diagnosis of 9.5 years and a ratio of 0.71 males to females were identified. 

Around 72% of the children were Black, and 94% had multiorgan disease, with an average of 4.8 organs affected, lymph nodes (65%), epidermis (63%), and eyes (60%) being the most common. Significant laboratory results included elevated serum calcium levels in 23% of patients and elevated angiotensin-converting enzyme levels in 76%. Six of the fourteen tested patients had mutations in the NOD2 gene. About 81% of patients received systemic steroids, and 90% received conventional disease-modifying antirheumatic drugs (DMARDs); in 25% of patients, a biologic, primarily anti–tumor necrosis factor (anti–TNF) was administered. Although most patients could be weaned off steroids (58%), most remained on long-term DMARDs (85%). Children under 5 years old were more likely to exhibit splenomegaly (P = 0.001), spleen involvement (P = 0.003), and elevated C-reactive protein levels (P= 0.10). Teenage weight loss was more prevalent (P = 0.006). 

Involvement of the kidney (P = 0.004), eye (P = 0.005), and liver (P = 0.03) was more prevalent in Black patients. No singular factor was identified by regression analysis as being associated with favorable treatment outcomes. Pediatric sarcoidosis is characterized by multiorgan involvement, steroid response, and chronic course. The phenotype varies considerably with age and race. Adding an anti-TNF agent was advantageous when conventional DMARDs were ineffective.

Source: jrheum.org/content/50/7/924