Population-level single-cell genomics reveals conserved gene programs in systemic juvenile idiopathic arthritis

Verweyen, Emely L.; Thakkar, Kairavee; Dhakal, Sanjeev; Baker, Elizabeth; Chetal, Kashish; Schnell, Daniel; Canna, Scott; Grom, Alexei A.; Salomonis, Nathan; Schulert, Grant S.

doi:10.1172/jci166741

Journal of Clinical Investigation

2023

DOI: 10.1172/jci166741

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Population-level single-cell genomics reveals conserved gene programs in systemic juvenile idiopathic arthritis

Emely L. Verweyen,

Kairavee Thakkar,

Sanjeev Dhakal

et al.

Abstract: AAG reports research support and consulting fees from Novartis, SOBI, and AB2Bio. GSS reports consulting fees from Novartis and SOBI and research support from IpiNovyx. SC has received consulting fees from SOBI, Apollo, and Simcha. All other authors have declared that no conflict of interest exists.

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2024

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Cited by 2 publications

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Interleukin (IL)-1/IL-6-Inhibitor–Associated Drug Reaction With Eosinophilia and Systemic Symptoms (DReSS) in Systemic Inflammatory Illnesses

Saper,

Tian,

Verstegen

et al. 2024

The Journal of Allergy and Clinical Immunology: In Practice

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Interleukin (IL)-1/IL-6-Inhibitor–Associated Drug Reaction With Eosinophilia and Systemic Symptoms (DReSS) in Systemic Inflammatory Illnesses

Saper,

Tian,

Verstegen

et al. 2024

The Journal of Allergy and Clinical Immunology: In Practice

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Clinical characteristics and prognosis of interstitial lung disease in systemic juvenile idiopathic arthritis: a two-center retrospective observational cohort study

Zhan,

Yang,

Qiu

et al. 2024

Pediatr Rheumatol

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Background Interstitial lung disease (ILD) is a serious complication in systemic juvenile idiopathic arthritis (SJIA). This study aimed to identify the clinical characteristics and prognosis of SJIA-ILD. Methods A two-center retrospective cohort study was conducted on patients newly diagnosed with SJIA in China from October 2010 to December 2021. Clinical characteristics, laboratory parameters, outcomes, and relapse rates were compared between ILD and non-ILD groups. Results A total of 176 children with SJIA were included, including 35 in ILD group and 141 in non-ILD group. The median age at onset of SJIA was 5.8 years (range 4.4–9.5) in patients with SJIA-ILD. It exhibited higher incidences of cervical spine (28.6%) and hip involvement (40.0%) in ILD group ( P = 0.031 and P = 0.029, respectively). The incidence of macrophage activation syndrome (MAS) in ILD group reached up to 40%, significantly elevated than that in non-ILD group ( P = 0.047). Children with ILD demonstrated a stronger inflammatory response and were more prone to developing lymphopenia ( P = 0.009), requiring more combination therapy ( P = 0.006) to control disease activity. 54.3% of patients received biologic therapies, with only three patient receiving biologics (one with IL-6 blockade, two with TNF inhibitor) prior to ILD onset and none receiving IL-1 blockade. The median follow-up duration was 6.0 years (range 3.9–9.5). The proportions of patients with SJIA-ILD achieving clinical inactive disease without glucocorticoids within 6 to 12 months of the treatment were significantly lower than control group (45.7% vs. 70.2%, P = 0.006). In ILD group, only 54.3% of patients achieved complete remission, and 17.1% were in a non-remission state, among whom two deaths from respiratory failure. There was no significant difference in disease relapse rates between the two groups ( P > 0.05). Conclusions Patients with SJIA-ILD exhibited heightened inflammation, increased hip joint and cervical spine involvement, and were more susceptible to developing lymphopenia and MAS, suggesting a relatively poor prognosis. They required a prolonged time to control inflammation and more aggressive treatment strategies to achieve inactive status. The unsatisfactory rate of complete remission highlighted an urgent need for focused clinical strategies.

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Population-level single-cell genomics reveals conserved gene programs in systemic juvenile idiopathic arthritis

Cited by 2 publications

References 56 publications

Interleukin (IL)-1/IL-6-Inhibitor–Associated Drug Reaction With Eosinophilia and Systemic Symptoms (DReSS) in Systemic Inflammatory Illnesses

Interleukin (IL)-1/IL-6-Inhibitor–Associated Drug Reaction With Eosinophilia and Systemic Symptoms (DReSS) in Systemic Inflammatory Illnesses

Clinical characteristics and prognosis of interstitial lung disease in systemic juvenile idiopathic arthritis: a two-center retrospective observational cohort study

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