Anti-mitochondrial antibodies as markers of disease activity in childhood-onset systemic lupus erythematosus: a longitudinal cohort study.

Objectives: Mitochondria are prominent antigenic sources, capable of triggering IFN-induced inflammatory pathways in SLE. Recent studies suggest presence of AMA in LN patients with adult-onset SLE. Whether AMA reflect disease activity in childhood-onset SLE (cSLE) remains unexplored.

Amlexanox inhibits production of type I interferon and suppresses B cell differentiation : a possible therapeutic option for systemic lupus erythematosus and other systemic inflammatory diseases.

Objectives: Activation of the type I interferon (IFN) pathway and autoreactive B cells are key immunopathogenic features of systemic lupus erythematosus (SLE), primary Sjögren's disease (pSjD) and systemic sclerosis (SSc). TANK-binding kinase 1 (TBK1) is a mediator of type I IFN and essential during B cell development in mice. We investigated the properties of the TBK1 inhibitor amlexanox in systemic autoimmune diseases.

Learning from serum markers reflecting endothelial activation: longitudinal data in childhood-onset systemic lupus erythematosus.

Objectives: In childhood-onset SLE (cSLE), patients have an increased risk of premature atherosclerosis. The pathophysiological mechanisms for this premature atherosclerosis are not yet completely understood, but besides traditional risk factors, the endothelium plays a major role. The first aim of this study was to measure levels of SLE-associated markers involved in endothelial cell (EC) function and lipids in a cSLE cohort longitudinally in comparison with healthy controls (HC).

Targeted multiomics in childhood-onset SLE reveal distinct biological phenotypes associated with disease activity: results from an explorative study.

Objective: To combine targeted transcriptomic and proteomic data in an unsupervised hierarchical clustering method to stratify patients with childhood-onset SLE (cSLE) into similar biological phenotypes, and study the immunological cellular landscape that characterises the clusters.

Methods: Targeted whole blood gene expression and serum cytokines were determined in patients with cSLE, preselected on disease activity state (at diagnosis, Low Lupus Disease Activity State (LLDAS), flare). Unsupervised hierarchical clustering, agnostic to disease characteristics, was used to identify clusters with distinct biological phenotypes.

LLDAS is an attainable treat-to-target goal in childhood-onset SLE.

Objectives: To study whether clinical remission (CR) and Low Lupus Disease Activity State (LLDAS) are achievable goals in childhood-onset SLE.

Methods: Data on medication use and disease activity were prospectively collected. LLDAS was defined as Safety of Estrogen in Lupus Erythematosus National Assesment-SLE disease Activity Index (SELENA-SLEDAI) ≤4 with zero scores for renal, Central Nervous System (CNS), serositis, vasculitis and constitutional components, no increase in any SLEDAI component since the previous visit, PGA ≤1, and prednisone dose ≤7.