Jan 16th, 2022 - WAIKOLOA, Hawaii — It is important for physicians to consider “the perils that dwell” in the diagnostic management of patients with uveitis, Steven Yeh, MD, said during a presentation at Hawaiian Eye 2022. “With these patients being very complex oftentimes, we really just don’t want to miss something,” Yeh said. To better identify “the pearls amongst the perils” in uveitis diagnosis, it is impo...

Nov 14th, 2022 - Background/Purpose: Patients with systemic lupus erythematosus (SLE) experience photosensitivity where exposure to ultraviolet light B (UVB) drives lupus flares and systemic symptoms. Although the mechanisms linking UVB exposure to these effects are unclear, type I IFNs are known to play a role. TRIM21, an autoantigen in SLE, functions as a negative regulator on the pathways driving IFN express...

Nov 14th, 2022 - Background/Purpose: There is increased interest in machine learning (ML)-based prediction models in systemic lupus erythematosus (SLE). We made a systematic review of adherence in diagnostic and prognostic applications of ML in SLE using the Transparent Reporting of a multivariable prediction model for Individual Prognosis Or Diagnosis (TRIPOD) Statement. Methods: A systematic review using 5 da...

Nov 14th, 2022 - Background/Purpose: Pain is a hallmark feature of psoriatic arthritis (PsA); however, its apparent multi-factorial nature makes it difficult for rheumatologists to assess and manage. Clinically, nociplastic processes appear to contribute to the PsA pain experience, as shown by frequent co-existing fibromyalgia (FM), a prototypical nociplastic pain disorder [1,2]. Yet there are no objective data...

Nov 14th, 2022 - Background/Purpose: Matrix metalloproteinases (MMPs) degrade extracellular matrix and have been implicated to play a role in a number of fibrotic processes. Specifically, MMPs have been speculated to play a role in the pathogenesis of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) as well as idiopathic pulmonary fibrosis (IPF), which shares historadiological features with RA...

Nov 14th, 2022 - Background/Purpose: Immune-mediated inflammatory arthritis (IMIA) is a group of diseases characterized by chronic synovitis including rheumatoid arthritis (RA), psoriatic arthritis (PsA), and spondyloarthritis (SpA). Some disease modifying antirheumatic drugs (DMARDs) have demonstrated efficacy in several of these diseases (e.g. TNFα inhibitors) while others have not (e.g. IL-17A inhibitors in ...

Nov 14th, 2022 - Background/Purpose: Pathologic inflammation is a major driver of kidney damage in lupus nephritis (LN), but the immune mechanisms of disease progression and risk factors for end organ damage are poorly understood. Previous studies established the NZM2328 lupus-prone mouse strain as a model for human proliferative glomerulonephritis (GN). These studies determined that disease in female NZM2328 m...

Nov 13th, 2022 - Background/Purpose: Inflammatory and degenerative processes in the joint tissues are hallmarks of patients with psoriatic arthritis (PsA). Here, proteases play a major role in remodeling of the extracellular matrix (ECM) where collagens are major structural components. During inflammation, proteolytic processing of the ECM leads to unique protease-mediated breakdown products of collagens and pr...

Nov 13th, 2022 - Background/Purpose: Major adverse cardiovascular events (MACE) and neoplasms are a concern in autoimmune diseases. RA and PsA are diseases where inflammation plays a key role in the development of the disease and the production of short- and long-term damage. TNF alpha plays a significant role in inflammation and the JAK/STAT signaling pathway is an important regulatory signaling cascades of in...

Nov 13th, 2022 - Background/Purpose: By converting extracellular ATP into homeostatic adenosine, the ectonucleotidases CD39 (ATP to AMP) and CD73 (AMP to adenosine) dictate extracellular purinergic gradients and as such are critical players in the regulation of leukocyte and platelet functions. Insufficient activity of these homeostatic ectoenzymes has been associated with neutrophil-driven inflammation. Crosst...

Nov 13th, 2022 - Background/Purpose: One of the hallmarks of SLE is clinical and molecular heterogeneity. Recent work has leveraged next-generation sequencing techniques to identify molecular signatures characteristic of subsets of patients’ disease. Here, we employed gene set variation analysis (GSVA) of informative gene modules coupled with machine learning techniques to identify molecular endotypes of SLE pa...

Nov 13th, 2022 - Background/Purpose: Macrophage activation syndrome is a diagnosis along the spectrum of hemophagocytic lymphohistiocytosis (HLH) spectrum disorders. It is a rare, yet potentially fatal disease characterized by hyperferritinemia, coagulopathy and multisystem inflammation. Predictive biomarkers for outcomes in MAS are lacking and hence this study yearns to elucidate them. Activation of complement...

Nov 13th, 2022 - Background/Purpose: Chronic pain is one of the most common complaints of individuals with Ehlers-Danlos syndrome (EDS) and the effects of the existing therapeutical modalities are at best only partially effective. The etiology and mechanisms of pain in EDS have barely been studied, and the current understanding of the underlying neural, molecular and cellular pathways is minimal. Classical EDS ...

Nov 13th, 2022 - Background/Purpose: Intercellular adhesion molecule-1 (ICAM-1) regulates leukocyte recruitment from circulation to sites of inflammation, being considered as a marker of underlying endothelial damage [1]. Moreover, ICAM-1 contributes to the development of interstitial lung disease (ILD) and it has been proposed as a prognostic biomarker in idiopathic pulmonary fibrosis (IPF) [2]. Although ILD i...

Nov 13th, 2022 - Background/Purpose: SARS-CoV-2 causes a severe inflammatory syndrome (COVID-19) leading, in many cases, to bilateral pneumonia, severe dyspnea and in ~5% of these, death. DNA methylation is known to play an important role in the regulation of the immune processes behind COVID-19 progression, however it has not been studied in depth. In this study, we aimed at evaluating the implication of DNA m...

Nov 13th, 2022 - Background/Purpose: Pediatric scleroderma consists of two diseases, juvenile localized scleroderma (jLS) and juvenile systemic sclerosis (jSSc). While jLS and jSSc share some disease processes, there are major differences in their clinical features and morbidity patterns. Co-existence of LS and SSc has been reported in adults, with a recent adult systematic review reporting a frequency of 2.4-7...

Nov 13th, 2022 - Background/Purpose: Systemic lupus erythematosus (SLE) may be triggered by gene-environment interactions. Data are scarce on how epigenetic variance contributes to disease risk in pediatric SLE (pSLE). Our object was to identify differences in chromatin accessibility in treatment-naive pSLE compared to healthy children (HC) and pSLE patients post-induction therapy. Methods: We used assays for t...

Nov 12th, 2022 - Background/Purpose: Interstitial lung disease (ILD) is one of the main comorbidities of patients with RA contributing to an increased mortality risk [1]. The pathogenesis of RA-ILD remains poorly defined, constituting its early diagnosis a challenge for clinicians [1]. The endothelium plays a pivotal role in inflammatory diseases and in the pulmonary vascular regulation contributing to the deve...

Nov 12th, 2022 - Background/Purpose: Interstitial lung disease (ILD) may occur in patients with a rheumatic autoimmune disease (AD), increasing their morbidity and mortality [1]. In this regard, the identification of serum biomarkers of AD-ILD is of potential interest. Endothelin-1 (ET-1) is a proinflammatory protein involved in multiple vascular and fibrosing abnormalities [2-3], characteristic processes of AD...

Nov 12th, 2022 - Background/Purpose: In 2001 I started to experience forearm, elbow, and wrist pain. My fingers were constantly swollen, and sensitive. After 6 months of living in pain and stiffness, I sought out help. I was then diagnosed with Systemic Diffuse Scleroderma. I developed Interstitial Lung disease, hypertension and heart complications almost immediately. My skin became hard and tight. My hands con...