Treatment of systemic vasculitis
This review will attempt to summarize the most potentially impactful new data on the treatment of systemic vasculitic conditions, including ANCA-associated vasculitis (AAV), giant cell arteritis, polymyalgia rheumatica and Takayasu arteritis.
Vasculitis associated with nonhematological malignancies
There is a complex relationship between nonhematological malignancies and vasculitis Paraneoplastic vasculitis may present in many different forms. Cancer risk is high in patients with some types of vasculitis. Also, immune checkpoint inhibitors (ICIs) used in treatment of many solid tumors may cause vasculitis.
Vasculitis in autoinflammatory diseases
This review aims to explore the relationship between autoinflammatory diseases (AIDs) and vasculitis, with a focus on recently identified syndromes and newly published data since 2016.
Decoding VEXAS syndrome: emerging insights into pathogenesis and clinical management
VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is a newly discovered, adult-onset, hemato-inflammatory disease driven by clonal dominance of pro-inflammatory hematopoietic cells bearing a somatic mutation in the UBA1 gene. This review aims to integrate and discuss the most recent insights into the evolving understanding of VEXAS pathogenesis and clinical management.
CAR T-cell therapy in systemic sclerosis: the next frontier in immune modulation
Cellular therapies such as CD19-targeting CAR T cells are a rapidly evolving field in an area of unmet clinical needs: autoimmune diseases including systemic sclerosis (SSc). The aim of this review is to summarize the available data on safety and efficacy of CAR T-cell therapy in SSc and to discuss upcoming developments and challenges for the near future.
Vasculitis associated with haematologic malignancies
This review examines the complex bidirectional relationship between vasculitis and hematologic malignancies, highlighting the importance of meticulous diagnostic assessment.
Advances in the diagnosis and treatment of Sjögren disease
Sjögren disease (SjD) constitutes a diagnostic and therapeutic challenge due to its clinical heterogeneity and complex pathophysiology. This review synthesizes recent advances in diagnostics, disease stratification, and targeted therapies, highlighting their potential to optimize patient care.
What have we learned about systemic sclerosis from the EUSTAR database?
This review provides a timely synthesis of key findings derived from the EUSTAR (European Scleroderma Trials and Research) database, the largest international registry dedicated to systemic sclerosis (SSc), now including over 27 000 patients worldwide. As interest grows in real-world data and precision medicine in rare diseases, EUSTAR offers a uniquely rich, longitudinal dataset built over two decades of global collaboration. With sustained growth, more than 1000 new patients enrolled annually, this registry continues to inform clinical practice and research with contemporary, diverse patient data.
Harnessing artificial intelligence to advance insights in systemic sclerosis skin and lung disease
The purpose of this review is to summarize the uses of artificial intelligence for advancing systemic sclerosis (SSc) skin and lung disease research through 2024.
Spatial transcriptomics: challenges and future directions in musculoskeletal diseases
This review examines recent advancements in spatial transcriptomics and its current and potential use to advance musculoskeletal (MSK) research. These insights will be vital to address the complexity of MSK diseases and will pave the way for future therapeutic developments.
Targeted therapies in systemic sclerosis: a narrative review of novel drugs in clinical trials
Systemic sclerosis (SSc) remains a therapeutic challenge, with conventional immunosuppressive strategies showing inconsistent effects and no disease modifying activity. The lack of head-head trials comparing immunosuppressives with emerging antifibrotic agents further complicates treatment decisions in SSc. This review aims to provide an update on the recent advances in targeted therapies for SSc, with a focus on novel biologics and small molecules that specifically modulate key mechanisms.
Targeting inflammation, fibrosis, and vascular dysfunction in systemic sclerosis: the role of diet and complementary and alternative medicine
Patients with systemic sclerosis (SSc) often seek advice regarding diet including functional foods, and complementary and alternative medicine (CAM) as adjunctive therapies. This review summarizes existing literature regarding these approaches.
Updates in uricase therapy for gout
Urate-lowering therapy (ULT) plays a pivotal role in treating gout patients. Unfortunately, some patients receiving oral ULT fail to achieve the target serum urate levels of < 6.8 mg/dl, the solubility level of uric acid. Exogenous uricases, considered "enzyme replacement therapy," are a therapeutic option for patients with uncontrolled gout in whom oral ULT has not been efficacious, is not tolerated, or is contraindicated, in some due to underlying comorbidities. Currently, two uricases are available: pegloticase and rasburicase. Pegloticase is indicated for treating uncontrolled gout, while rasburicase is used to prevent tumor lysis syndrome.
Gouty inflammation: genetic mechanisms towards flare therapy
This review presents evidence for pathways that have genetic underpinnings in gout that should be prioritized for further study and therapeutic development.
A comprehensive review of systemic sclerosis-primary biliary cholangitis overlap: emerging evidence for a distinct clinical subtype
To synthesize current knowledge on the genetic, immunopathogenic, and clinical presentations of systemic sclerosis (SSc) and primary biliary cholangitis (PBC) with a focus on their co-occurrence as a clinically relevant overlap syndrome. This narrative review summarizes preclinical and clinical studies addressing SSc-PBC overlap.
Decoding vascular dysfunction in systemic sclerosis: from endothelial damage to clinical implications
This review explores the evolving understanding of vascular dysfunction in systemic sclerosis (SSc), from early endothelial injury to clinical manifestations and emerging therapeutic strategies.
Fueling fibrosis: metabolic dysregulation in systemic sclerosis
This review examines how metabolic reprogramming drives fibrosis and immune dysregulation in systemic sclerosis (SSc), emphasizing the role of nutrient-sensing and energy pathways in disease progression.
Neuropsychiatric systemic lupus erythematosus - current and novel treatments
Here, we provide a broad overview of the current treatment landscape of neuropsychiatric systemic lupus erythematosus (NPSLE) focusing on diffuse central nervous system manifestations and potential new treatments based on studies of murine models and neuroimaging studies of patients.
Pathogenesis of skin damage in lupus: recent advances and future directions
Lupus erythematosus (LE) encompasses a spectrum of autoimmune diseases with significant heterogeneity, ranging from cutaneous lupus erythematosus (CLE), confined to the skin, to systemic lupus erythematosus (SLE), which affects multiple internal organs. The underlying pathogenesis of lupus skin lesions and the heterogeneity among various subtypes remain elusive and require further investigation. This review synthesizes recent progress in elucidating the mechanisms of lupus skin injury, providing novel perspectives on diagnosis and therapeutic strategies, while also outlining promising avenues for future investigation.
Pathophysiology of glomerulonephritis in autoimmune diseases
Autoimmune glomerulonephritis (GN) emerges when self-reactive humoral and cellular immunity converge in the kidney. Combined immunofluorescence and electron microscopy aids in classifying GN; however, more stratification strategies are required for personalized therapy. We aimed to review biopsy-anchored clinicopathologic classification and pathophysiology of GN-associated disorders based on immunofluorescence and electron microscopy. Additionally, we sought to integrate mechanistic insights from multiomics and spatial profiling that resolve the composition and spatial organization of the cellular "neighborhoods" that drive injury and repair across IgA vasculitis/nephropathy, lupus nephritis, antiglomerular basement membrane disease, and antineutrophil cytoplasmic antibody-associated vasculitis.
Environmental and occupational contributors to autoimmune, inflammatory, and musculoskeletal rheumatic disease: a review of emerging evidence and clinical implications
Autoimmune and inflammatory rheumatic diseases as well as certain musculoskeletal diseases treated by rheumatologists result from a complex interplay between genetic predisposition and environmental factors.
