Sjögren's disease is a systemic autoimmune disorder characterised by hyperactivation of B-cells and cytokine production. The condition may evolve from an asymptomatic, indolent course, with glandular involvement, to several extra-glandular systemic manifestations up to lymphoma development. Recent efforts have been undertaken to identify patient phenotypes at risk of developing specific extraglandular manifestations in order to improve patient management.
View Article and Find Full Text PDFAlopecia universalis (AU), an advanced form of alopecia areata (AA), is a condition characterized by the complete loss of hair over the entire skin surface. Recent progress has significantly enhanced our understanding of the pathogenesis of AU. In particular, interferon-γ (IFN-γ) and interleukin (IL)-15 seem to play a pivotal role in the pathogenesis of the disease.
View Article and Find Full Text PDFThe treatment of patients with rheumatoid arthritis (RA) has dramatically changed in the past 30 years. Currently, numerous conventional, biologic, and targeted synthetic DMARDs have been licensed and used following recommendations provided by international and national scientific societies. However, the availability of biosimilars and the increasing necessity of savings impacted on the local/national prescription of these drugs.
View Article and Find Full Text PDFExpert Rev Clin Immunol
October 2024
Introduction: Primary Sjögren's syndrome (pSS) is an autoimmune disorder primarily affecting salivary and lacrimal glands, although about 40% of patients experience systemic complications. In this setting, the identification of patient phenotypes characterized by increased risk of extra-glandular involvement still represents an unmet need.
Areas Covered: The aim of this paper is to review the scientific evidence on the utility of salivary gland biopsies in pSS, emphasizing their role in defining prognosis.
The relationship between intestinal microbiota and arthritis has garnered significant attention, with emerging evidence suggesting a potential association between dysbiosis and various forms of inflammatory arthropathies. While observational studies have provided valuable insights into microbiota alterations in patients with arthritis, establishing causality remains challenging. Observational data, influenced by multiple confounders such as environmental factors, medication effects, and dietary habits, are insufficient to conclusively determine whether microbiota changes are somehow causally linked to arthritis.
View Article and Find Full Text PDFAdenosine deaminase 2 deficiency (DADA2) is a rare monogenic vasculopathy caused by loss-of-function homozygous or compound heterozygous mutations in ADA2, formerly CECR1 (cat eye syndrome chromosome region 1) gene. The DADA2 phenotype is widely heterogeneous, and patients may present with fever, weight loss, livedo reticularis/racemosa, digital ischemia, cutaneous ulceration, peripheral neuropathy, abdominal pain, bowel perforation, and portal or nephrogenic hypertension. More specific manifestations include early-onset ischemic or hemorrhagic stroke, mild immunodeficiency and hypogammaglobinemia, cytopenia, and vision disturbances.
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