Background: Erdheim-Chester disease (ECD) is a rare non-Langerhans cells histiocytosis associated with intense immune activation. In our clinical center, an ECD patient was treated with anakinra, IL1RA (interleukin1 receptor antagonist), resulting in clinical improvement and major decrease of pathological fatigue. The aim of the study was to evaluate changes in cytokine profile and shift of immune cells estimated by flow cytometric analysis of ECD patient before, during initial stages of anakinra treatment as well as after treatment ceased in comparison to healthy donors.
Methods: Singleplex reactions of 19 individual cytokines from serum of ECD patient were measured by FACS array. Flow cytometric analyses were performed on peripheral blood cells.
Results: The most striking result is substantial decrease of IL6 immediately after anakinra treatment started suggesting a major role of IL1 pathway in ECD pathophysiology. As for flow cytometric analysis, increased number of CD16+ monocytes before treatment is a new finding.
Conclusion: Our results suggest that IL6 may be a marker of early treatment response of ECD patients treated with anakinra.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.14735/amko2014276 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Background and Clinical Features of a Unique and Mysterious Autoinflammatory Disease, Schnitzler Syndrome.
Int J Mol Sci
January 2025
Immunology Division, Department of Internal Medicine and Hematology, Semmelweis University, 1088 Budapest, Hungary.
Schnitzler syndrome is a unique autoinflammatory disease, of which 747 cases have been described worldwide to date. The main features of the syndrome are a triad of recurrent urticaria, monoclonal IgM gammopathy, systemic inflammation associated with recurrent fever, joint and bone pain, and atypical bone remodeling (osteosclerosis). The abnormal activation of the NLRP3 inflammasome produces IL-1, which drives the disease pathology, but it also involves IL-6 and IL-18.
View Article and Find Full Text PDFTrends in drug repurposing: Advancing cardiovascular disease management in geriatric populations.
Curr Res Transl Med
January 2025
Department of Diabetology and Endocrinology, Hindu Mission Hospital, Tambaram, Chennai, 600045, Tamil Nadu, India.
Drug repurposing is a promising strategy for managing cardiovascular disease (CVD) in geriatric populations, offering efficient and cost-effective solutions. CVDs are prevalent across all age groups, with a significant increase in prevalence among geriatric populations. The middle-age period (40-65 years) is critical due to factors like obesity, sedentary lifestyle, and psychosocial stress.
View Article and Find Full Text PDFOff-label use of biologics in urticarial vasculitis: a European retrospective cohort study.
Rheumatology (Oxford)
January 2025
Department of internal medicine, CHU Cochin, AP-HP, Paris Cité University, Paris, F-75006, France.
Objectives: Urticarial vasculitis (UV) is characterized by atypical urticarial lesions and leukocytoclastic vasculitis, sometimes with extracutaneous manifestations. First-line treatment is based on colchicine, hydroxychloroquine, dapsone or low-dose glucocorticoids. In refractory forms, the use of biologics has been anecdotally described as potentially effective.
View Article and Find Full Text PDFDuration of Disease and Long-Term Outcomes in Patients With Difficult-To-Treat Recurrent Pericarditis: A Chronic Condition Treated With Nonsteroidal Anti-Inflammatory Drugs, Colchicine, Corticosteroids, and Anti-Interleukin-1 Agents.
ACR Open Rheumatol
January 2025
University of Udine and University Hospital Santa Maria della Misericordia, Udine, Italy.
Objective: We aimed to investigate the remission rate and disease duration in idiopathic or post-cardiac injury pericarditis and risk factors for disease duration and anti-interleukin-1 (IL-1) agent discontinuation.
Methods: This was a multicenter, longitudinal, observational study including 370 patients (51.4% female).
Management of chimeric antigen receptor T-cell-related toxicity of a patient affected by cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome, followed by an intestinal perforation: a case report.
J Med Case Rep
January 2025
Director of Hospital Pharmacy, Santa Croce e Carle Hospital, Cuneo, Italy.
Background: Mantle cell lymphoma is a diverse B-cell lymphoma with varying clinical behaviors. Treating relapsed or refractory mantle cell lymphoma is challenging, with Bruton's tyrosine kinase inhibitors proving effective but not curative. Post-Bruton's tyrosine kinase inhibitor failure, the prognosis remains unfavorable.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!