Objective: To assess the use of mycophenolate mofetil (MMF) in the treatment of refractory primary angiitis of the CNS in childhood (cPACNS).
Methods: A retrospective chart review was performed in patients with cPACNS who were treated with MMF following failure of a combination of corticosteroids and another immunosuppressant.
Results: Three patients from two centres were included in this study. The age of onset of disease was 5, 6 and 9 years. All the patients improved when treated with MMF, such that the dose of corticosteroids could be weaned or stopped.
Conclusions: MMF should be considered for maintenance treatment in the management of patients with cPACNS refractory to the combination of corticosteroids and first-line immunosuppressive agents.
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http://dx.doi.org/10.1093/rheumatology/kep453 | DOI Listing |
Radiol Clin North Am
March 2025
Department of Pathology and Laboratory Medicine, University of Wisconsin, Madison, WI, USA. Electronic address:
Pulmonary hypertensive changes are commonly seen by the surgical pathologist, but the majority represents secondary changes due to some process extrinsic to the lung. Some primary, or idiopathic, vascular diseases result in unique pathologic changes including the plexiform lesion and venous hypertensive changes. Thromboembolic disease also shows unique pathologic features.
View Article and Find Full Text PDFBiomedicines
January 2025
Department of Gastroenterology and Hepatology, Kyorin University School of Medicine, Tokyo 181-0004, Japan.
Behçet's disease is an autoinflammatory disorder characterized by relapsing and remitting vasculitis that can manifest in various forms, including gastrointestinal Behçet's disease (GIBD). Its complications (e.g.
View Article and Find Full Text PDFBMC Rheumatol
January 2025
Department of Nephrology and Rheumatology, Aichi Medical University, 1-1 Karimata, Yazako, Nagakute, Aichi, 480-1195, Japan.
Background: Avacopan, an oral C5a receptor antagonist, demonstrated efficacy as an alternative to glucocorticoid therapy in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in the phase 3 ADVOCATE trial. However, limited real-world data exist on the outcomes and experiences associated with avacopan use for AAV in Japan.
Methods: We performed a single-centre retrospective analysis and evaluated 21 patients with newly diagnosed or relapsed AAV who received avacopan.
Front Immunol
January 2025
Department of Clinical Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Objectives: The feasibility of corticosteroid withdrawal (CW) for Takayasu arteritis (TAK) remains uncertain. Two autoantibodies (Abs) are identified against endothelial protein C receptor (EPCR) and scavenger receptor class B type 1 (SR-BI) in TAK, determining its three subgroups. This study aimed to evaluate CW using tocilizumab (TCZ) and its association with the Ab profile.
View Article and Find Full Text PDFJ Pak Med Assoc
January 2025
Department of Rheumatology, Shalamar Institute of Health Sciences, Lahore, Pakistan.
Primary Sjogren's syndrome (pSS) typically presents with Sicca symptoms xerostomia and xeropthalmia. This study highlights atypical presentations of Primary Sjogren's syndrome posing diagnostic and therapeutic challenges. Four female patients (median age 30 years, IQR = 15.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!