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How We Treat ANCA-Associated Vasculitis: A Focus on the Maintenance Therapy.
J Clin Med
January 2025
University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-Net, ERN-Reconnet and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital, ASL Città di Torino and University of Torino, 10154 Turin, Italy.
Recent progress has notably improved outcomes for patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), namely granulomatosis with polyangiitis and microscopic polyangiitis. Since 2021, several international scientific societies have recommended rituximab (RTX) as the preferred primary treatment for maintaining remission in AAV patients. Decisions regarding retreatment with RTX are based on individual patient risk factors for disease flare-ups and the potential consequences of such flares.
View Article and Find Full Text PDFDistribution of Airway Findings in ANCA-Associated Vasculitis: A 20-Year Observational Analysis.
Diagnostics (Basel)
December 2024
Department of Internal Medicine, Division of Rheumatology, Mayo Clinic, Jacksonville, FL 32224, USA.
Pulmonary involvement is commonly observed in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), presenting with manifestations such as diffuse alveolar hemorrhage, inflammatory infiltrates, pulmonary nodules, and tracheobronchial disease. We aimed to identify distinct subgroups of tracheobronchial disease patterns in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) using latent class analysis (LCA), and to evaluate their clinical characteristics and outcomes. We conducted a retrospective cohort study using electronic medical records of patients aged >18 years diagnosed with AAV and tracheobronchial disease between 1 January 2002 and 6 September 2022.
View Article and Find Full Text PDFMaternal autoimmune systemic connective tissue disease and vasculitis and electrocardiographic findings in the offspring.
J Autoimmun
January 2025
Department of Cardiology, The Heart Centre, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark; Department of Cardiology, Copenhagen University Hospital, Herlev-Gentofte Hospital, Copenhagen, Denmark.
Introduction: Maternal autoimmune systemic connective tissue diseases (CTDs) and their related antibodies have been associated with adverse fetal outcomes, including complete heart block. In this study, we assessed the association between maternal CTD or vasculitis and neonatal electrocardiographic (ECG) parameters.
Methods: Our study population was drawn from the Copenhagen Baby Heart Study (CBHS), a prospective, population-based cohort study open to all neonates born in the Copenhagen area.
Impact of corticosteroids on improving lung compliance in a patient with pulmonary microscopic polyangiitis.
BMJ Case Rep
January 2025
Pulmonary and Critical Care Medicine, The University of Texas Health Science Center at Houston, Houston, Texas, USA.
Microscopic polyangiitis (MPA) is a small-vessel vasculitis characterised by systemic infiltration, with a primary focus on the renal and pulmonary systems. One of its more lethal pulmonary manifestations is diffuse alveolar haemorrhage (DAH), although the spectrum of lung pathology in MPA is vast and calls for immediate immunosuppressive therapy. Our case looks at an older woman initially presenting with MPA-induced rapid progressive glomerulonephritis.
View Article and Find Full Text PDFA single-center, single-arm, prospective, open-label, and comparative trial to evaluate the safety and tolerability profile of a 90-day oral L-arginine hydrochloride intervention for patients with amyotrophic lateral sclerosis.
Sci Rep
January 2025
Department of Clinical Neuroscience and Therapeutics, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, Hiroshima, 734-8551, Japan.
Weight loss, a key indicator of malnutrition in amyotrophic lateral sclerosis (ALS) patients, negatively impacts patient prognosis. However, effective nutritional interventions have not been adequately established. Research in ALS model mice has shown that L-arginine can prolong survival; however, no human intervention studies have been conducted.
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