Meningeal involvement is an infrequent manifestation of Wegener's granulomatosis. Clinical manifestations can be headache with high protein level in the cerebrospinal fluid and an enhanced MRI signal of granulomatous thickening of the duramater in the brain. We report a 57 year-old male with Wegener granulomatosis with onset manifestations of asymptomatic granulomatous meningitis, upper respiratory tract, ears and orbits involvement. He progressively developed ANCA positive multiple mononeuritis and crescentic glomerulonephritis. The diagnostic confirmation of Wegener's granulomatosis based on a positive ANCA test and on the evidence of systemic disease (crescentic glomerulonephritis and involvement of the upper respiratory tract, ears, orbits, peripheral nerves and duramater) allowed a prompt initiation of aggressive immunosuppressive treatment with systemic cyclophosphamide and high - dosis corticosteroids. The patient entered into a sustained clinical remission with mild residual neurosensorial hearing loss and renal failure.
Download full-text PDF |
Source |
---|
BMC Nephrol
January 2025
Renal Department and Nephrology Institute, Sichuan Provincial People's Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, 610072, China.
Background: The factors influencing diffuse crescentic glomerulonephritis renal survival and prognosis remain uncertain. Additionally, there's no literature on the clinical outcomes of IgA nephropathy, lupus nephritis, and IgA vasculitis nephritis in type II patients.
Methods: This study retrospectively examined 107 patients diagnosed with diffuse crescentic glomerulonephritis through biopsy.
CEN Case Rep
January 2025
Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
A 69-year-old Japanese man developed abdominal pain, purpura, proteinuria, and hematuria while receiving treatment for pulmonary tuberculosis. A skin biopsy revealed IgA-positive leukocytoclastic vasculitis, and a renal biopsy showed IgA-positive mesangial proliferative glomerulonephritis with crescent formation. Based on these findings, we diagnosed IgA vasculitis with nephritis (IgAVN) and initiated treatment.
View Article and Find Full Text PDFClin Nephrol Case Stud
December 2024
Nephrology Center and the Okinaka Memorial Institute for Medical Research.
A 47-year-old woman with a 12-year history of anemia and high C-reactive protein (CRP) levels was admitted to our hospital with worsening fatigue and night sweats. She had high levels of immunoglobulin G (IgG; 4182 mg/dL), IgA (630.6 mg/dL), and CRP (7.
View Article and Find Full Text PDFFront Immunol
January 2025
Department of Rheumatology, University of Texas Southwestern Medical Center, Dallas, TX, United States.
Background: Both intrinsic renal cells and immune cells contribute to driving renal inflammation and damage. However, the respective roles of intrinsic renal cells and immune cells in crescentic glomerulonephritis, and the key molecular factors driving pathogenesis are still unclear.
Methods: The roles of intrinsic renal cells and renal infiltrating immune cells in crescent formation were explored using renal transplantation after experimental anti-GBM disease induction in 129x1/svJ and C57BL/6J mice.
Zhongguo Dang Dai Er Ke Za Zhi
December 2024
Department of Pediatrics, First Affiliated Hospital of Henan University of Chinese Medicine, Zhengzhou 450000, China.
Objectives: To investigate the impact of the different proportions of crescent formation on clinical manifestations and pathological features in children with immunoglobulin A vasculitis with nephritis (IgAVN).
Methods: The children with IgAVN were divided into no-crescent group (75 children), ≤25% crescent group (156 children), and >25% crescent group (33 children).
Results: Compared with the no-crescent group, the other two groups had significant increases in 24-hour urinary protein, urinary immunoglobulin G (IgG)/creatinine ratio, urine red blood cell count, fibrinogen, and neutrophil-lymphocyte ratio, a significant reduction in serum IgG, and a significantly higher proportion of children with low albumin and hypercoagulability, pathological grade III+IV or diffuse mesangial proliferation (<0.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!