Renal-limited vasculitis (RLV) is a type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents with crescentic glomerulonephritis with no other organ involvement. Although several studies reported patients with crescentic glomerulonephritis who were dual positive for proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA or ANCA and anti-glomerular basement membrane (GBM) antibody, patients positive for all three antibodies, i.e., triple-positive patients, were rarely reported. We herein report the case of a male with pauci-immune type crescentic glomerulonephritis positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibody. Renal biopsy led to the definitive diagnosis of RLV with pauci-immune-type crescentic glomerulonephritis. Fluorescence immunostaining showed no linear deposition of IgG on GBM, indicating no involvement of anti-GBM associated diseases. Intensive therapy, including prednisolone, plasma exchange, and intravenous cyclophosphamide, was effective. We report the case of triple-positive patient with crescentic glomerulonephritis, who was successfully treated with glucocorticoid, plasma exchange, and cyclophosphamide, suggesting that treatment for RLV in the patient with serological triple antibodies positivity in the absence of linear IgG deposition could benefit from the combination therapy regimen for plasma exchange and primary induction of remission against microscopic polyangiitis.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5438812 | PMC |
http://dx.doi.org/10.1007/s13730-017-0248-3 | DOI Listing |
Front 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.
World J Nephrol
December 2024
Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi 74200, Pakistan.
Background: Primary immunoglobulin (Ig)-associated mesangiocapillary glomerulonephritis (Ig-MCGN) is an immune complex glomerulonephritis of unknown etiology. It is a common cause of chronic kidney disease in developing countries. There is limited data available on renal and patient outcomes of this disease from developing countries.
View Article and Find Full Text PDFKidney360
December 2024
Division of Nephrology and Hypertension, Department of Internal Medicine, Jikei University School of Medicine, Tokyo, Japan.
Background: Lupus nephritis (LN) is a major complication of systemic lupus erythematosus. Like other types of glomerulonephritis, podocyte injury has been observed in patients with LN. However, the association between podocyte injury and kidney prognosis in patients with LN has not been well elucidated.
View Article and Find Full Text PDFCEN Case Rep
December 2024
Division of Rheumatology, University of Missouri, Kansas City, MO, 64108, USA.
Granulomatosis with polyangiitis is an ANCA-associated vasculitis that involves small to medium-sized vessels. The extent of renal involvement varies, which is also associated with disease prognosis, with aggressive renal involvement having worse outcomes. Rapidly progressive glomerulonephritis with severe inflammatory features and extensive crescent formation can be challenging to treat.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!