Pauci-immune glomerulonephritis in the native kidney presents with renal insufficiency, proteinuria, and hematuria, and is usually due to anti-neutrophil cytoplasmic antibodies. Rarely, kidney transplants can show this pattern as de novo disease. We performed a retrospective analysis in 10 cases of de novo pauci-immune glomerulonephritis. The mean time from transplant to diagnostic biopsy was 32 months (range, 4-96). All biopsies showed focal necrotizing or crescentic glomerulonephritis (mean 16% glomeruli, range 2-36%). Immunofluorescence and electron microscopy showed a pauci-immune pattern. No patients had evidence of systemic vasculitis. Anti-neutrophil cytoplasmic antibody results were available for 7 patients and were negative in all but one. Most patients had functioning grafts at one year after diagnosis. Two patients had repeat biopsies that showed continued active glomerulonephritis. We report the first clinicopathologic series of de novo pauci-immune glomerulonephritis which appears to be a unique pathologic entity that may occur early or late post-transplant and in our cohort is not associated with systemic vasculitis and usually not associated with anti-neutrophil cytoplasmic antibodies. The degree of crescent formation and renal impairment are milder than those of pauci-immune crescentic glomerulonephritis in the native kidney.
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http://dx.doi.org/10.1038/s41379-019-0355-0 | DOI Listing |
Diagnostics (Basel)
November 2024
Division of Nephrology, Hospital das Clínicas, University of São Paulo School of Medicine, São Paulo 05403-010, SP, Brazil.
Background: Since the introduction of the SLICC criteria in 2012, biopsy-proven lupus nephritis (LN) has been the only independent diagnostic criterion for systemic lupus erythematosus (SLE). This was reaffirmed by the EULAR/ACR in 2019, emphasizing the importance of renal biopsy in LN. However, the current classification lacks specific histopathological criteria for defining LN.
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November 2024
Department of Medicine, University of Illinois Chicago, Chicago, USA.
Pauci-immune glomerulonephritis is a rapidly progressive form of glomerulonephritis. It is distinguished from other rapidly progressive glomerular diseases by the lack of immune deposits seen on renal biopsy, hence the name "pauci-immune." We present a case of pauci-immune glomerulonephritis in a patient with a history of malignancy that was treated with chemotherapy.
View Article and Find Full Text PDFEur J Case Rep Intern Med
November 2024
CHU Marie-Curie Humani, Charleroi, Belgium.
Unlabelled: We report on a 67-year-old male patient admitted to the Internal Medicine department for fever, joint pain and exertional dyspnoea. Two months before his admission, the patient had been diagnosed with pauci-immune necrotising glomerulonephritis, for which he had been treated with rituximab and corticosteroids. Upon admission the patient was stable, but within a few hours he became unstable as liver failure and acute cytopaenia occurred.
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November 2024
Internal Medicine, University of Texas Medical Branch, Galveston, USA.
Rapidly progressive glomerulonephritis (RPGN) is a severe type of nephritic syndrome that involves progressive loss of kidney function and can lead to significant morbidity and mortality. RPGN has many etiologies, of which pauci-immune crescenting glomerulonephritis (PICGN) is the most common; however, patients often present with nonspecific symptoms, which can lead to a delay in diagnosis and treatment. We describe one such case that was correctly identified only after multiple clinic and hospital encounters.
View Article and Find Full Text PDFIndian J Nephrol
June 2024
Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
The spectrum of kidney disease among human immunodeficiency virus (HIV) infected patients is extensive. We describe a young male who was recently detected with HIV infection and antineutrophil cytoplasmic antibody (ANCA) negative pauci-immune crescentic glomerulonephritis. The patient had no extrarenal vasculitis involvement.
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