Related Experiment Video
Updated: Jun 12, 2026
06:56
Isolation of Double Negative αβ T Cells from the Kidney
Published on: May 16, 2014
ANCA negative pauci-immune glomerulonephritis with systemic involvement
K Sampathkumar1, M Ramakrishnan, A K Sah
1Department of Nephrology, Meenakshi Mission Hospital and Research Centre, Madurai - 625 107, India.
Indian Journal of Nephrology
|June 11, 2010
Summary
ANCA-negative pauci-immune glomerulonephritis often presents with systemic symptoms and severe kidney issues. Prompt immunosuppressive therapy can lead to significant renal recovery in most patients.
Area of Science:
- Nephrology
- Rheumatology
- Immunology
Background:
- Systemic vasculitides (SV) are diverse diseases affecting multiple organ systems.
- Anti-neutrophil cytoplasmic antibody (ANCA) testing is crucial for diagnosing SV.
- Pauci-immune glomerulonephritis (PIGN) can occur without ANCA positivity.
Observation:
- This study details four patients with ANCA-negative PIGN and systemic involvement.
- Patients experienced symptoms like fever, arthralgias, neurological issues, and severe renal failure.
- Renal biopsies showed focal proliferative glomerulonephritis with crescents and chronic interstitial changes.
Findings:
- All patients had nephrotic proteinuria and hematuria; 66% required dialysis.
- Mean Birmingham Vasculitis Score was 23, indicating significant systemic inflammation.
- Immunosuppressive therapy (steroids, cyclophosphamide, MMF) was effective in 75% of patients.
Implications:
- ANCA-negative PIGN frequently involves multiple organ systems at diagnosis.
- Renal biopsy findings are key for diagnosis and prognosis.
- Aggressive immunosuppression offers substantial renal recovery in most cases.
Related Concept Videos
01:24Nephrotic Syndrome I : Introduction
Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of fluid...
01:28Diabetic Nephropathy
Definition Diabetic nephropathy is a chronic kidney complication that results from prolonged hyperglycemia.Prevalence It is the most common cause of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide, affecting up to half of individuals with diabetes.Pathophysiology • Sustained hyperglycemia triggers multiple hemodynamic and metabolic changes in the kidney. • Early in the disease, increased renal blood flow and glomerular hyperfiltration occur due to afferent arteriolar...
01:26Nephrotic Syndrome II : Assessment and Medical Management
IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document any history...
01:24Nephrotic Syndrome III : Nursing Management
Nursing management for nephrotic syndrome adapts as the disease progresses, with strategies evolving to address advancing symptoms and complications.Early-Stage Management In the early stages, nursing interventions for nephrotic syndrome resemble those used in managing acute glomerulonephritis, focusing on symptom monitoring, fluid balance, and managing mild to moderate edema.Vital Signs: Regularly monitor blood pressure, pulse, respiratory rate, and temperature to promptly identify...
01:25Endocarditis II: Clinical Features of Infective Endocarditis
Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...