Clinical outcomes of childhood lupus nephritis: a single center's experience

Byong Sop Lee1, Hee Yeon Cho, Eo Jin Kim

  • 1Department of Pediatrics, Seoul National University Children's Hospital, 28 Yongon-dong, Chongno-gu, Seoul, 110-744, South Korea.

Insights

Pediatric lupus nephritis (LN) patients often respond to initial treatment, but flares and progression to chronic renal failure (CRF) remain significant concerns. Male gender, hypertension, and lack of remission are key predictors of CRF in class IV LN.

Area of Science:

  • Pediatric Nephrology
  • Rheumatology
  • Immunology

Background:

  • Lupus nephritis (LN) is a severe complication of childhood-onset systemic lupus erythematosus.
  • Long-term outcomes and prognostic factors in pediatric LN require further elucidation.

Purpose of the Study:

  • To retrospectively analyze the clinical course, treatment responses, and long-term outcomes of pediatric patients with lupus nephritis.
  • To identify prognostic factors associated with the progression to chronic renal failure (CRF) in this population.

Main Methods:

  • Retrospective review of medical records of 77 children diagnosed with lupus nephritis between 1986 and 2005.
  • Analysis of initial biopsy findings (WHO classification), treatment regimens, clinical flares, and progression to CRF or end-stage renal disease (ESRD).
  • Multivariate analysis and Kaplan-Meier survival estimates were used to identify risk factors for CRF and assess patient survival.

Main Results:

  • Most patients (87.0%) initially responded to corticosteroids and/or immunosuppressants.
  • 44.8% of responders experienced subsequent flares (proteinuric or nephritic).
  • 16.9% progressed to CRF/ESRD, and 7.8% died. Male gender, initial hypertension, and absence of remission predicted CRF. Glomerulosclerosis (≥10%), nephritic flares, and antiphospholipid antibodies/syndrome were independent risk factors for CRF.

Conclusions:

  • Pediatric lupus nephritis has a significant risk of flares and progression to chronic renal failure despite initial treatment responses.
  • Male gender, hypertension, lack of remission, specific histological findings, and antiphospholipid syndrome are critical prognostic factors for CRF.
  • Cyclophosphamide pulse therapy did not show superiority over other combined immunosuppressive regimens for diffuse proliferative LN.

Related Concept Videos

Nephrotic Syndrome II : Assessment and Medical Management01:26

Nephrotic 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...
Acute Kidney Injury III: Clinical Manifestations01:29

Acute Kidney Injury III: Clinical Manifestations

Acute Kidney Injury (AKI) progresses through distinct clinical phases: the oliguric, diuretic, and recovery phases, each marked by unique manifestations and challenges.Oliguric Phase:The oliguric phase is the initial stage of AKI, typically lasting 10 to 14 days. This phase is marked by a significant reduction in urine output, usually less than 400 mL per day, indicating decreased kidney function. Fluid retention is a prominent feature, leading to symptoms such as edema, hypertension, and...
Nephrotic Syndrome I : Introduction01:24

Nephrotic 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...
Chronic Kidney Disease II: Clinical Manifestations01:24

Chronic Kidney Disease II: Clinical Manifestations

Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...
Nephrotic Syndrome III : Nursing Management01:24

Nephrotic 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...
Acute Kidney Injury I: Introduction01:22

Acute Kidney Injury I: Introduction

Introduction:Acute Kidney Injury (AKI) describes a swift decrease in kidney function occurring over hours to days, characterized by the kidneys' failure to remove waste products from the bloodstream. This leads to dangerous complications like metabolic acidosis, fluid overload, and electrolyte imbalances, such as hyperkalemia, which can cause life-threatening arrhythmias. AKI is common in both hospital and outpatient settings, often triggered by dehydration, sepsis, or exposure to nephrotoxic...