This document discusses nephrotic syndrome and nephritic syndrome. It defines the two syndromes and identifies their clinical features and causes. It presents two patient case studies, discussing their lab results and diagnoses. Common glomerular diseases are described in detail, including their clinical presentations and pathophysiologies. Evaluation and management of glomerular diseases is also addressed.
This document discusses nephrotic syndrome and nephritic syndrome. It defines the two syndromes and identifies their clinical features and causes. It presents two patient case studies, discussing their lab results and diagnoses. Common glomerular diseases are described in detail, including their clinical presentations and pathophysiologies. Evaluation and management of glomerular diseases is also addressed.
This document discusses nephrotic syndrome and nephritic syndrome. It defines the two syndromes and identifies their clinical features and causes. It presents two patient case studies, discussing their lab results and diagnoses. Common glomerular diseases are described in detail, including their clinical presentations and pathophysiologies. Evaluation and management of glomerular diseases is also addressed.
This document discusses nephrotic syndrome and nephritic syndrome. It defines the two syndromes and identifies their clinical features and causes. It presents two patient case studies, discussing their lab results and diagnoses. Common glomerular diseases are described in detail, including their clinical presentations and pathophysiologies. Evaluation and management of glomerular diseases is also addressed.
Download as PPT, PDF, TXT or read online from Scribd
Download as ppt, pdf, or txt
You are on page 1/ 65
Nephrotic and Nephritic
Syndrome January 22, 2008 Pamela J. Fall, M.D. Section of Nephrology
Objectives
Define nephrotic syndrome and
glomerulonephritis Identify clinical features of both syndromes Identify causes of each syndrome Evaluation of glomerular disease Management of glomerular diseases
Case
A 65 year old Caucasian man presents
with several months of lower extremity edema. PMH is unremarkable. No medications. ROS is notable for fatigue and some weight loss. Appetite remains good. BP is 150/100 mm Hg, pulse 92, periorbital plaques, edema and guaiac +
Case
Lab: Cr 1.4 mg/dL, Hg 8 g/dL, MCV
70, cholesterol 450 mg/dL, albumin 2.0 g/dL UA with 4+ protein, no blood and bland sediment. 24 hour urine 6 g protein Ultrasound shows 11 cm kidneys bilaterally with increased echogenicity
presents with right flank pain and gross hematuria. PMH: 3-4 episodes of hematuria per year, 6 hospitalizations for pain control, multiple evaluations with no diagnosis.
Case
Exam: BP 140/95 mm Hg, pulse 78,
chest, abdomen, extremities are normal. He has no rash or arthritis. Lab: Cr 1.1 mg/dL, Hg 14 g/dl, UA shows numerous RBCs, no casts. 24 hr urine protein is 1.5 g. Ultrasound is normal.
Case
What is his diagnosis?
Lupus nephritis IgA Nephropathy Membranous nephropathy Membranoproliferative glomerulonephritis from hepatitis C Nephrolithiasis
in which proliferation and inflammation of the glomerulus is secondary to an immunologic mechanism. Presentation of GN varies from microscopic asymptomatic hematuria or proteinuria to acute nephritis, to rapidly progressive nephritis.
Mesangioproliferative glomerulonephritis Asians and Caucasians Rare in African-Americans Age 20-30 Males > Females Pathogenesis altered regulation of IgA
IgA Nephropathy
Clinical presentation
50-60% episodic gross hematuria (synpharyngitic)
30-40% persistent microscopic hematuria <5% acute glomerulonephritis ESRD 20-40% at 5-25 years
Uncommon over age 40 (< 10%) Symptoms develop 7 days to 12 weeks after the infection Low complement levels (C3 and CH50) Spontaneous recovery is the rule Hematuria can persist 6 months Proteinuria, mild can persist years
Alternative pathway of complement activation IgG and C3 found in glomeruli
Case
T.G. is a 47 year old white man who presents
with 3 weeks of malaise, anorexia, weight loss, cough and dark urine. PMH: mild asthma, nonsmoker Exam: BP 145/70, lungs with diffuse rhonchi, heart, abdomen normal, no rash, 2+ edema Lab: Cr 6.3 mg/dL, UA 3+ blood and protein, dysmorphic rbcs
Rapid decline in renal function Rare 2-4% of all glomerulonephritis Pathologic hallmark crescents Classified based on presence or absence of immune complexes
Rapidly Progressive Glomerulonephritis
Immune complex mediated:
Henoch-Schonlein purpura Cryoglobulinemia (often associated with hepatitis C) Lupus nephritis Acute postinfectious glomerulonephritis Bacterial endocarditis
Rapidly Progressive Glomerulonephritis
Direct antibody attack mediated
Anti-GBM disease/Goodpastures syndrome
Pauci-immune (ANCA associated)
Wegeners granulomatosis Microscopic polyarteritis
Anti-GBM Disease
Clinical presentation:
Bimodal age distribution (3rd and 6th
decades) 60-70% present with pulmonary hemorrhage Systemic symptoms - malaise, fatigue, anorexia, weight loss, arthralgias, myalgias Caucasians Rare in African-Americans
Anti-GBM Disease
Pathogenesis
Antibodies develop against 3 chain
type IV collagen in GBM Linear deposition of IgG along GBM Antibodies detected by ELISA ANCA found in ~30% of patients
Anti-GBM Disease
Outcome poor without therapy
Treatment
Corticosteroids alone insufficient
Cyclophosphamide Plasma exchange with albumin 14 days
Renal recovery rare if patients
present needing dialysis
Case
24 year old black female presents with
hypertension and edema. Two weeks prior she developed a cough, chest pain, nasal congestion and pain in her right ear and received a course of amoxicillin. One week prior she noted a rash on her face and chest and complained of some pain in her hands.
Case
PMH: unremarkable Exam: BP 180/126 mm Hg. She has periorbital edema, normal oropharynx. Heart, lungs and abdomen are normal. She has lower extremity edema and an erythematous maculopapular rash over her chest.
Poststreptococcal glomerulonephritis IgA nephropathy Lupus nephritis Membranous nephropathy Wegeners granulomatosis
Systemic Lupus Erythematosis
Complex multisystem autoimmune
disease 11 criteria 4 present for diagnosis Kidney is most common organ involved (50-75%) Females > Males African-Americans have higher rates of lupus nephritis and worse renal survival
Lupus Nephritis
Clinical spectrum
Pathogenesis of renal involvement
Mild urinary abnormalities
Acute and chronic kidney failure Usually develops within 3 years Histone-DNA complex planted antigen Anti-dsDNA antibodies eluted from nephritic kidneys
WHO recognizes 6 classes
Classification of Lupus Nephritis
Class I normal Class II variable mesangial hypercellularity and immune deposits Class III focal proliferative glomerulonephritis Class IV diffuse proliferative glomerulonephritis Class V membranous nephropathy Class VI chronic glomerulosclerosis
Renal diseases IgA nephropathy Idiopathic rapidly progressive glomerulonephritis
Anti-GBM disease IC disease
Summary
Glomerular disease is an important
cause of CKD and ESRD. Patients can present with a variety of clinical syndromes. A good history, exam and certain lab tests in conjunction with renal biopsy can often lead to a diagnosis.
