Updated: 10/13/2018

Nephritic Syndrome

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Snapshot
  • A 14-year-old boy presents with hematuria, problems with vision, and deafness. Approximately 2 weeks prior to symptom development, he recovered from a viral upper respiratory tract infection. Family history is significant for deafness and early initiation of lisinopril in his father. His temperature is 99°F (37.2°C), blood pressure is 140/90 mmHg, pulse is 75/min, and respirations are 18/min. Physical examination is signifcant for anterior lenticonus and sensorineural hearing loss. Urinalysis demonstrates dysmorphic red blood cells and red blood cell casts. (Alport syndrome)
Introduction
  • Clinical definition
    • renal disease secondary to an inflammatory process injuring the glomerulus
      • this results in damage involving the
        • basement membrane
        • capillary endothelium
        • mesangium
  • Presentation
    • symptoms
      • hypertension
      • hematuria
      • oliguria
      • headache
    • physical exam
      • edema
        • can be peripheral and/or periorbital
  • Diagnosis
    • studies
      • complete blood cell count
        • anemia may be noted
      • azotemia
      • complement levels
        • C3, C4, and CH50 should be obtained
      • urinalysis
        • dysmorphic red blood cells (RBCs)
          • suggests hematuria is of glomerular origin
        • RBC casts
        • subnephrotic range proteinuria (< 3.5 g/day)
          • if the nephritic syndrome is severe enough it can lead to nephrotic range proteinuria (> 3.5 g/day)
    • renal biopsy
      • may be necessary to arrive to a definitive diagnosis and to determine prognosis
 
Nephritic Syndrome
Type
Pathophysiology
Renal Biopsy
Diagnostic Studies and Treatment
Acute poststreptococcal glomerulonephritis
  • Glomerulonephritis secondary to nephritogenic strains of streptococcus
    • type III hypersensitivity reaction
  • Light microscopy
    • glomerular hypercellularity
      • e.g., polymorphonuclear leukocytes 
  • Immunofluorescence
    • diffuse granular pattern on glomerular capillary walls and mesangium
      • deposition of IgG, IgM, and C3
  • Electron microscopy  
    • electron-dense, glomerular subepithelial immune-complex deposits ("humps")
  • Streptococcus titers and serologies are positive
  • ↓ serum C3 levels
  • Typically self-resolves
Rapidly progressive glomerulonephritis
  •  Goodpasture syndrome 
    • type II hypersensitivity
    • anti-GBM antibodies against α3-chain of collagen type IV
      • antibodies to the alveolar basement membrane result in hemoptysis and lung disease
  • Pauci-immune processes
    • granulomatosis with polyangiitis
    • microscopic polyangiitis
  • Light microscopy and immunofluorescence
    • crescent-shaped deposition of fibrin, C3b, glomerular parietal cells, monocytes, and macrophages
  • Granulomatosis with polyangiitis
    • PR3-ANCA/c-ANCA positive 
  • Microscopic polyangiitis
    • MPO-ANCA/p-ANCA
  • Corticosteroids and cyclophosphamide
IgA nephropathy (Berger's disease)
  • IgA immune-complex deposition in glomerular mesangial cells that results in its proliferation
    • patients present with hematuria and upper respiratory tract or gastrointestinal infection
  • Light microscopy
    • mesangial proliferation
  • Immunofluorescence
    • IgA immune-complex deposition in the mesangium
  • ACE inhibitor or ARB for proteinuria and hypertension
Alport syndrome
  • Collagen type IV mutation that results in an abnormal basement membrane
    • more commonly an X-linked dominant disorder 
    • characterized by
      • renal involvement
      • ocular involvement
      • sensorineural hearing loss
  • Electron microscopy
    • glomerular basement membrane lamellation
    • "basket weave" appearance
  • No curative treatment
Membranoproliferative glomerulonephritis (MPGN)
  • Immune-complex and/or complement protein deposition in the mesangium and subendothelium of the capillaries
    • this results in proliferation of the mesangium and remodeling of the capillary wall
    • may be secondary to
      • hepatitis C virus
      • hepatitis B virus
      • C3 nephritic factor
        • MPGN type II
  • Light microscopy
    • mesangial proliferation and thickening of the capillary wall
  • Immunofluorescence
    • "tram-track" appearance
  • ↓ serum C3 and C4 levels
  • Treat underlying disease in secondary causes
    • e.g., control of hepatitis C virus infection
Diffuse proliferative glomerulonephritis (DPGN)
  • > 50% of glomeruli (thus diffuse) demonstrates an inflammatory process on histology
  • Common in patients with systemic inflammatory diseases (e.g., systemic lupus erythematosus)
    • can be secondary to membranoproliferative glomerulonephritis
  • Light microscopy
    • capillary "wire-looping"
  • Electron microscopy
    • immune complex deposition affecting the
      • subendothelial region
      • maybe intramembranous
  • Immunofluorescence
    • granular appearance
  • Can lead to death in patients with systemic lupus erythematosus
  • Aggressively treat with steroids
 
 

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Questions (16)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
Calculator

(M1.RL.85) Three weeks after recovering from pharyngitis, a nine-year-old girl presents with periorbital edema and dark brown urine. An image of the kidney biopsy is shown in Figure A. What is responsible for these changes? Review Topic

QID: 101039
FIGURES:
1

Glomerular basement membrane lamellation

9%

(1/11)

2

IgA deposition and subsequent mesangial proliferation

9%

(1/11)

3

Neutrophil infiltration in response to immune complex deposition along the glomerulus basement membrane and mesangium

82%

(9/11)

4

Loss of negative charge on the glomerular basement membrane

0%

(0/11)

5

Widespread intermembraneous deposits due to an autoantibody against C3 convertase

0%

(0/11)

M1

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PREFERRED RESPONSE 3
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(M1.RL.32) A 9-year-old Caucasian girl presents to your office with hematuria. An electron micrograph of her renal biopsy is shown below in Figure A. Which of the following is the most likely composition of the structures marked by the white arrows? Review Topic

QID: 100986
FIGURES:
1

Albumin

2%

(2/80)

2

Non-enzymatic glycosylation

1%

(1/80)

3

IgA

10%

(8/80)

4

IgG

8%

(6/80)

5

IgG, IgM, C3

75%

(60/80)

M1

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PREFERRED RESPONSE 5
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(M1.RL.4671) A 62-year-old man presents to his primary care doctor complaining of recent-onset hemoptysis. He has not had any fevers, night sweats, or weight loss. He recently traveled to Italy with his wife. He has a 5 pack-year smoking history. On review of systems, he reports that his urine has been “red-tinged” for several months. Urinalysis reveals the findings shown in Figure A. He is referred to a nephrologist and undergoes a renal biopsy. Immunofluorescence staining of the biopsy is shown in Figure B. Which of the following is the most likely underlying pathogenesis of his disease? Review Topic

QID: 107162
FIGURES:
1

Type III hypersensitivity reaction

12%

(8/64)

2

Antibodies to collagen type IV

69%

(44/64)

3

Presence of C-ANCA

16%

(10/64)

4

Mycobacterial infection

0%

(0/64)

5

Metastatic malignancy

0%

(0/64)

M1

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PREFERRED RESPONSE 2
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(M1.RL.67) A 21-year-old male presents to your office with hematuria 3 days after the onset of a productive cough and fever. Following renal biopsy, immunofluorescence shows granular IgA deposits in the glomerular mesangium. Which of the following do you suspect in this patient? Review Topic

QID: 101021
1

Lipoid nephrosis

7%

(1/15)

2

Berger’s disease

93%

(14/15)

3

Poststreptococcal glomerulonephritis

0%

(0/15)

4

Systemic lupus erythematosus

0%

(0/15)

5

HIV infection

0%

(0/15)

M1

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PREFERRED RESPONSE 2

(M1.RL.11) Multiple patients present to your office with hematuria following an outbreak of Group A Streptococcus. Biopsy reveals that all of the patients have the same disease, characterized by large, hypercellular glomeruli with neutrophil infiltration. Which patient has the best prognosis? Review Topic

QID: 100965
1

65-year-old nulliparous woman

1%

(1/95)

2

50-year-old man with a history of strep infection

6%

(6/95)

3

8-year-old boy who undergoes no treatment

74%

(70/95)

4

38-year-old man with sickle cell trait

3%

(3/95)

5

18-year-old man treated with corticosteroids

12%

(11/95)

M1

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PREFERRED RESPONSE 3

(M1.RL.35) A 6-year-old boy presents to your office with hematuria. Two weeks ago the patient had symptoms of a sore throat and fever. Although physical exam is unremarkable, laboratory results show a decreased serum C3 level and an elevated anti-DNAse B titer. Which of the following would you most expect to see on renal biopsy? Review Topic

QID: 100989
1

Large, hypercellular glomeruli on light microscopy

76%

(13/17)

2

Polyclonal IgA deposition on immunofluorescence

6%

(1/17)

3

Immune complex deposits with a "spike and dome" appearance on electron microscopy

0%

(0/17)

4

Wirelooping and hyaline thrombi on light microscopy

0%

(0/17)

5

Antibodies to GBM resulting in a linear immunofluorescence pattern

6%

(1/17)

M1

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PREFERRED RESPONSE 1

(M1.RL.86) A 37-year-old man presents with significant hematuria and hemoptysis. The results of the immunoflouresence are shown in Figure A. What pathologic changes would be expected under light microscopy? Review Topic

QID: 101040
FIGURES:
1

"Wire looping" of the capillaries

6%

(1/16)

2

Hypercellular glomeruli

0%

(0/16)

3

Crescentic glomerulonephritis

88%

(14/16)

4

Focal proliferative glomerulonephritis

6%

(1/16)

5

Normal glomeruli

0%

(0/16)

M1

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PREFERRED RESPONSE 3

(M1.RL.73) A 25-year-old male visits his primary care physician with complaints of hemoptysis and dysuria. Serum blood urea nitrogen and creatinine are elevated, blood pressure is 160/100 mm Hg, and urinalysis shows hematuria and RBC casts. A 24-hour urine excretion yields 1 gm/day protein. A kidney biopsy is obtained, and immunofluorescence shows linear IgG staining in the glomeruli. Which of the following antibodies is likely pathogenic for this patient’s disease? Review Topic

QID: 101027
1

Anti-DNA antibody

0%

(0/13)

2

Anti-neutrophil cytoplasmic antibody (C-ANCA)

15%

(2/13)

3

Anti-neutrophil perinuclear antibody (P-ANCA)

0%

(0/13)

4

Anti-glomerular basement membrane antibody (Anti-GBM)

69%

(9/13)

5

Anti-phospholipid antibody

0%

(0/13)

M1

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PREFERRED RESPONSE 4
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