Nephritic syndromes Flashcards

1
Q

IgA nephropathy (Maladie de Berger): pathology and causes

A
  • abnormal IgA forms, -> IgA-antibody complex formation -> deposits in kidneys → kidney damage

Associated with gastrointestinal (GI)/respiratory tract infections

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2
Q

IgA nephropathy (Maladie de Berger): RF

A
  • Most common nephropathy worldwide
  • usually presents in childhood
  • Highest prevalence in people of East Asian/European ancestry
  • Family history of chronic nephritis, alcohol consumption, recurrent infections
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3
Q

IgA nephropathy (Maladie de Berger): Signs and symptoms

A
  • Episodic hematuria
    Sometimes accompanying upper respiratory tract infections
  • Asymptomatic microscopic hematuria, with subnephrotic proteinuria
  • Classic nephrotic syndrome/kidney injury (minority)
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4
Q

IgA nephropathy (Maladie de Berger): Diagnosis

A

Perform urinalysis and a renal biopsy

Urinalysis -> hematuria
- RBCs, RBC casts

Renal biopsy:

  • Light microscopy: Mesangial proliferation, immune complexes deposited in mesangium
  • Electron microscopy: Immune complexes deposited in mesangium
  • Immunofluorescence: Mesangial IgA deposits, +/- IgA, +/- IgM
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5
Q

IgA nephropathy (Maladie de Berger): treatment

A
  • Prednisone (Oral/parenteral glucocorticoid intermediate actions)
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6
Q

Rapidly Progressive Glomerulonephritis: pathology and causes

A
  • Inflammation of kidney’s glomeruli → crescent-shaped proliferation of cells in Bowman’s capsule → renal failure within weeks/months
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7
Q

RPG: Primary type

A
  • Idiopathic
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8
Q

RPG: Secondary type

A

Type I: anti-GBM antibodies
- Goodpasture syndrome

Type II: immune complexes

  • Poststreptococcal glomerulonephritis
  • systemic lupus erythematosus
  • IgA nephropathy
  • Henoch-Schonlein purpura

Type III: anti-neutrophilic cytoplasmic antibodies (ANCA)

  • Cytoplasmic ANCA (C-ANCA): Wegener’s granulomatosis
  • Perinuclear ANCA (P-ANCA): microscopic polyangiitis, Churg-Strauss syndrome
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9
Q

RPG: Signs and symptoms

A

Nephritic syndrome:

  • Hematuria,
  • oliguria,
  • edema,
  • hypertension
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10
Q

RPG: Complications

A

-> rapid progressive to acute renal failure if untreated

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11
Q

RPG: Diagnosis

A

Perform a kidney biopsy + immunofluorescence

Urinalysis: -> hematuria

Kidney biopsy
- Light microscopy: crescent-shaped glomeruli

Immunofluorescence

  • Type I: linear, antibodies bind to collagen of glomerular basement membrane
  • Type II: granular, immune complex deposition in subendothelium
  • Type III: negative (pauci-immune), associated with ANCAs in blood
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12
Q

RPG: treatment

A

Pulse methylprednisolone, then:

  • prednisone
  • cyclophosphamide
  • rituximab
  • plasmapheresis

In case of renal failure -> dialysis + kidney transplant

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13
Q

Goodpasture syndrome: pathology and cause

A
  • anti-GBM antibody disease -> damage to the basement membrane in lungs + kidneys
    Mostly composed of Type IV collagen
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14
Q

Goodpasture syndrome: RF

A
  • Bimodal distribution: male between 20-30, female between 60-70
  • genetic: predisposition for genes that code for HLA-DR15
  • Environmental: infection, smoking, oxidative stress, hydrocarbon-based solvents
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15
Q

Goodpasture syndrome: Signs and symptoms

A

Pulmonary manifestations usually occur before renal ones. Minority (20–40%) with only renal manifestations

  • Damaged lung alveoli → cough, hemoptysis, dyspnea
  • Kidney filtration problems -> hematuria, proteinuria), nephritic syndrome
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16
Q

Goodpasture syndrome: complications

A
  • Chronic renal failure

will require dialysis/kidney transplant

17
Q

Goodpasture syndrome: Diagnosis

A

Perform a renal biopsy

  • Light microscopy: Crescentic glomerulonephritis
  • Electron microscopy: Diffuse thickening of glomerular basement membrane
  • Immunofl uorescence: Linear deposition along basement membrane
18
Q

Goodpasture syndrome: treatment

A
  • Prednisone
  • cyclophosphamide,
  • plasmapheresis to filter plasma/fluid of blood (reduces risk of chronic renal failure
19
Q

Post-streptococcal glomerulonephritis (PSGN): pathology and causes

A

Also called Acute proliferative Glomerulonephritis

  • Inflammation of glomeruli, complication of bacterial infection
  • Commonly arises several weeks after group A beta-hemolytic streptococcus infection
  • IgG/IgM antibodies bind to bacterial antigens, form immune complexes →complexes travel through bloodstream to glomerulus, deposit in glomerular basement membrane
20
Q

PSGN: causes

A
  • Group A beta-hemolytic streptococcus infection
21
Q

PSGN: RF

A
  • Most commonly in children, male

Six weeks after impetigo, 1–2 weeks after throat infection

22
Q

PSGN: symptoms

A
  • Nephritic syndrome: hematuria, oliguria, edema, hypertension
  • Fever, headache, malaise, anorexia, nausea
23
Q

PSGN: complications

A

Rapidly progressive glomerulonephritis -> renal failure

24
Q

PSGN: Diagnosis

A

Lab results

  • proteinuria + hematuria
  • Antibodies against group A streptococcus (e.g. anti-DNase B antibodies, anti-Streptolysin O antibody)
  • decreased complement levels

Renal biopsy

  • Light microscopy: Mesangial proliferation → hypercellular glomerulus
  • Electron microscopy: Subepithelial deposits of immune complexes, “humps”
  • Immuno fluorescence“Starry sky -> granular deposition of IgG, complement in basement membrane, mesangium
25
Q

PSGN: treatment

A
  • usually supportive
26
Q

Lupus nephritis: pathology and causes

A
  • Inflammation of kidney due to systemic lupus erythematosus
  • Focal (nephrons in one area)/diffuse (all nephrons in both kidneys)
  • Caused by antinuclear antibodies ( anti-dsDNA): bind to nuclear antigens, form antigen-antibody complexes -> deposits in capillary walls, basement membrane, Bowman’s space -> type III hypersensitivity reaction
27
Q

Lupus nephritis: types

A

Class I: Minimal mesangial glomerulonephritis
Class II: Mesangial proliferative glomerulonephritis
Class III: Focal glomerulonephritis
Class IV: Diffuse proliferative nephritis
Class V: Membranous glomerulonephritis
Class VI: Advanced sclerosing lupus nephritis

28
Q

Lupus nephritis: signs and symptoms

A
  • Nephrotic, nephritic syndrome
  • hyperlipidemia (less protein more fat)
  • Nephritic syndrome:
    hematuria, hypertension, proteinuria -> hypoalbuminemia -> decreased oncotic pressure -> edema, oliguria
29
Q

Lupus nephritis: complications

A
  • Renal vein thrombosis
  • pulmonary embolism
  • rapidly progressive glomerulonephritis
30
Q

Lupus nephritis: diagnosis

A
Perform a kidney biopsy
- Microscopic presentation depends on class of lupus nephritis
31
Q

Lupus nephritis: T

A
  • Prednisone
  • mycophenolate
  • cyclophosphamide