Renal Disorders II Flashcards
Features of nephritic syndrome
- hematuria
- microscopic analysis of urine will show erythrocytes
- may also have kidney injury (increased creatinine and BUN) in which case this is call acute nephritic syndrome
Diseases that cause asymptomatic isolated hematuria
- IgA nephropathy
- alport syndrome
IgA nephropathy facts
- most common type of primary glomerulonephritis worldwide
- affects children and young adults
- quite common in young adults (non african americans)
- recurrent hematuria
Histologic feature of IgA nephropathy and immunofluorescence
- mesangial proliferation: mesangial hypercellularity of > 3 cells per mesangial region
- granular mesangial pattern of staining for IgA
IgA nephropathy prognosis and treatment
- most patients have good prognosis
- proteinuria is a predictor of progressive disease
- no specific treatment available: in progressive cases steroids and immunosuppressive agents may be tried
Henoch Schonlein Purpura (HSP)
- vasculitic variant of IgA nephropathy
- usually seen in CHILDREN
- features of vasculitis, arthritis, abdominal pain, rashes
Kidney and Skin biopsy findings in HSP
- kidney: IgA in the mesangium
- skin: IgA in sub dermal vessels
Alport syndrome facts
- defect in glomerular basement membrane due to mutation in alpha-5 chain of collagen type IV
- X linked dominant inheritance
- COL4A5 gene (Xq22)
- affects males, females are carriers
Alport syndrome features
- presents with microscopic hematuria
- sensorineural deafness
- progresses to chronic kidney disease
- proteinuria is a sign of progression
Alport syndrome key lesions
- GBM: thickening, splitting, and lamination
- “basket-weave” pattern
Isolated hematuria is caused by 2 diseases
- IgA nephropathy
- alport’s disease
Acute nephritic syndrome caused by 2 conditions
- crescentic glomerulonephritis
2. diffuse proliferative glomerulonephritis
Crescentic glomerulonephritis facts
- glomerular crescents are formed
- cause severe glomerular injury
- severe kidney injury and death can result if untreated
Crescentic glomerulonephritis types
Type 1: Anti GBM disease
Type 2: Immune complex type
Type 3: Pauci-immune type
Anti GBM disease immunofluorescence
- linear capillary loop IgG
Anti GBM disease lab values
- elevated serum anti-GBM antibody
Anti GBM disease causes
- Anti GBM disease, Goodpasture’s disease
Immune complex type immunofluorescence
- granular deposits (IgG/IgA) in mesangium or capillary loops
Immune complex type lab values
- elevated serum anti nuclear antibodies, anti dsDNA
Immune complex type causes
- lupus nephritis
- IgA nephropathy (only Henoch-Schonlein pupura)
Pauci-immune type immunofluorescence
- negative for deposits
Pauci-immune type lab values
- elevated serum anti neutrophil cytoplasmic antibodies (ANCA)
Pauci-immune type causes
- ANCA associated diseases: wegener’s granulomatosis, microscopic polyarteritis
Anti-GBM disease (goodpasture’s disease)
- autoimmune disease: abnormal production of antibodies directed against collagen IV, which constitutes the basement membranes of glomerulus and lung
Anti-GBM Disease treatment
- steroids, cytotoxic agents, PLASMAPHERESIS
Pauci-immune crescentic GN
- group of vasculitic disorders affecting small vessels
- can cause pulmonary renal syndrome
- associated with anti-neutrophil cytoplasmic antibody (ANCA)**
- classic example of wegener’s granulomatosis
Treatment of Crescentic GN
- immunosuppressive therapy: corticosteroids
- cytotoxic agents: cyclophosphamide
- in addition to above anti-GBM needs plasmapheresis*
Three causes of crescentic GN
- anti GBM disease
- immune complex GM
- pauci-immune disorders
Diffuse proliferative GN (DPGN) causes
- acute post streptococcal glomerulonephritis
- proliferative (active) lupus nephritis
Acute post streptococcal GN facts: who’s affected
- affects children
- history of respiratory infection 2 weeks prior
- only certain “nephritogenic” strains of B-hemolytic streptococci cause this
Acute post-streptococcal GN: presentation
- acute nephritic syndrome
- kidney injury
- Hypertension
- Low Complement 3 levels
- ASO titer serially elevated
Key features of acute post-streptococcal GN
- diffuse endocapillary proliferative GN with subepithelial “humps”
- IgG and C3 deposits by immunofluorescence
Proliferative lupus nephritis
- acute nephritic syndrome
- systemic symptoms of active lupus: arthritis, rashes
Proliferative lupus nephritis evidence
- serology: anti nuclear antibody, anti-dsDNA+, low complements C3 and C4
Key features of lupus nephritis
- endocapillary proliferation, crescents, necrosis
- FULL house pattern by immunofluorescence
- subendothelial deposits
Three types of hypercellularity
- mesangial
- endocapillary
- crescents
Causes of acute nephritic syndrome
- crescentic GN (3 types)
2. diffuse proliferative GN (2 types)
Types of nephritic syndrome
- isolated hematuria
- acute nephritic syndrome
