Glomerulonephropathies Flashcards
List the 6 glomerulonephropathies
- nephritic syndrome
- post strep glomerulonephritis
- IgA nephropathy
- Henoch-Schonlein Purpura
- Rapidly progressive glomerulonephritis
- Interstitial nephritis
What is the difference between Nephritic and Nephrotic?
- *Nephritic**:
- positive for RBC*
- red cell or mixed cellular casts*
- variable degrees of proteinuria
- *Nephrotic**
- proteinuria greater than 3.5g/day
- lipiduria
- fatty casts
- edema
Nephritic Causes
- Postinfectious glomerulonephritis
- IgA nephropathy
- Henoch-Schonlein purpura (aka IgA Vasculitis)
- Mesangial Proliferative Glomerulonephritis
- Lupus Nephritis
- Rapidly Progressive(crescentic) glomerulonephritis
Nephrotic Causes
- systemic… Diabetic nephropathy, amyloidosis, systemic lupus
- minimal change dz (children)
- focal segmental glomerulosclerosis
- membranous nephropathy
What are the two types of Nephritic Dz, describe each and give an example.
Focal nephritic: -inflammatory lesions in less than 1/2 of glomeruli
-UA shows RBC, occasional RBC casts, mild proteinuria
Example: IgA Nephropathy
- *Diffuse Nephritic**:
- Affects greater than 1/2 of glomeruli
- UA similar to focal but with more proteinuria
- edema, HTN, renal insufficiency
Example: Post Strep Glomerulonephritis
Nephrotic Dz Patterns, describe and give an example.
Affects many glomeruli
Without obvious inflammation or immune complexes
edema, hyperlipidemia, hypoalbuminemia
Ex. Diabetic neuropathy
Post Streptococcal Glomerularnephritis
- primarily affects what age group?
- when does this develop?
- does abx treatment prevent glomerular dz?
- primarily affects children between 5-12yrs
- develops 1-3wks post strep throat infection or 3-6wks post skin infection w/ group A beta-hemolytic strep
- Abx tx does not always prevent glomerular dz
Post strep Glomerulonephritis
- pathophys
- Can viral illness and parasitic infections be associated with glomerulonephritis?
Pathophys: caused by glomerular immune complex dz induced by group A beta hemolytic strep.
Glomerular immune complex dz triggers complement activation and inflammation.
*immune complex irritates glomerular vessel leading to inflammation and narrowing of capillary lumen
-you bet they can!!
Clinical Signs and Sx of Post strep glomerulonephritis
- often asymptomatic
- microscopic hematuria….gross hematuria
- proteinuria (can reach nephrotic range)
- Edema
- HTN
- Increased SCr
- Variable decline in GFR
- Hypocomplementemia (C3, C4)
- acute renal failure and need for dialysis is uncommon.
Posts strep glomerulonephritis
-serology
Serology: looking for elevated titers of abys to extracellular strep products.
- Streptozyme test measures 5 streptoccocal abys:
1. Antistreptolysin (ASO)**
2. Anti-hyaluronidase
3. Anti-streptokinase
4. Anti-nicotinamide-adenine dinucleotidase
5. Anti-DNAse B abys
-*Is positive in 95% of pts d/t pharyngitis and about 80% of those with skin infections.
Biopsy is not typically performed in PSGN pts to confirm diagnosis since hx of recent strep infection is very specific, but biopsy can be performed if ?
- other glomerular disorders are being considered b/t they deviate from natural course of PSGN
- presents late without a clear history of prior strep infection.
PSGN:
-persistently low C3 levels beyond 6wks are suggestive of a dx of what type of glomerulonephritis?
-Recurrent episodes of hematuria are suggestive of what type of glomerulonephropathy?
membranoproliferative
recurrent episodes of hematuria are suggestive of IgA nephropathy and are Rare in PSGN.
Post strep glomerularnephritis Tx & Prognosis
Treatment:
-Treat the volume overload
–sodium and water restriction
–Lasix (volume and HTN)
–Those with acute renal failure, dialysis may be required.
Prognosis:
- complete recovery, resolution begins within first 2 wks
- smaller subset of pts have late renal complications (HTN, increased proteinuria, renal insufficiency)
IgA Nephropathy
- aka
- most common in who?
- primarily affects what age group?
aka: Bergers Dz
Most common in Asians and Caucasians, Males
primarily affects ages 15-35
WHat is the most common cause of acute nephritis worldwide?
What is the most common lesion to cause primary glomerulonephritis in the developed world?
What is the most common cause of vasculitis in children?
- Post strep glomerulonephritis
- IgA Nephropathy
- Henoch Scholein Purpura
Etiology? Pathophysiology of IgA nephropathy
Etiology: unknown
Pathophysiology:
-dysregulated synthesis and metabolism of IgA resulting in IgA immune complexes that favor mesangial deposition.
IgA nephropathy Presentation (3 ways)
- )Gross hematuria that may be recurrent*
- flank pain
- fever
* 50%* - )Microscopic hematuria…eventually gross hematuria
- mild proteinuria
* 30%* - )Nephrotic Syndrome (proteinuria, lipiduria, fatty casts)
- edema
- renal insufficiency
- HTN
- Hematuria
* 10%*
IgA nephropathy Diagnosis
-Urinalysis
- confirmation is by kidney bx:
- -prominent IgA deposits in the mesangium and along glomerular capillary walls
–C3 and IgG are also deposited
–mesangial IgA deposition may be seen with cirrhosis, celiac dz, and HIV infection
Treatment of IgA nephropathy
Prognosis
Treatment:
Blood pressure control: ACEI/ARB
Severe or progressive Dz tx:
-corticosteroids/immunosuppressants
Prognosis:
-most pts enter sustained clinical remission
-some may have persistent low-grade hematuria &/or proteinuria
-slow progression ot ESRD in 50% signs this will happen... --elevated SCr --HTN --persistent protein excretion about 1000mg/day
Henoch-Scholein Purpura
-aka
- pathophys
- cause
- most common in what age group?
Aka: IgA vasculitis
Patho: systemic vasculitis characterized by tissue deposition of IgA immune complexes
Cause: unknown, immune-mediated vasculitis
-3-15years
Tetrad of clinical manifestations for HSP/IgA Vasculitis
- palpable purpura (typically over LE and feet) w/o thrombocytopenia or coagulopathy
- arthritis/arthralgias
- abdominal pain
-
renal dz/failure
- other manifestations may be mild asymptomatic hematuria, proteinuria, nephrotic syndrome and even renal failure.
HSP Nephritis:
- how does this differ from IgA Glomerulonephritis?
- Dx
- prognosis
- Tx
- has identical histological findings, but HSP has IgA immune complex deposition vasculitis systemically.
- Dx: confirmed by kidney bx, but this is reserved fro when dx is uncertain or severe renal involvement.
- Prognosis:
- recurrence is common
-minority develop persistent renal manifestations of end-stage renal dz
Tx:
- supportive; glucocorticosteroids
- for those who develop ESRD dialysis and renal transplant can be performed.
Rapidly Progressive Glomerulonephritis:
-characterized by?
- Characterized by rapid progression to end stage renal failure in days, weeks, months.
- Will see crescent formation on bx
Crescent formation pathophysiology
formation is in response to severe injury to glomerular capillary wall. initially begins as active inflammation of capillary wall resulting in movement of plasma products including fibrinogen into bowmans space w/ subsequent fibrin formation, the influx of Mfs and Tcells release proinflamm cytokines. Fibrocellular and fibrous crescents develop.
