Nephritic Syndrome Flashcards
Traits of nephrOTIC syndrome
- 3+ proteinuria
- high cholesterol
- hypercoaguable
- low albumin
What are the classic NEPHROTIC syndromes?
- Minimal change disease (podocyte damage)
- Focal segmental glomerulosclerosis (podocyte damage)
- Membranous nephropathy (subepithelial immune complex)
What are some immune complex mediated NephrITIC syndromes?
- Post-infectious glomerulonephritis (sub- epithelial)
- Membranoproliferative glomerulonephritis (MPGN) (subendothelial)
- IgA nephropathy (mesangial)
What are the classic signs and symptoms of nephritic syndrome?
acute onset of hematuria, HTN, oliguria, MILD edema, and azotemia
THERE CAN BE EDEMA WITH NEPHRITIC
T or F. The hematuria associated with nephritic syndrome can be macroscopic or microscopic
T.
What other diseases show immune deposits in the sub endothelial and mesangial spaces?
- Lupus nephritis
- Post infectious GN (can be)
What things can cause glomerular endothelial cell injury?
- immune complexes
- TTP/HUS
- entrapment of paraproteins (common in MM)
What does damage of endothelial cells result in?
Cytokine and autacoid release which (along with activated complement) up regulate adhesion molecules on endothelial and circulating immune cells resulting in inflammation and thus HEMATURIA
Case 1. A 14-year-old Caucasian teenager presents with nephrotic syndrome and microhematuria. The patient has no history of fever, rash, arthralgias, or gross hematuria. Past medical history is unremarkable.
Physical examination reveals a BP of 138/80 mmHg and 2+ lower extremity edema.
Laboratory studies include serum creatinine level of 1.3 mg/dl, BUN of 43 mg/dl, 24-hour urine protein of 5.12 g, serum albumin of 2.7 g/dl, hemoglobin 10 g/dl, white blood cell count of 7.2k, platelets of 340k, and normal serum electrolytes.
Urinalysis shows 3+ protein and microhematuria without red blood cell casts.
Serologies were negative (included negative anti-nuclear antibody, ANCAs, hepatitis B surface antigen, hepatitis C virus antibody, and HIV).
Serum complement 3 (C3) was low
Kidney size by ultrasound was 10.4 and 10.3 cm.
creatinine is a little high for a 14 yo
albumin is low; hemoglobin is low
This patient has Membranoproliferazive glomerulonephritis (idiopathic in this age group)
Why is fever, rash, and arthralgias an important part of the history of someone with glomerular disease?
these would be indicative of vasculiitis
What are the types of MPGN? Most common?
I: most common form
II: also referred to as Dense Deposit Disease, likely a separate disease
III: VERY rare
What is MPGN type I?
hypercellularity of the glomerular turf and expanded mesangium and capillary wall changes
What do the capillary wall changes result in?
a double contour (replication) of the glomerular basement appearance referred to as a “tram track appearance” caused by displacement of immune deposits and infiltrating mesangium cells. Mesangial cells actually act like macrophages and try to remove the deposits and do this in the process
How can MPGN type I present?
- microscopic hematuria
- non-nephrotic range proteinuria (or nephrotic syndrome)
- RPGN (loss of kidney function rapidly)
Cause of MPGN type I?
may be idiopathic (in teenagers), but often associated with hep C infection in adults (sometimes hep B)
How is MPGN type I treated?
Don’t know but steroids used. Spontaneous remission can occur rarely but usually progresses to end stage renal disease
How does Dense deposit disease present?
similar to MPGN type I (also have low C3) but may have macular deposits and/or acquired partial lipodystrophy
What do 80% of DDD patients have?
C3 nephritic factor which is an antibody that stabilizes an enzyme that leads to continued destruction of C3
