Exam 3: Glomerulonephritis Flashcards
What is the basic filtering unit of the kidney?
Glomerulus
What is glomerular disease?
Damage to the major components of the glomerulus (the podocytes, glomerular basement membrane, capillary endothelium, and mesangium
What are the two classifications of glomerular disease?
Nephritic and nephrotic
What is primary glomerular disease?
Glomerular injury that is limited to the kidney
What is secondary glomerular disease?
Renal abnormalities that result from systemic disease
What is the etiology of glomerulonephritis?
Most commonly the cause is related to the deposition of immune complexes in the glomerulus
What are the clinical findings of nephritic syndrome?
Dysmorphic RBCs with RBC casts, Smokey or cola colored urine, proteinuria, elevated creatinine, oliguria, edema, and HTN
What is rapidly progressive (or crescentic) glomerulonephritis?
The most severe and clinically urgent end of the nephritis spectrum, causes rapid loss of renal function over a comparatively short period of time
What is rapidly progressive glomerulonephritis commonly characterized by?
Extensive crescent formation
What are the 3 diseases in the nephritic spectrum?
Asymptomatic glomerular hematuria, nephritic syndrome, and rapidly progressive glomerulonephritis
What symptom is common with nephritic diseases?
Hematuria
What are the major causes of hematuria?
Transient and unexplained, UTI, kidney stones, cancer, and BPH
Patient presents with red or pink hematuria with clots present. There is no proteinuria and RBC morphology is normal. Is this extraglomerular or glomerular hematuria?
Extraglomerular
Patient presents with cola-colored hematuria without clots. There is some proteinuria present and RBCs are dysmorphic with RBC casts. Is this extraglomerular or glomerular hematuria?
Glomerular
What level of proteinuria is typically seen with nephritic syndrome?
Typically subnephrotic <3.0 g/day
What kind of treatment do patients with acute nephritic syndrome or RPGN need?
Consider immediate hospitalization
What is the most common cause of primary GN in the world?
IgA nephropathy
What diseases is IgA nephropathy commonly associated with?
Cirrhosis, celiac disease, and other infections such as HIV and EBV
What are the essential tests needs for GM?
Urine dipstick and urine microscopy
What serum tests should you order for GM?
-Creatinine/GFR. ANA, ANCA, anti-GBM, and antistreptolysin O
If there is evidence of proteinuria with GM, what should you consider prescribing?
ACE or ARB
When in life is IgA nephropathy likely to occur?
2nd and 3rd decades of life
What is the pathogenesis of IgA nephropathy?
IgA deposition in the glomerular mesangium resulting in inflammation and glomerular injury
Patient presents with gross hematuria 1-2 days following a URI. What are you suspicious of?
IgA nephropathy
How is IgA nephropathy diagnosed?
- clinical suspicion and labs
- Confirmed ONLY by kidney biopsy
What are the two typical disease courses for IgA nephropathy?
1) Slow progression to ESRD in 50%
2) the rest may have sustained clinical remission or persistent low -grade hematuria
What is poststreptococcal GN (PSGN)?
immune complex mediated sequela of a pharyngitis or skin infection
** glomerular immune complex disease that triggers complement activation and inflammation
What most commonly causes PSGN?
Group A B-hemolytic strep
How soon does PSGN occur after a strep infection?
1-3 weeks
- good way to distinguish from IgA since IgA occurs a few days after illness
How is PSGN diagnosed?
- Clinical presentation and history
- Low complement
- Recent GAS infection
What patients has a risk of progression to ESRD in IgA nephropathy?
If there is persistent proteinuria >1 g/d, evaluated serum Cr/reduced GFR, or HTN
What is IgA vasculitis?
AKA henoch-schonlein purpura, an immune mediated vasculitis characterized by deposition of IgA containing immune complexes
What is the classic tetrad with IgA vasculitis?
Palpable purpura, arthralgias, abdominal pain, and renal disease
What is the treatment for IgAV nephritis?
Supportive care, symptomatic therapy, and targeted therapy to decreased complications
When do renal symptoms occur in IgAV nephritis?
Typically a few days to one month after onset of systemic symptoms, may present with nephritic or nephrotic syndrome, and favorable prognosis in most
What is anti-GBM disease?
AKA goodpasture disease, an autoimmune disorder in which circulating antibodies are directed against an antigen intrinsic to the glomerular basement membrane an the alveolar basement membrane
What is goodpasture syndrome?
When patient has syndrome of GN and pulmonary hemorrhage
When in life are patients more susceptible to developing anti-GBM disease?
- first peak: third decade of life (more males)
- Second peak: 6th and 7th decade (more females)
What is the difference between goodpasture disease and goodpasture syndrome?
Syndrome: describes the constellation of GN+ pulmonary hemorrhage
Disease: reserved for those with GN+ pulmonary hemorrhage+ anti-GBM antibodies
What is the most common etiology of anti-GBM disease?
Idiopathic
Goodpasture disease have renal involvement with clinical features of ***.
RPGN
How is goodpasture disease diagnosed?
-Demonstration of anti-GBM antibodies in serum or kidney biopsy
There is a potential for ‘double positive anti-GBM and ANCA associated disease’ ** test for ANCA!
What is the treatment for goodpasture disease?
Plasmapheresis and immunomodulating agents (prednisone and cyclophosphamide)
What is Lupus nephritis?
-an immune complex mediated glomerular disease with Anti-ds DNA antibodies
How is lupus nephritis diagnosed?
-Suspected by an abnormal urinalysis and elevation of serum creatinine, but diagnosis confirmed by renal biopsy
What are the 3 systemic ANCA associated vasculitides with Pauci-immune GN?
1) GPA
2) Microscopic polyangitis
3) Eosinophilic granulomatosis with polyangitis
What are the antibodies that produce tissue an vascular damage?
P-ANCI and C-ANCA
What is GPA and what is it characterized by?
C-ANCA associated vasculitis of small-medium vessels characterized by granulomatous inflammation
What are then renal manifestations of GPA?
RPGN is most common with crescentic necrotizing glomerulonephritis
How does GPA affect the upper and lower airway?
Nasal and oral inflammation and saddle node deformity
What is microscopic polyangitis (MPA)?
P- ANCA associated vasculitis of small-medium vessel that clinically appears similar to GPA
What are the features that distinguish MPA from GPA?
MPA has absence of granuloma formation and has sparing of the upper respiratory tract
What is eosinophilic granulomatosis with polyangitis (EGPA)?
P- ANCA associated vasculitis associated with asthma and esosinophilia
What are the 3 disease phases of EGPA?
1) prodromal
2) eosinophilic infiltrative phase
3) vasculitis phase
What is the prodromal phase of EGPA characterized by?
Atopic disease, allergic rhinitis, and asthma
What is the treatment for Pauci-immune GN?
- Referral
- Immunposuppressants (corticosteroids)
*** prognosis is very poor w/o treatment
What are the benign features of renal cysts?
Round and sharply demarcated with smooth walls, anechoic, and no enhancement with contrast
What is poly cystic kidney disease?
An inherited disease that causes an irreversible decline in kidney function (may be both autosomal dominant or recessive)
What do the majority of patients with PKD eventually require?
Dialysis or kidney transplant
What is the most common genetic cause of CKD?
Autosomal dominant PKD (ADPKD)
What are the two genes that account for most cases of ADPKD?
PKD1 and PKD2
What is ADPKD characterized by?
- Massive kidney enlargement
- Progressive decline in GFR (50% of patients will have ESRD by 60)
What are some of the extra renal manifestations of ADPKD?
HTN, abdominal pain, and hepatic cyst
What is the initial modality used for screening and diagnosis of ADPKD?
US
How is ADPKD treated?
- Tolvaptan
- Dialysis and kidney transplant
- strict BP control and low salt diet
What are the two primary organ systemic affected in autosomal recessive PKD (ARPKD)?
Kidneys and hepatobiliary tract
Who is ARPKD most common in and what is it caused by?
- Primarily a disease of infants and children
- Mutation of PKHD1 gene
What is ARPKD characterized by?
Bilaterally markedly enlarged kidneys and congenital hepatic fibrosis
How is ARPKD diagnosed?
US- may be detected by routine pregnancy US after 24 weeks gestation
What are the 6 etiologies of glomerulonephritis?
IgA nephropathy, Poststreptococcal GN, Anti-GBM, lupus nephritis, IgA vasculitis HSP, and Pauci immune GN
What is glomerulonephritis?
A term given to those diseases that present in the nephritic spectrum and usually signifies an inflammatory process causing renal dysfunction
A patient with IgA nephropathy has persistent proteinuria > 1g/day, elevated Cr and reduced GFR, or HTN. How should they be treated?
ACE or ARB
What are the two diseases of the nephritic spectrum that present with RPGN?
Anti-GBM glomerulonephritis and pauci immune GN
