Exam 3 - Glomerulonephritis & Cystic Disease of Kidney Flashcards
What is a general description of Glomuerular disease?
Damage to the major components of the glomerulus
What is the difference between primary and secondary glomerular disease?
Primary = Glomerular injury is limited to the kidney
Secondary = Renal abnormalities result from a systemic disease
What is glomerulonephritis?
A term given to those diseases that present in the nephritic spectrum and usually signifies an inflammatory process causing renal dysfunction?
What is the etiology of Glomerulonephritis?
Deposition of immune complexes in the glomerulus
RBC casts are pathognomonic for what disorder?
Glomerulonephritis
The following clinical findings should cause concern for what?
- RBC casts
- Dysmorphic RBCs
- Cola-colored urine
- Proteinuria < 3.0 g/day
- Elevated creatinine, decreased GFR
- Oliguira
- Edema
- HTN
Nephritic Syndrome
What is commonly characterized morphologically by extensive crescent formation?
Rapidly Progressive Glomerulonephritis (RPGN)
Is hematuria more associated with the nephritic or nephrotic spectrum?
Nephritic
Are the following urine findings associated with extraglomerular or glomerular hematuria?
- Red or pink color
- Clots present
- Proteinuria usually absent
- Normal RBC morphology
- No RBC casts
Extraglomerular
Are the following urine findings associated with extraglomerular or glomerular hematuria?
- “Cola-colored”
- No clots
- Proteinuria may be present
- Dysmorphic RBC morphology
- RBC casts present
Glomerular
What is the most common cause or primary glomerularnephritis in the world?
IgA Nephropathy “Berger Disease”
When are the peak incidences of IgA Nephropathy?
2nd and 3rd decades of life
IgA depositions in the glomerular mesangium are seen on biopsy, what should you suspect?
IgA Nephropathy
What are clinical features/classic presentation seen with IgA Nephropathy?
Gross hematuria following shortly after a URI (1-2 days after onset of illness)
Which patients are likely to progress to ESRD with IgA Nephropathy?
How are these patients treated?
Those with persistent proteinuria > 1 g/day, elevated serum Cr, HTN
Treat with an ACE-I or ARB
What is the cause of Poststreptococcal GN (PSGN)?
Caused by nephritogenic strains of group AB-hemolytic strep
What are clinical features/classic presentation seen with PSGN?
Nephritic spectrum symptoms starting 1-3 weeks after infection
How is PSGN diagnosed?
- Clinical presentation
- Low complement (C3)
- Documentation of recent GAS infection (elevated ASO titers and/or positive throat or skin cultures)
What is the classic tetra associated with IgA Vasculitis (HSP)?
- Rash (palpable purpura)
- Arthralgias
- Abdominal pain
- Renal disease
A child presents with nephritic spectrum symptoms 1-3 weeks after a GAS infection. What should you suspect?
PSGN
A 27 year old presents with nephritic spectrum symptoms 1-2 days post URI. What should you suspect?
IgA Nephropathy
A child presents with post-URI with purpura, joint pain, abdominal discomfort, and noted renal dysfunction on labs. What should you suspect?
IgA Vasculitis (HSP)
What is the etiology of Anti-GBM Antibody (Goodpasture) Disease?
Antibodies are directed against an antigen intrinsic to the GBM
While the terms are used interchangeably, what is the difference in nomenclature between Goodpasture Syndrome and Goodpasture Disease?
Goodpasture Syndrome:
GN + pulmonary hemorrhage
Goodpasture Disease:
GN + pulmonary hemorrhage + anti-GMB antibodies
If your patient is positive for anti-GBM antibodies in serum or kidney biopsy, what else should you check for?
Potential for “double-positive anti-GBM and ANCA-associated disease”
Need to also test for ANCA as many patients can present with both
What is the treatment for Anti-GBM Antibody (Goodpasture) Disease?
Plasmapheresis + immunosuppressive agents (prednisone and cyclophosphamide)
Pauci-Immune Glomerulonephritis includes what three systemic ANCA-associated vasculitides?
- Granulomatosis with polyangiitis (GPA)
- Microscopic polyangiitis (MPA)
- Eosinophilic granulomatosis with polyangiitis (EGPA)
What is the triad of GPA?
- Upper respiratory symptoms
- Lower respiratory symptoms
- GN
What are some upper/lower airway symptoms associated with GPA?
- Nasal or oral inflammation
- Saddle nose deformity
- Dyspnea
- Hemoptysis
What are common renal manifestations associated with GPA?
Cresentic necrotizing GN
Is GPA more associated with C-ANCA or P-ANCA?
C-ANCA
How does MPA differ from GPA in regards to presentation.
Spares upper respiratory tract and has no granuloma formation
How does EGPA differ from MPA and GPA?
EGPA is associated with asthma and eosinophilia
What are the three phases of EGPA?
- Prodromal phase (atopic triad)
- Eosinophilic infiltrative phase
- Vasculitic phase
What will serology show in Lupus Nephritis?
+ Anti-Ds DNA titers
What are some features of a benign renal cyst?
Round and sharply demarcated with smooth walls and no enhancement with constrast
What is the etiology of polycystic kidney disease (PKD)?
Inherited disease that causes an irreversible decline in kidney function
What is the most common genetic cause of PKD?
Autosomal dominant PKD
What are common manifestations of ADPKD?
- Renal HTN
- Abdominal/flank pain
- Hematuria
- Hepatic cysts
What is the initial diagnostic study used for screening and diagnosis of ADPKD?
Ultrasound
What is required for definitive diagnosis of ADPKD?
Genetic testing
What is the management for ADPKD?
- Strict BP control, low-salt diet
- Supportive care
- Tolvaptan
- Dialysis or kidney transplant
What populations does ARPKD primarily affect?
Infants and children
What is the mechanism of ARPKD?
Mutation of the PKHD1 gene
What organ systems are primarily affected in ARPKD?
Kidneys and hepatobiliary tract (hallmark)
While both ADPKD and ARPKD are associated with bilateral markedly enlarged kidneys, progressive renal impairment, and HTN, what distinguishes ARPKD?
ARPKD is characterized by congenital hepatic fibrosis which will leads to portal HTN
