1. Glomerular diseases Flashcards
What are the clinical manifestations of glomerular diseases?
- Hematuria and/or proteinuria,
- deterioration of kidney function,
- development of hypertension,
- edema (peripheral, periorbital),
- hypercoagulability,
- appearance of systemic symptoms.
What are the clinical features of acute nephritis syndrome?
- Decrease in eGFR in a few days
- oliguria
- microscopic or macroscopic hematuria, dysmorphic RBC
- subnephrotic proteinuria
- normal or slightly low serum albumin,
- generalized edema,
- high blood pressure
- may include headache and anorexia.
What are the clinical features of rapid progressive glomerulonephritis?
- Decrease in eGFR in a few days to 1-2 months,
- can have oliguria,
- mostly microscopic hematuria with dysmorphic RBCs and acanthrocytes,
- subnephrotic proteinuria,
- normal or slightly low serum albumin,
- maybe mild edema,
- normal blood pressure,
What are the systemic symptoms associated with Rapid Progressive Glomerulonephritis (RPGN) syndrome?
Fever, weight loss, joint pain, muscle pain, weakness, palpable purpura.
What are the only presenting symptoms in patients with Chronic (asymptomatic) nephritis?
- Microscopic hematuria with dysmorphic RBC
- high BP
What is the general treatment for nephritis syndromes?
Fluid status, table salt and fluid intake reduction, Furosemide, regulation of blood pressure.
What is the effect of ACEi/ARB on eGFR?
Decrease.
What is the typical fluid intake reduction for patients with nephritis syndromes?
500-1000 ml/day.
What is the typical table salt intake for patients with nephritis syndromes?
less than 5 gr/day.
What is the definition of Nephrotic syndrome?
Nephrotic syndrome is characterized by heavy proteinuria (> 3 g/day), hypoalbuminemia, and pronounced edema (face and limbs)
What is the typical urine volume in Nephrotic syndrome?
Urine volume is normal in Nephrotic syndrome.
What is the level of Hematuria in Nephrotic syndrome?
Hematuria is not typical in Nephrotic syndrome.
What is the level of Serum albumin in Nephrotic syndrome?
Serum albumin is low in Nephrotic syndrome.
What is the level of Blood pressure in Nephrotic syndrome?
Blood pressure is normal or low in Nephrotic syndrome.
What are the consequences of proteinuria in Nephrotic syndrome?
The consequences of proteinuria in Nephrotic syndrome include generalized edema and hyperlipidemia.
What is the general treatment for Nephrotic syndrome?
- weight loss,
- liquid and salt restriction,
- diuretics,
- proteinuria reducing medications such as ACE inhibitors or ARBs.
- anticoagulant treatment
- lipid lowering drugs
What is the recommended protein restriction for Nephrotic syndrome patients?
The recommended protein restriction for Nephrotic syndrome patients is 0.8 g/kg/day.
What is the recommended anticoagulant treatment for Nephrotic syndrome patients with low serum albumin?
The recommended anticoagulant treatment for Nephrotic syndrome patients with low serum albumin is LMWH.
What are the recommended lipid-lowering drugs for patients with normal kidney function?
Fibrates - otherwise use statins
What are the recommended serum tests for investigating glomerulopathies?
Creatinine, Urea, Ions, Albumin, CRP, AST.
What are the recommended immunoglobulin tests for investigating glomerulopathies?
IgA, IgG, IgM, cryoglobulin.
What are the recommended complement tests for investigating glomerulopathies?
C3, C4.
What are the recommended serum light chain tests for investigating glomerulopathies?
ELFO, lamda, kappa
What are the recommended antibody tests for investigating glomerulopathies?
ANA, anti-dsDNA, ANCA, antiPLA2R (antibodies to phospholipase A2)
What are the recommended protein excretion tests for investigating glomerulopathies?
mg/24 h or ~mg/mmol (albumin/creatinine or total protein/creatinine).
What are the recommended urine tests for investigating glomerulopathies?
Urine sediment test, Stix, protein ELFO.
What are the two histological classifications of glomerular diseases?
Proliferative and non-proliferative.
What is the clinical picture associated with poststreptococcus GN?
- Acute nephrITIC syndrome
- Proliferative
- Can be isolated hematuria (asymptomatic).
What is the histology associated with poststreptococcus GN?
- Endocapillary proliferative glomerulonephritis
- Granular deposits
What is ANCA vasculitis?
- Rapidly progressing glomerulonephritis
- Proliferative
- Autoantibodies against MPO or PR3 (neutrophil proteins)
Histological findings in ANCA vasculitis?
- Extracapillary proliferation
- Crescent formation
- Pauci immune (!)
Clinical picture of Goodpasture syndrome
- RPGN (nephritic syndrome)
- Proliferation
Histological findings of goodpasture syndrome
- Extracapillary proliferation
- LINEAR depositions
What are the special laboratory findings in a patient with SLE nephritis?
- ANA-Screen positive,
- Anti-dsDNA and anti-C1q elevated,
- C4 and C3 decreased.
What is the histological finding in SLE nephritis?
- Mesangial and endocapillary proliferation w/ or w/o crescents
- Granular deposition
What is the histological finding in IgA nephropathy?
- Mesangial proliferation
- Granular deposition
What is the clinical presentation of Sch̦nlein-Enoch Purpura?
- Mostly nephritic syndrome, but it can also present as mixed nephrotic / nephritic.
- Palpable purpura, joint pain, abdominal pain, bleeding with lower GI, hematuria, and deteriorating eGFR.
What is the clinical presentation of membranoproliferative/mesangiocapillaris GN?
- Almost anything but most often mixed or nephritic syndrome.
- Both proliferative and non proliferative abnormalities
What is the clinical picture of minimal change disease?
- Nephrotic syndrome.
- Non proliferative
What is the clinical picture of focal segmental glomerulosclerosis?
- Nephrotic syndrome with or without decreasing GFR.
- Non proliferative
What disease can cause minimal change disease?
Hodgkin’s disease.
What are other causes of focal segmental glomerulosclerosis?
HIV, heroin abuse, extremely low birth weight, extreme obesity, bodybuilding, obstructive uropathy.
What are the histological findings in membranous nephropathy?
Granular deposition, thickening of basement membrane
What is the clinical picture of membranous nephropathy?
- Nephrotic syndrome.
- Non proliferative
What are secondary causes of membranous nephropathy?
Solid tumor/chronic infection/autoimmune disease.
What is the clinical picture of diabetic nephropathy?
- Nephrotic syndrome/isolated proteinuria with or without decreasing GFR.
- Non proliferative
What is the clinical picture of a patient with amyloidosis?
- Nephrotic syndrome (with or without decreasing GFR)
- Non proliferative
What is the recommended treatment for acute nephritis
- Supportive therapy
- volume and salt absorption control,
- loop diuretic (furosemide)
- antihypertensive therapy (CCB; ACE inhibitors / ARB only with caution!),
- renal replacement therapy.
What is the recommended treatment for chronic nephritis?
- Antihypertensive and anti-proteinuria therapy (ACE inhibitors / ARB),
- fish oil
- depending on the tissue p.o. steroids
What is the recommended treatment for RPGN?
- Aggressive immunosuppression is required
- IV then per os steroids
- rituximab maintenance therapy
What is the recommended treatment for minimal change nephrosis?
Usually a good response to steroids.
What is the recommended treatment for FSGS?
Longer-term steroid therapy is required.
What is the recommended treatment for membranous nephropathy?
Steroid + Cyclophosphamide / Cyclosporine and in a therapy-resistant case use rituximab.
What is the recommended treatment for diabetic nephropathy?
Adequate blood sugar control, ACE inhibitors, or ARB to control blood pressure and reduce proteinuria, and reduction of salt intake.
What is the recommended treatment for amyloid (AL)?
Hematological treatment.
What diseases typically manifest as RPGN?
- SLE nephritis
- ANCA vasculitis
- Goodpasture syndrome