308 Glomerulolar Disease Flashcards
What test will you do to assess risk for diabetic nephropathy?
Urine Creatinine: protein ratio
50M mine worker presents with 2 months chronic cough, purulent nasal discharge and low grade fever. Treated with co-amoxiclav, however, develops shortness of breath and hemoptysis. PE +purpura on both hands and legs and crackles on right lower lung fields. Labs leukocytosis with neutrophilia, Creatinine 3.2 mg/dl and urinalysis +hematuria and proteinuria. CXR shows right lung nodule and non specific infiltrates. Biopsy of the pulmonary nodule shows neutrophilic microabscesses and necrotizing granuloma. What is the most likely diagnosis? A. Goodpasture's syndrome B. Churg Strauss syndrome C. Granulomatosis with polyangitis D. Microscopic polyangitis
C. Granulomatosis with polyangitis
Glomerulonephritis that presents with gross hematuria
IgA nephropathy
Sickle cell disease
Other differentials for microscopic hematuria not necessarily GN
BPH Interstitial nephritis Papillary necrosis Renal stones Cystic kidney disease Renal vascular injury
Causes of transient proteinuria
Fever Exercise Obesity Sleep apnea Emotional stress CHF
Proteinuria only seen in upright posture
Orthostatic proteinuria
True or false. Proteinuria in adults and children with glomerular disase is selective and composed of albumin
False. Adults non selective. Albumin and mixture of other serum proteins
Most common causes of GN in the western hemisphere
Malari
Schistosomiasis
HIV
Hepatitis B and C
True or false. In chronic GN, decreased kidney size if often seen
True.
When does GN occur after a skin infection? After strep pharyngitis?
Skin infection: 2-6 weeks
Pharyngitis: 1-3 weeks
When does endocarditis associated GN typically occurs
Subacute bacterial endocarditis
Untreated for a long time
Negative blood cultures
Right sided endocarditis
When goes GB in acute bacterial endocarditis occur
10-14 days
Most common and serious complication of SLE
Lupus nephritis
Most varies course of lupus nephritis
Class III
The only reliable method of identifying morphologic variant of lupus nephritis
Renal biopsy
Complication of class V lupus nephritis
Renal vein thrombosjs
Other thrombotic complications
True or false. SLE tends to became quiescent once there is renal failure because of immunosuppressant effects of uremia
True
When can renal transplant be done in patient with lupus nephritis
After 6 months of inactive disease
What is the target epitopes for anti GBM disease
A3 NC1 domain of collagen IV
Most common Glomerulonephritis world
IgA nephropathy
Single most common cause of chronic renal failure
DM nephropathy
What is a the characteristic lesion in HIV associated nephropathy
FSGS
Collapsing glomerulopathy
plasma of water filtered by the glomerular capillaries
120-180 L/day
True or false. The glomerulus is an imperfect barrier in the case of serum albumin
True.
what is the physical radius of albumin compared to the pores in the GBM and slit pore membranes
albumin has a physical radius of 3.6 nm while pores in the GBM and split ore membranes have a radius of 4 nm
how much albumin is voided in the urine daily
8-10 mg of albumin in daily voided urine
hematuria suspicous for GN
3-5 RBC in the spun sediment from first voided morning urine is suspicious
GN which presents with gross hematuria
IgA nephropathy and sickle cell disease
differential of microscopic hematuria
malignancy of urinary tract, BPH, interstitial nephritis, papillary necrosis, hypercalciuria, renal stones, cystic kidney disease, or renal vascular injury
found in the urine sediment which points more likely to glomerulonephritis
RBC casts, dysmorphic RBC
proteinuria in microalbuminuria
30-300 mg in 24 hrs
represents frank proteinuria and more advanced renal disease
more than 300 mg in 24 hours
Urine assays for Albuminuria/Proteinuria. Normal
24hr urine: 8-10 mg ACR less than 30 mg/g Dipstick proteinuria none 24hr urine protein less than 150 mg in 24 hr
24hr urine: 8-10 mg ACR less than 30 mg/g Dipstick proteinuria none 24hr urine protein less than 150 mg in 24 hr
24hr urine 30-300 mg ACR 30-300 mg/g Dipstick proteinuria trace to 1+ 24hr urine protein none
Urine assays for Albuminuria/Proteinuria. Proteinuria
24hr urine more than 300 mg ACR more than 300 Dipstick proteinuria 3+ 24hr urine protein more than 150
defined sustained proteinuria
more than 1-2 g/ 24 hrs
define benign proteinuria
less than 1 g/ 24 hrs
examples of functional or transient proteinuria
fever, exercise, obesity, sleep apnea, emotional stress, and CHF
True or false. Proteinuria in adults is nonselective containing albumin and other serum proteins whereas in children it is selective
True,
Patterns of clinical glomerulonephritis (6)
acute nephritic syndromes, pulmonary renal syndromes, nephrotic syndromes, basement membrane syndromes, glomerular vascular syndromes, infectious disease associated syndromes
stain used in renal biopsy to enhance basement membrane structure
Jones methenamine silver
stain used in renal biopsy to identify collagen deposition and assess the degree of glomerulosclerosis and interstitial fibrosis
Masson’s trichrome
When do you say that the lesion is focal
less than 50% glomeruli involved
When do you say that the lesion is diffuse
more than 50% glomeruli involved
injury involving most of the glomerulus
global
injury involving portion of the tuft
segmental
refers to glomeruli showing increased cellularity
proliferative
refers to cellularity in the capillary tuft
endocapillary
when cellular proliferation extends into the Bowman’s space
extracapillary
formed when epithelial podocytes attach to Bowman’s capsule in setting of glomerular injury
synechiae
extension of the synechiae when fibrocellular fibrin collections fills all or part of Bowman’s space
?
acellular, amorphous accumulations of proteinaceous material throughout the tuft with loss of capillaries and normal mesangium
sclerotic glomeruli
Examples of acute nephritic syndromes
Post strep GN, Subacute bacterial endocarditis, lupus nephritis, anti GBM disease, IgA nephropathy, Henoch Schonlein purpura etc
prototypical for acute endocapillary proliferative glomerulonephritis
postreptococcal glomerulonephritis
strain of streptococci which antedate glomerular disease in poststrep GN
M types of streptococci
M types streptococci seen in impetigo
47, 49, 55, 2, 60, 57 = higher number impetigo
M types streptococci seen in pharyngitis
1,2,3,4, 25,49, 12 = lower number pharyngitis
how many weeks does post strep GN develop after pharyngitis
1-3 weeks
how many weeks does post strep GN develop after impetigo
2-6 weeks
associated with subendothelial deposits or humps
post strep GN
nephritogenic antigen demonstrated inside the subepithelial humps on renal biopsy on poststrep GN
streptococcal pyrogenic exotoxin B (SPEB)
treatment of poststrep GN
supportive
What is the prognosis of poststrep GN
good in adulthood; worse in elderly with 60% progressing to ESRD
when does complete resolution of hematuria occurs in patients with poststrep GN
Within 3-6 week of the onset of nephritis
True or false. Endocarditis associated glomerulonephritis is a complication of SBE particularly in patients who remain untreated for a long time, have negative blood cultures or have right sided endocarditis
True.
why is glomerunephritis unusual in acute bacterial endocarditis
because it takes 10-14 days for immune complex mediated injury
what is the gross appearance of the kidneys in patients with endocarditis- associated glomerulonephritis
subcapsular hemorrhages with a flea beaten appearance
what is the primary treatment of endocarditis associated glomerulonephritis
4-6 weeks of antibiotics
what is the prognosis of SBE glomerulonephritis
guarded
common and serious complication of SLE
lupus nephritis
most common clinical sign of renal disease in SLE
proteinuria
antibodies that correlated best with presence of acute lupus nephritis
anti dsDNA
Class of lupus nephritis with most varied course
Class III
Class of lupus nephritis. Normal glomerular histoloy
Class I
Class of lupus nephritis. Mesangial immune complexes with mesangial proliferation
Class II
Class of lupus nephritis. Focal lesions with scarring.
Class III
Class of lupus nephritis. Diffuse proliferation
Class IV
Class of lupus nephritis. Thickened basement membrane
Class V
Class of lupus nephritis. Global sclerosis
Class VI
aggressive lesion in lupus nephritis has the worst renal prognosis
crescents on biopsy
define remission in lupus nephritis
near normal renal function and proteinuria less than 330 mg/dL per day
Lupus nephritis class predisposed to renal vein thrombosis and other thrombotic complications
lupus nephritis class V
when can patient with lupus nephritis have renal transplantation
After 6 months of inactive disease
targets epitopes for anti GBM disease
quaternary structure of a3 NC1 domain of collagen IV
True or false. Goodpasture’s syndrome in younger age groups are usually explosive
True.
What is the prognosis of anti GBM glomerulonephritis
good prognosis with treatment
what is the treatment of anti GBM disease
plasmapharesis, oral prednisone and cyclophosphamide
one of the most common forms of glomerulonephritis worldwide
IgA nephropathy
Differential IgA with Henoch Schonlein purpura
HSP has prominent systemic symptoms, younger age, preceding infection and abdominal complaints
two most common presentations of IgA nephropathy
recurrent episodes of macroscopic hematuria during or immediately following an upper respiratory infection often accompanied by proteinuria or persistent asymptomatic microscopic heamturia
True or false. IgA nephropathy is a benign disease. Renal faillure seen only 25-30% over 20-25 years
True,
True or false. A group of patients with small vessel vasculitis and glomerulonephritis have serum ANCA
True.
two types of antibodies in ANCA small vessel vaculitis
anti proteinase (PR3) and anti myeloperoxidase (MPO)
ANCA positive vasculitis
granulamotosis with polynagiitis, microscopic polyangitis, Churg- Strauss syndrome
what is the induction therapy for ANCA small vessel disease
glucocorticoids and either cyclophosphamide or rituximab
given to induce remission in ANCA associated vasculitis
monthly pulse IV cyclophasphamide
patients with this disease present with fever, purulent rhinorrhea, nasal ulcers, sinus pain, polyarthrias/arthritis, cough, hemoptysis, shortness of breath, microscopic hematuria
granulomatosis with polyangitis
patients with this disease present with peripheral eosinophilia, cutaneous purpura, mononeuritis, asthma, and allergic rhinitis
Churg Strauss Syndrome
defined morphologically with dense deposits forming ribbons in the GBM
Dense Deposit Disease (DDD)
Formerly called MPGN type II
Dense Deposit Disease (DDD)
Recent disease classification that is defined by the glomerular accumulation of C3 with little or no immunoglobulin and encompasses dense deposit disease
C3 glomerulopathy
what is the prognosis of C3 glomerulopathy
poor with 50% progressing to ESRD
immune mediated glomerulonephritis characterized by thickening of the GBM with mesangioproliferative changes
MPGN or mesangiocapillary glomerulonephritis
Type of MPGN assocaitred with persistent hepatitis C infections, autoimmune disease like lupus
type I
most proliferative type of MPGN
type I
associated with tram tracking on renal biopsy
Type I MPGN
Characterized MPGN type II
low serum C3, dense thickening of the GBM containing ribbons of dense deposits and C3
MPGN characterized by focal proliferation with widened segments of the GBM that appear laminated and disrupted
Type III MPGN
True or false. Fifty percent of patients with MPGN develop ESRD 10 years after diagnosis and 90% have renal insufficiency after 20 years
True,
glomerulonephritis associated with plasmodium falciparum
mesangioproliferative glomerulonephritis
characterized by expansion of the mesangium, sometimes assocaited with mesangial hypercellularity, single contoured capillary walls and mesangial immune deposits
mesangioproliferative glomerulonephritis
classic presentation of nephrotic syndrome
heavy proteinuria, minimal hematuria, hypoalbuminemia, hypercholesterolemia, edema and hypertension
True or false. All patients with hypercholesterolemia secondary to nephrotic syndrome should be treated with lipid lowering agents because they are at increased risk for cardiovascular disease
True.
Causes 70-90% of nephrotic syndrome in children
Minimal change disease or nil lesions
how many percent does MCD cause nephrotic syndrome in adults
10-15%
characteristic of MCD
effacement of foot process on electron microscopy
who are called primary responders in patient with minimal change disease
remission or less than 0.2 mg/24h of proteinuria after single course of prednisone
who are called frequent relapser
two or more relapses in 6 months after taper
when an adult patient with MCD called steroid resistant
no response after 4 months of therapy
how long can adults have a remission in MCD
after a course of 20-24 weeks
what is the prognosis of MCD in adult
less favorable when acute renal failure or steroid resistance occurs
refers to a pattern of renal injury characterized by segmental glomerular scars that involve some but not all glomeruli
focal segmental glomerulosclerosis FSGS
the pathologic changes of FSGN are most prominent where
prominent in glomeruli located at the corticomedullary junction
most common cause of nephrotic syndrome in the elderly
membranous glomerulonephritis
what is the characteristic of membranous glomerulonephritis
uniform thickening of the basement membrane along the peripheral capillary loops
true or false. 20-30% of membranous glomerulonephritis is associated with malignancy
True,
has the highest reported incidences of renal vein thrombosis, pulmonary embolism, and deep vein thrombosis
membranous glomerulonephritis
sensitive indicator for the presence of diabetes but correlates poorly with the presence or absence of clinically significant nephropathy
thickening of the GBM
nodules associated with diabetic nephropathy
nodular glomerulosclerosis or Kimmelstiel- Wilson nodules
True or false. Renal biopsies from patients with types 1 and 2 diabetes are largely indistinguishable
True.
earliest manifestation of diabetic nephropathy
microalbuminuria or albuminura range of 30- 300 mg/24 hr
differential light chain deposition disease from cast nephropathy
light chain deposition disease cause nephrotic syndrome with renal failure; produce kappa light chains that do not have the biochemical features necessary to form amyloid fibrils
rare glomerulopathy characterized by glomerular accumulation of non branching randomly arranged fibrils
Fibrillary Glomerulonephritis
characteristic of renal amyloidosis
deposition of beta pleated sheets of serum amyloid protein
deficiency in Fabry’s disease
deficient lysosomal alpha galactosidase
what is the effect of Fabry’s disease
excess deposition of globotriaosylceramide
glomerulopathy what revels enlarged visceral epithelial cells packed with small clear vacuoles
Fabry’s disease
chronic glomerulosclerosis leading to renal failure associated with sensorineural deafness
Alport’s syndrome
some patients with this disease develop lenticonus of the anterior lens capsule, dot and fleck retinopathy
Alport’s syndrome
True or false. Thin basement membrane disease have a benign course
True,
characterized by persistent or recurrent hematuria but no associated proteinuria, hypertension or loss of renal function or extrarenal disease
thin membrane disease TMD
what the renal biopsy picture of patient with HIV associated nephropathy
FSGS revealing a collapsing glomerulopathy
viral infection that can cuase endocapillary proliferative glomerulonephritis
dengue hemorrhagic fever and measles
viral infection that cause mesangial proliferative glomerulonephritis
Hanta virus, parvovirus, mumps, EBV,
viral infection that cuase focal glomerulonephritis or DPGN
coxsackie virus
schistosoma associated with clinical renal disease
schistosoma mansoni
glomerular lesson in Class I nephritis from schistosoma
Class I mesangioproliferative
glomerular lesson in Class II nephritis from schistosoma
extracapillar proliferative
glomerular lesson in Class III nephritis from schistosoma
membranoproliferative
glomerular lesson in Class IV nephritis from schistosoma
focal segmental glomerulonephritis
glomerular lesson in Class V nephritis from schistosoma
amyloidosis
classes of schistosoma nephritis associated with IgA immune deposits and progress despite antiparasitic or immunosuppressive therapy
Class III and IV
