#26 Virmani - Glomerular Disease Flashcards
Identify
Maltese CrOss
NephrOtic Syndrome
Identify
Oval Fat bodies
NephrOtic Proteinuria
Identify
Minimal Change Glomerulopathy
Thickening of basement membrane
Tx: prednisone
Identify
Focal Segmental Glomeruloscelerosis
podocyte alteration
Rapid deterioration to ESRD with collapsing variant
Identify
Collapsing FSGS
Commonly seen with HIV infection or bisphosphonates
Jones Silver stain:
The significant proliferation of podocytes to a point where it forms a pseudo crescent that obliterates the urinary space
Severe wrinkling and retraction of the GBM
Identify
Membranous GN
Nephrotic syndrome
development of immune complexes in SubEPIthelial
M-type phospholipase A2 recepter
Identify
Diabetic Nephropathy
(Nodular Glomerulosclerosis)
Identify
Membranoproliferative Glomerulonephritis (MPGN) SubENDOthelial deposits
Hematuria and proteinueria almost always present
‘tram track’
Identify and list lab results that might be seen.
Poststreptococcal GN
Group A, beta-hemolytic streptococcal
Cola-colored urine - RBCs and RBC casts
elevated ASO titer
Identify
Normal
What is the major component of the slit diaphragm in the podocyte?
Nephrin
Podocyte - highly differentiated epithlial cell
What type of collagen makes up the GBM?
Type IV
Structural support of capillary loop
What is the function of fenestrated endothelial cells?
Negatively charged and limit the passage of albumin/large molecules
When trying to distinguish the mechanism of glomerular disease which cause will target the podocytes?
Non-immune or Immune
Non-immune
Podocytopathy - fusion of the epithelial cells
Minimal change disease and FSGS
filtration barrier is compromised
What underlies most forms of primary GN and many secondary forms?
Immune mechanisms:
Antibody-mediated injury
Immune complex formation
Cytotoxic antibodies
Cell-mediatied Immune Injury
Alternative Complement Pathway
What are the general clinical manifestations of glomerular disease?
Hypertension
Malaise
Edema
Proteinuria
Hematuria
What are the five major features of nephrotic syndrome?
Proteinuria >3.5 g/day
Hypoalbuminemia
Edema
Hyperlipiduria
Hyperlipidemia
What makes urine “bland”?
Which nephrotic diseases have bland urine?
Bland - No casts
1* - Minimal change nephropathy
Focal GN
Membranous Nephropathy
2* - Diabetic nephropathy
Amyloidosis
A boy comes in with a rapid onset of nephrotic syndrome. Lab findings indicate normal renal function and normal complement levels. On EM foot process are fused. Diagnosis?
Minimal Change nephropathy
(Nil lesion, Lipoid nephrosis)
Secondary Causes: Hodgkin’s lymphoma
NSAIDS
Atopy/Eczema
How would you treat minimal change disease?
Predisone
Relapse - adjunctive therapy with alkylating agents (cyclosporine, cyclophosphamide, chlorambucil)
Define focal vs diffuse.
Focal - the disease is “focused” on only one part of the glomeruli (less than 50% of the glomeruli is affected)
Diffuse - the disease has diffused across the entire glomeruli (more than 50% of the glomeruli is affected)
Define segmental vs global.
Segmental - only a segment of the glomerulus is affected
Global - the glomerulus is a globe; the entire glomerulus is affected.
What is a distinctive basement membrane feature in membranous GN?
spike formation with granular deposits of IgG
FSGS is not a diagnosis but a pattern of disease. What could be the cause?
Perihilar lesion (hyperfiltration in setting of HTN)
Collapsing Variant (HIV, IV drug abuse)
Glomerular tip lesion (no HTN, *sensitive to steroids, other side of vascular pole)
Identify
Perihilar FSGS
IgM
20% of cases of membranous GN are non-idiopathic. What can it be associated with?
Malignancy (lung, stomach, breast cancer)
Infections (hep B, syphilis)
Autoimmune disease (SLE, mixed connective tissue disease)
Drugs (gold, D-penicillamine, captopril)
Membranous GN is characterized by deposits in what area of the glomerulus?
SubEPIthelial space
(subpodocyte)
How would you treat Membranous GN?
Tx: ACEi or ARB
Severe: prednisone and chlorambucil/cyclophosphamide
What would you find on 20% of diabetic nephropathy renal biopsies?
Kimmelstield-Wilson nodular glomerulosclerosis
Immune complexes (IgG + C3) are depositing in the subENDOthelial lining. What is the pattern of injury called?
Membranoproliferative GN (MPGN)
young people
HEP C
Pathology: Tram trak
(MPs going to get on the tram trak)
How would you treat MPGN?
treat underlying condition
(Type 1 - treat HepC)
Steroids - poor results
A patient comes in with ANA (anti-neutrophilic antibody) + dsDNA with multi-organ involvement. What is the cause and how would you treat it?
Systemic Lupus Erythematosus GN
Tx: steroids + cytotoxic drugs
Where can isolated hematuria (with or without proteinuria) be found?
IgA nephropathy
Hereditary (Alports, thin basement membrane)
Benign recurrent hematuria
On histology, mesangial hypercellularity, tubular atrophy and segmental sclerosis are found. There are also mesangial deposits of an Ig. Diagnosis?
IgA nephropathy
Patient comes in with oliguria, azotemia, salt and water retention with edema. BP is also high. Syndrome?
Acute nephritic syndrome
Laboratory findings indicate low serum complement, azotemia, and elevated ASO titer. Diagnosis?
Poststreptococcal Glomerulonephritis (PSGN)
RPGN can be classified in three ways which will affect treatment. What are they and what are the treatments?
AntiGBM (Goodpasture’s) - [IgG] prednisone and plasma exchange
Immune complex (MPGN, IgA, Lupus, PostGN)- treat underlying condition
Non-immune-mediated (Wegeners, microPAN) - cyclophospamide and steroids
