Glomerular Diseases Flashcards
1
Q
glomerular diseases (or syndromes) are distinguished by: (4)
A
- clinical presentation
- urinalysis
- amount of protein in urine
- associations w/ other disease manifestations
2
Q
nephrotic syndrome is characterized by:
A
- > 3.5 g of protein in urine/ days (nephrotic range proteinuria)
- hyperlipidemia
- edema
- hypoalbuminemia
*many pts w/ high proteinuria may not have the full syndrome
3
Q
Conditions associated w/ nephrotic syndrome
A
- focal segmental glomerulosclerosis
- membranous glomerulopathy
- minimal change glomerulopathy
- DM neuropathy
4
Q
focal segmental glomerulosclerosis
A
- MC in african americans
- diagnosis made by renal biopsy
- tx: glucocorticoids and calcineurin inhibitors
5
Q
membranous glomerulopathy
A
- MC in white adults
- may be associated w/ hep B, C, malaria, syphilis, malignancy, meds, tumors, lymphomas
- diagnose by renal biopsy
- search for the cause (could be infection or malignancy)
6
Q
treatment of membranous glomerulopathy
A
- up to 30% resolve spontaneously w/i 6 months
- pts who persist after 6 mos or have thrombotic/embolic even should be treated:
- glucocorticoids; cyclophosphamide; calcineurin inhibitors
7
Q
what is the MC cause of nephrotic syndrome in children (and 10% of adults)?
A
minimal change glomerulopathy
8
Q
secondary causes of minimal change glomerulopathy
A
- meds: NSAIDs, lithium, pamidronate, interferons
- infection (viral)
- malignancy (thymoma, or hodgkin lymphoma)
9
Q
tx of minimal change disease
A
- treatment commonly works but relapse is typical
- glucocorticoids; cyclophosphamide; calcineurin inhibitors; rituximab
10
Q
Diabetic nephropathy
A
- MC cause of end stage kidney disease in US
- renal bx no idicated unless suspicion of another glomerulopathy
- tx: glycemic control
11
Q
nephritic syndrome
A
- inflammation of the glomerulus
- hematuria
- WBCs in urine
- proteinuria
12
Q
what is the hallmark in nephritic syndrome?
A
hematuria with the presence of dysmorphic RBCs in the urine w/ or w/o RBC casts
13
Q
protein amount in proteinuria
A
-can vary from a small amount to the high levels like in nephrotic syndrome
14
Q
3 pathophysiologic mechanisms in nephritic syndrome
A
- anti-GBM antibodies
- pauci-immune GN
- immune complex deposition
15
Q
pauci-immune GN is defined by what?
A
-necrotizing GN w/ few or no immune deposits
16
Q
serums complement of the immune processes
A
- anti-GBM and Pauci-immune: normal
- immune complex deposition: low levels
17
Q
conditions that cause nephritis syndrome
A
- rapidly progressive glomerularnephritis
- focal segmental glomerulosclerosis
- anti-GBM ab disease
- pauci-immune GN
- Immune complex mediated GN (IGAN) nephropathy
- lupus nephritis
- infection related GN (IRGN)
- membranoproliferative GN (MPGN)
18
Q
rapidly progressive GN (RPGN)
A
- esp common in anti-GBM disease
- renal failure occurs rapidly
- may be associated w/ sx of systemic vasculitis (arthitis, epistaxis, hemoptysis, lung hemorrhage)
19
Q
diagnosis of RPGN
A
- serum tests may be positive for ANCA
- may be positive for atibodies to anti-GBM
- immunofluorescence microscopy on bx
20
Q
tx of RPGN
A
- all those except related to infection should get high dose glucocorticoids
- usually also cyclophosphamide and or plasmaphoresis
21
Q
focal segmental glomerulosclerosis (FSGS)
A
- MC in african americans
- secondar cause: DM, HTN, obesity, SS disease, HIV, drugs
- diagnose w/ bx
22
Q
tx of FSGS
A
- only a few spontaneously resolve
- glucocorticoids and / or clacineurin inhibitors
23
Q
anti-GBM antibody disease
A
- autoimmune disease cause by antibodies directed against the noncollagenous domain of type IV collagen***
- bx: crescentric GN***
24
Q
when would you see similar pathology to anit-GBM disease?
A
goodpasture’s syndrome
25
diagnosis of anti-GBM disease
- usually see all characteristics of nephritic syndrome
- high anti-GBM antibodies
- normal complement levels
- proliferative nephritis w/ many crescents****
26
tx of anti-GBM
- immunosuppressive therapy
| - cyclophosphamide and glucocorticoids combined w/ daily plasmapheresis
27
pauci-immune GN
- cause by microscopic vessel vasculitis affecting the glomerulus w/ few or no immune deposits
- renal lesion can occur w/ or w/o a systemic vasculitits
- most patients have circulating ANCA directed against neutrophils
28
What are the 3 forms of vasculitis associated w/ Pauci?
- granulomatosis w/ polyangitis (GPA)
- microscopic polyangitis (MPA) - similar to GPA but w/o granulomas
- eosinophilic granulomatosis w/ polyangitis
29
GPA was formerly known as what?
Wegener's
30
eosinophilic granulomatosis w/ polyangitis was formally known as what?
Churg-Strauss syndrome
31
kidney manifestations of Pauci
- range from mild w/ some hematuria and proteinuria to RPGN
- systemic sx may be mild: low grade fever, fatigue ext
- could have systemic findings: leukocytoclastic vasculitis, pulm. dz, commonly have hs of asthma, pulmonary infiltrates, eosinophilia
32
diagnosis of Pauci
- more than 80% of GPA and MPA are ANCA positive
| - normal complement levels
33
tx of Pauci
- glucocorticoids and cyclophosphamide w/ or w/o plasmaphoresis
- rituximab in mild disease
34
what is the MC cause of chronic GN?
IGAN
| IgA Nephropathy
35
manifestation of IGAN
- chronic hematuria w/ or w/o proteinuria
| - may also have external sites (Henoch-Shonlein purpura) or IGA vasculitis
36
diagnosis of IGAN
renal bx
37
treatment of IGAN
- if mild not treated
| - if dangerous: ACE inhibitors or ARBs, sometimes glucogorticoids
38
lupus nephritis
- prototypical immune complex nephritis
| - immune deposits seen in all portions of the glomerulus
39
clinical manifestations of lupus nephritis
- external sx of SLE
- active lupus serology (pos. ANA) (pos anti-daDNA)
- decreased C3 C4 levels
- massive classification of the histology
40
tx of lupus nephritis
-for class 3 and 4 lesions, most pts benefit from combo immuno-suppressive therapy
41
post-strep GN is now known as?
- infection related glomerulonephritis (IRGN)
| - b/c now it's after staph and e.coli
42
in IRGN, what is the timing difference b/w different bacteria?
- strep: delayed 2 weeks
| - staph and e.coli: simultaneous
43
IRGN
- immune complex medicated disease
| - the antigen in the immune complex originates w/ the infectious organism
44
clinical manifestation of IRGN
-usually present w/ acute nephritic syndrome
45
diagnosis of IRGN
-usually requires bx to distinguish from other causes
46
tx of IRGN
tx underlying infection
47
membranoproliferative GN (MPGN)
-the pathology is in the mesangium and endocapillary proliferation along w/ thickening of the basement membrane
48
diagnosis of MPGN
bx
49
tx of MPGN
tx of the underlying pathology
50
cryoglobulinemia
- immune related proteins that precipiate at temps below 37 degrees
- in vitro may be associated w/ a systemic inflammatory syndrome involving sm. - med. vessel vasculitis
51
manifestations of cryoglobulinemia
can present w/ mild proteinuria to full blown nephrotic syndrome
52
type 1 cryoglobulinemia
monoclonal IgG
53
type 1 cryoglobulinemia was formerly known as what?
waldonstrom's macroglobulinemia
54
type II cryoglobulinemia
polyclonal IgG associated w/ hep C
55
type III cryoglobulinemia
polyclonal IgG and IgM associated w/ infections, including hep C and autoimmune diseases
