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Nephrology > Glomerulonephritis and pulm renal syndrome > Flashcards

Glomerulonephritis and pulm renal syndrome Flashcards

GPA, Good pastures

1
Q

anti glomerular basement membrane dx clinical features / which systems are involved

A

pulmonary: hemoptysis renal: nephritic syndrome

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2
Q

Granulomatosis with poly angiitis clinical features / which systems are involved?

A

pulmonary: hemoptysis and cavitary lesions
renal: nephritic syndrome

ENT: sinusitis, cartilaginous destruction

Skin: leukocytoclastic angiitis with purpura

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3
Q

are there systemic symptoms with anti glomerular basement membrane

A

no

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4
Q

are there systemic symptoms with GPA?

A

yes - fevers, chills, weight loss and night sweats

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5
Q

lab and imaging findings with anti glomerular basement membrane

A

CXR: pulmonary infiltrates

renal biopsy: linear deposits of IgG will have POSITIVE anti glomerular basement membrane antibodies

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6
Q

lab and imaging findings with GPA?

A

CXR: nodules, cavitations, transient infiltrates

Biopsy: granulomatous inflammation in artery and perivascular area ANCA positivity

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7
Q

Treatment of anti glomerular basement membrane

A

plasmaphresis with glucocorticoid cyclophosamide

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8
Q

Treatment of GPA

A

glucocorticoid + cyclophosphamide or ritixumab

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9
Q

SLE presentation with renal dx

A

see nephritic syndrome and rarely hemoptysis. But look for dsDNA positivity.

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10
Q

What is also known as anti glomerular basement membrane

A

Goodpasture’s syndrome - see progressive glomerulonephritis, renal failure and pulmonary hemorrhage.

NO systemic symptoms.

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11
Q

Focal segmental glomeruloscerosis presents as

A

nephrotic syndrome

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12
Q

nephrotic syndrome is

A

proteinura >3.5 g/day, see generalized edema and hypoalbuinemia, and hyperlipidemia

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13
Q

who gets focal segmental glomeruloscerosis (FSGS)?

A

HIV

uncontrolled HTN pts

obese

AA

heroin users

See low albumin, hyperlipidemia, renal insufficiency, and proteinuria

renal biopsy will show sclerosis at renal capillary tufts

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14
Q

microscopic polyangiitis MPA is associated with

A

pANCA and MPO

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15
Q

GPA antibiodies

A

GPA with cANCA

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16
Q

Treatment of GPA is with

A

methylprednisolone and (cyclophosphamide, Can use rituximab and rarely methotrexate)

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17
Q

When do we use plasmapheresis for treatment of GPA?

A

if there’s lifethreatening pulmonary hemorrhage would do plasmapheresis with methylprednisolone and cyclophosphamide or rituximab

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18
Q

focal segmental glomerulosclerosis is associated with:

A

severe obesity

heroin use

HIV

>50% are seen in AA reflux nephropathy

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19
Q

membranoproliferative GN is associated with

A

autoimmune disorders (SLE, Sjogren’s, RA)

infections (Hep B and C)

mixed cryoglobulinemias

monoclonal gammopathies

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20
Q

membranous GN is associated with

A

75% is all idiopathic

Seen with SLE drugs (penicilliamine, gold NSAIDS)

infections (Hep B and C or syphilis)

malignancy (lung, breast, GI)

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21
Q

minimal change dx is seen with (medication? and medical condition?)

A

drugs (NSAIDS) lymphomas

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22
Q

HIV associated nephropathy is associated with

A

FSGS especially with low CD4 counts. But this can occur still in pts who are on ART and well controlled HIV

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23
Q

Focal segmental glomerulosclerosis lab and imaging findings.

A

See large echogenic kidneys on ultrasound and few RBCs and WBCs on urinalysis, heavy proteinuria and hypoalbuminemia.

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24
Q

how to stop the progression of focal segmental glomerulosclerosis even in pts with controlled HIV?

A

HAART, ACE i or ARBs to reduce proteinuria and some cases steroids

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25
IgA nephropathy have
hematuria (micro or macroscopic) and follows a URI
26
membranoproliferative glomerulonephritis UA results are
hematuria and RC casts on UA
27
post infectious glomerulonephritis features
edema, hematuria, leukocyturia and HTN seen 2 weeks after skin or respiratory tract infection.
28
Cryoglobulinemia type 1 is associated with
lymphoproliferative or hematological dx like Multiple myeloma
29
Cryoglobulinemia type 2 and 3 is associated with
chronic HCV, HIV SLE
30
clinical features of cryoglobulinemia type 1
asymptomatic hyperviscosity (blurry vision) thrombosis (Raynaud's syndrome) Skin manifestations such as livedo reticularis, purpura
31
complement levels with cryoglobulinemia type 1
normal
32
complement levels with cryoglobulinemia type 2
low C4
33
clinical features of cryoglobulinemia type 2 and 3
systemic fatigue, arthralgias, renal glomerulonephritis (from Hep C containing immune complexes), HTN Pulmonary: see dyspnea, pleurisy, Skin: see palpable purpura and leukocytoclastic vasculitis
34
Diagnosis of cryoglobulinemia is associated with
elevated cryoglubulins (cryocrit), ESR, CRP and autoantibodies (RF and ANA).
35
what is mixed cryoglubulinemia?
associated with HCV and elevated LFTs
36
Long term complication associated with HCV associated cryoglobulinemia?
high risk for developing lymphoma about 5-10 years later
37
treatment of HCV associated cryoglobulinemia (Type 2/3)
treat hep C (eradication), _immunosuppression with prednisone and cyclophosphamide_, and/or _plasmaphresis_ for moderate to severe cyroglobulinemia.
38
purpose of getting a bone marrow biopsy for type 1 cryoglobulinemia
diagnose underlying hematological disorder associated with type 1 cryoglobulinemia
39
mixed cryoglobulinemia generally is referred to
type 2 or 3 and not type 1`
40
Cryoglobinemia chart
41
When to suspect GPA?
2 or more of the following: nasal or oral inflammation, abnormal CXR, abnormal urinary sediment, granulomatous inflammation on biopsy of artery or perivascular area. pathogenesis is from ANCA antibodies react with 1 or 2 proteins PR3 or MPO on surface of cytokine stimulated WBCs.
42
can GCA have negative ANCA antibodies
yes. Although 90% of pts are positive. to diagnose need tissue biopsy at the site of active disease (skin, kidney, lung) to make difinitive diagnosis. Kidney biospy will show pauci immune segmental necrotizing glomerulonephritis with few or no immune deposits on electron microscopy.
43
if ANCA negative but suspect GCA what to do?
get tissue biopsy and if there's a delay in getting biopsy at least start high dose steroids first
44
tetrad of HSP or IgA vasculitis
palpable purpura, raised multiple erythematous spots, non blanching in lower legs) abdominal pain arthritis renal involvement in form of glomerulonephritis Most pts may not have tetrad of symptoms.
45
What is seen on skin biopsy of palpable purpura of Henoch Schonlein purpura?
see prominent IgA immune complex deposition in small vessels.
46
Ig A vasculitis or Henoch Schlonlein purpura casues renal dysfunction because it
has IgA deposits affecting the mesangium and results in wide spectrum of renal dysfunctio nranging from asymptmatic hematuria to nephrotic syndrome with AKI
47
Treatment of IgA vasculitis and nephritis
Treatment is supportive and steroids are used in pts who have severe renal dx (\>750 mg/day of proteinuria or nephrotic syndrome Adults are more likely than kids to expereince progressive renal decline.
48
clinical manifestations of IgA vasculitis
49
If we suspect SLE renal disease we would also see these other symptoms
_evidence of other SLE manifestations_ and **positive ds DNA** for active disease see malar and discoid rash and not palpable purpura on skin.
50
what distinguishes cryoglobulinemia from HSP?
_cryoglobulinemia is associated with peripheral neuropathy_ and abdominal pain is less common. _HSP will have no peripheral neuropathy_ and have abd pain (generally) Cryoglobulinemia and HSP will both have palpable purpura, renal disease and arthralgia.
51
focal glomerular sclerosis is seen with
severe obesity HIV, lupus nephritis heroin use. seen in AA HSP won't have lupus or heroine use or HIV association. .
52
Sensitivity related to ANCA vasculitis chart
53
Treatment of ANCA associated vasculitdes are: GPA EGPA MPA
treatment involves a induction and maintainence phase **induction therapy**: high dose methylprednisolone for 3-5 days combined with either cyclophosphamide or rituximab +/- plasmapheresis. **Maintenance therapy:** cyclophosamide, mycophenolate moeftil, azathioprine, or methotrexate. EGPA can get biologic agent = mepolizumab
54
Difference between eosinophilic GPA or Churg Strauss syndrome vs ANCA associated vasculaitis with GPA?
_Churg strauss_ - will have **poorly controlled asthma** and **peripheral eosinophilia\>10**% and **mononeuritis multiplex**. Will also have **migratory pulmonary infiltrates** _GPA-_ will have **eye symptoms**, oral ulcers, and **upper sinus symptoms**: epistaxis, sinusitis, otitis media, hoarseness, well controlled asthma and **pulmonary cavitary lesions, infiltrates, and pleural effusion**
55
anti glomerular basement membrane associations
_Good pasture's dx_ is _antiglomerular basement dx_- see hemoptysis and glmoerulonephritis. **No systemic symptoms** (fever, malaise, weight loss, arthralgia). CXR will have **patchy basilar infiltrates no cavitation** and no pleural effusion. Good pastures .=/= MPA or micropolyangiitis.
56
microscopic polyangitis is
not the same as polyarteritis nodosa or PAN and NOT The same as Goodpastures. microscopic polyangitis - hematuria, hemoptysis, constitutional symptoms and on biopsy see GN and positive for PANCA and MPO. Tx with steroids and rituxman or cyclophosphamide.
57
what is polyarteritis nodosa
seen with hep B, hep C and hairy cell leukemia has constitutional symptoms and see testicular pain, abdominal pain, and mononeuritis multiplex too. **NO lung involvement** **NO glomerulonephritis** just tender subcutaneous nodules and biopsy of affected organ- shows panarteritis and transmural necrosis and homogenous eosinophilic appearance of blood vessel (fibrinoid necrosis) Treat with steroids and cytotoxic meds cyclophosphamide. HBV associated PAN- with steroids and plasma exchange followed by antiviral therapy
58
what causes PAN or polyarteritis nodosa?
i**mmune mediated, medium sized vessel damage** (sparing of thearterioles and capillaries) with eventual narrowed lumen and reduced blood flow and thrombosis of affected vessels leading to tissue infarction and or weakened vessel walls leading to aneurysmal formation and rupture this causes abdominal pain, Tx is steroids and cyclophosphamide.
59
labs and imaging of PAN
negative ANCA mesenteric angiogram should be done to look for beaded aneurysms of mesenteric circulation. positive hep B or C. renal biopsy: shows vasculitis and nongranulomatous
60
microscopic polyangiitis
involves small blood vessel inflammation and see glomerulonephritis and pulmonary findings of: hemoptysis, diffuse alveolar hemorrhage or pulmonary fibrosis. positive ANCA
61
clinical features of PAN
see **erythema nodosum**, **purpura,** or **livedo reticularis.** **seen with Hep B and C** and **hairy cell leukmia** see **mononeuritis multiplex causing sensory or motor deficits** in radial, ulnar, or peroneal distribution with foot drop or wrist drop. see abdominal pain - worse after meals **"intestinal angina"** can **see myocardial ischemia too** ***_DOESN'T involve the lungs._***
62
3 indications to do plasma exchange for the pulmonary renal syndrome for treatment: Can do this with steroids and cyclophosphamide.
1. **advanced kidney dysfunction** due to dx (Cr \>4) or need for dialysis 2. **positive anti GBM antibody** 3. **pulmonary hemorrhage** that is life threatening or doesn't respond to steroids.
63
treatment of focal segmental glomerulsclerosis depends upon if it's
**primary or secondary** **primary FSGS**- most common presentation is acute or subacute onset of nephrotic syndrome with associated peripheral edema **Secondary FSGS-** gradual proteinuria that often falls in the non nephrotic range and serum albumin levels are normal and no peripheral edema Treatment is with immunosupression and so first start with steroids if they have proteinuria \> 3.5g/day and hypoalbuminemia 3.5
64
Treatment of all anti-GBM dx pts is with
**plasmapheresis** unlike other ANCA associated glomerulonephritis plasmapheresis for other ANCA associated glomerulonephritis is - alveolar hemorrhage or severe kidney dx needing HD or serum Cr \>5.8
65
membranoproliferative glomerulonephritis is associated with
hepatitis C infections see MPGN pattern on kidney biopsy.
66
young female with gross hematuria for 12 hrs after having a URI a week ago. Had same thing after a marathon
**IgA nephropathy** If imaging is clear then can be diagnosed by history and may forego biopsy (but definitely need a kidney biopsy) **NOT the same as IgA vasculitis** or Henoch Schonlein Purpura) - childhood vasculitis and tends to be after URI. rare in adults. **Post infectious glomerulonephritis from strep -** appears 2-3 weeks after infection.

Nephrology (26 decks)

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