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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
Q

IgA nephropathy have

A

hematuria (micro or macroscopic) and follows a URI

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26
Q

membranoproliferative glomerulonephritis UA results are

A

hematuria and RC casts on UA

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27
Q

post infectious glomerulonephritis features

A

edema, hematuria, leukocyturia and HTN seen 2 weeks after skin or respiratory tract infection.

28
Q

Cryoglobulinemia type 1 is associated with

A

lymphoproliferative or hematological dx like Multiple myeloma

29
Q

Cryoglobulinemia type 2 and 3 is associated with

A

chronic HCV, HIV SLE

30
Q

clinical features of cryoglobulinemia type 1

A

asymptomatic

hyperviscosity (blurry vision)

thrombosis

(Raynaud’s syndrome)

Skin manifestations such as livedo reticularis, purpura

31
Q

complement levels with cryoglobulinemia type 1

A

normal

32
Q

complement levels with cryoglobulinemia type 2

A

low C4

33
Q

clinical features of cryoglobulinemia type 2 and 3

A

systemic fatigue, arthralgias,

renal glomerulonephritis (from Hep C containing immune complexes), HTN

Pulmonary: see dyspnea, pleurisy,

Skin: see palpable purpura and leukocytoclastic vasculitis

34
Q

Diagnosis of cryoglobulinemia is associated with

A

elevated cryoglubulins (cryocrit), ESR, CRP and autoantibodies (RF and ANA).

35
Q

what is mixed cryoglubulinemia?

A

associated with HCV and elevated LFTs

36
Q

Long term complication associated with HCV associated cryoglobulinemia?

A

high risk for developing lymphoma about 5-10 years later

37
Q

treatment of HCV associated cryoglobulinemia (Type 2/3)

A

treat hep C (eradication),

immunosuppression with prednisone and cyclophosphamide,

and/or plasmaphresis for moderate to severe cyroglobulinemia.

38
Q

purpose of getting a bone marrow biopsy for type 1 cryoglobulinemia

A

diagnose underlying hematological disorder associated with type 1 cryoglobulinemia

39
Q

mixed cryoglobulinemia generally is referred to

A

type 2 or 3 and not type 1`

40
Q

Cryoglobinemia chart

A
41
Q

When to suspect GPA?

A

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
Q

can GCA have negative ANCA antibodies

A

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
Q

if ANCA negative but suspect GCA what to do?

A

get tissue biopsy and if there’s a delay in getting biopsy at least start high dose steroids first

44
Q

tetrad of HSP or IgA vasculitis

A

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
Q

What is seen on skin biopsy of palpable purpura of Henoch Schonlein purpura?

A

see prominent IgA immune complex deposition in small vessels.

46
Q

Ig A vasculitis or Henoch Schlonlein purpura casues renal dysfunction because it

A

has IgA deposits affecting the mesangium and results in wide spectrum of renal dysfunctio nranging from asymptmatic hematuria to nephrotic syndrome with AKI

47
Q

Treatment of IgA vasculitis and nephritis

A

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
Q

clinical manifestations of IgA vasculitis

A
49
Q

If we suspect SLE renal disease we would also see these other symptoms

A

evidence of other SLE manifestations and positive ds DNA for active disease

see malar and discoid rash and not palpable purpura on skin.

50
Q

what distinguishes cryoglobulinemia from HSP?

A

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
Q

focal glomerular sclerosis is seen with

A

severe obesity

HIV,

lupus nephritis

heroin use.

seen in AA

HSP won’t have lupus or heroine use or HIV association. .

52
Q

Sensitivity related to ANCA vasculitis chart

A
53
Q

Treatment of ANCA associated vasculitdes are:

GPA

EGPA

MPA

A

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
Q

Difference between eosinophilic GPA or Churg Strauss syndrome vs ANCA associated vasculaitis with GPA?

A

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
Q

anti glomerular basement membrane associations

A

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
Q

microscopic polyangitis is

A

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
Q

what is polyarteritis nodosa

A

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
Q

what causes PAN or polyarteritis nodosa?

A

immune 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
Q

labs and imaging of PAN

A

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
Q

microscopic polyangiitis

A

involves small blood vessel inflammation and

see glomerulonephritis and pulmonary findings of: hemoptysis, diffuse alveolar hemorrhage or pulmonary fibrosis.

positive ANCA

61
Q

clinical features of PAN

A

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
Q

3 indications to do plasma exchange for the pulmonary renal syndrome for treatment:

Can do this with steroids and cyclophosphamide.

A
  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
Q

treatment of focal segmental glomerulsclerosis depends upon if it’s

A

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
Q

Treatment of all anti-GBM dx pts is with

A

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
Q

membranoproliferative glomerulonephritis is associated with

A

hepatitis C infections

see MPGN pattern on kidney biopsy.

66
Q

young female with gross hematuria for 12 hrs after having a URI a week ago. Had same thing after a marathon

A

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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