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CPR Test II > ANCA and Anti GBM antibody disease > Flashcards

ANCA and Anti GBM antibody disease Flashcards

1
Q

what are the pulmonary renal syndrome and what are the 2 subtypes

A

development of diffuse alveolar hemorrhage and glomerulonephritis

  • ANCA-associated vasculitis
  • Anti-GBM disease (goodpasture disease)
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2
Q

what is a cardiorenal syndrome?

A

Development of renal failure in setting of acute or chronic heart failure
-there are 5 types

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3
Q

what is a hepatorenal syndrome?

A

Development of renal failure in the setting of cirrhosis (portal hypertension)
-type 1 and 2

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4
Q

what are the three ANCA-Associated small vessel vasculitis

A

Microscopic polyangitis
Granulomatosis with polyangiitis
Eosinophillic granulamatosis with polyangiitis

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5
Q

what are immune complex small vessel vasculitis

A

Cryoglobulinemic vasculitis
IgA vasculitis (henoch-schonlen)
Hypocomplementemic urticarial vasculitis

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6
Q

what does ANCA stand for and what are the two types

A

Antineutrophil cytoplasmic antibodies

  • PR3 causes C-ANCA
  • MPO causes P-ANCA
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7
Q

characteristics of Granuomatosis with polyangitis?

A

necrotizing vasculitis with necrotizing granulomas affecting respiratory tract (upper or lower) with no asthma symptoms or eosiophilla

associated with PR3-ANCA (c-ANCA)

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8
Q

characteristics of Microscopic polyangiitis?

A

Nectorizing vasculitis without granulomas r asthma symptoms or eosinophilia

associated with MPO-antibody

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9
Q

Characteristics of Eosinophilic granulomatosis with polyangiitis

A

necrotizing vasculitis with necrotizing granulomas affecting respiratory tract (upper and lower) with asthma symptoms and eosinophila

typically associated with MPO antibody

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10
Q

PR3-ANCA vs MPO-ANCA which one has higher relapse rates, which has higher mortality rates?

A

PR3-ANCA disease has a higher relapse rate

MPO-ANCA disease has a higher mortality rate

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11
Q

Epidemiology of ANCA associated Vasculitis

A

AAV more common in men and Caucasians and asians than African americans

GPA more common in North America and australia
MPA more common in southern Europe and asia

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12
Q

Major clinical manifestations of ANCA associated vasculitis

A

Constitutional symptoms:
-fever, malaise, anorexia, weight loss, myalgias, and migratory arthralgias

ENT manifestations
Pulmonary cough, dyspnea and hemoptysis

Skin: palpable purpura

Renal: hematuria, proteinuria, and renal failure

Neurologic:
-Mononeuritis multiplex

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13
Q

what vasculitis is ENT symptoms more common

A

GPA: Granuomatosis with polyangitis

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14
Q

what is palpable purpura suggestive of

what is non-palapable purpura suggestive of

A

Palpable purpura: raised non blanching erythematous lesion suggestive of vasculitis

Non-palpable purpura is a non blanching erythematous lesion usually from simple hemorrhage in skin suggestive of thrombocytopenia

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15
Q

what do you need for a definitive diagnosis of ANCA associated vasculitis

A

Definitive diagnosis requires a biopsy

ANCA testing

Anti GBM antibody testing

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16
Q

what is the Treatment for ANCA associated vasculitis

A

Induction therapy:
High dose glucocorticoids + rituximab
High dose glucocorticoids + cyclophosphamide

Maintenance therapy:

  • 1st line azathioprine or mycophenolate or rituximab
  • 2nd line Methotrexate
17
Q

what must you test for to determine dosing of azathioprine in ANCA associated vasculitis

A

Thiopurine methyltransferase testing because this effects the dosage of azathioprine initially

18
Q

what are the main complications of ANCA associated Vasculitis

A

Lungs:

  • Hemoptysis from diffuse alveolar hemorrhage
  • respiratory failure

Kidneys:

  • Pauci-immune glomerulonephritis (can be rapidly progressive GN, RPGN)
  • Renal failure
19
Q

what is the epidemiology of anti-GBM disease and what is special about its distribution

A

Anti-GBM disease is a small vessel vasculitis in which antibodies are directed against the glomerular basement membrane (GBM) and alveolar basement membrane (ABM)

more common in caucasians

Bimodal distribution

  • 2nd decade of life (more likely to be male and both renal and pulmonary involvement)
  • 6th decade of life (more likely female and usually only renal involvement)
20
Q

what can Anti-GBM disease be associate with as well?

A

ANCA associated vasculitis (usually MPO)

21
Q

what is anti GBM antibodies directed at?

A

Type 4 collagen in Mature GBM specifically A3 chains

22
Q

what mutation is found in alport syndrome

A

Type 4 collage specifically mutation in the a5 chain

23
Q

what is the pathogenesis of Anti-GBM disease

A

Not fully understood

  • genetic predisposition
  • environmental trigger or infection may expose A3 chain to the immune system
  • antibodies are directed primarily at the NC1 portion of the a3 chain
24
Q

what are the classic clinical presentation for the anti-GBM disease

A

Most patients present with systemic signs and symptoms

  • fever, malaise, weight loss, and arthralgias but just for a few weeks
  • symptoms longer then suggest patient has both anti-GBM disease and ANCA positivity

Rapidly progressive GN (RPGN)
Nephritic syndrome

Diffuse alveolar hemorrhage
-dyspnea, cough, hemoptysis, pulmonary infiltrates on CXR

25
Q

what is the diagnosis of anti-GBM disease?

A

should be suspected in any patient with RPGN and nephritic syndrome, especially if there is concurrent pulmonary hemorrhage

Testing:

  • anti-GBM antibody
  • ANCA testing
  • Renal biopsy
  • crescentic glomerulonephritis with liner IgG staining along the GBM
  • chest x-ray, CT chest , and/or bronchoscopy with BAL
26
Q

what is the pathologic diagnosis of Anti-GBM?

A

demonstration by immunofluorescence of diffuse linear IgG staining along the GBMs in the setting of crescentic glomerulonephritis

27
Q

Treatment of Anti-GBM disease

A

Plasmapheresis + high dose glucocorticoids + cyclophosphamide

rituximab used in refactory disease

28
Q

blood layers in plasmapharesis

A
  • Plasma
  • Platelets
  • Buffy Coat
  • Packed RBCs

CPR Test II (6 decks)

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