Renal Clinical Medicine Part 5: Pulmonary Renal Syndromes (Selby) Flashcards

1
Q

Pulmonary-renal syndrome is characterized by development of?

A

Diffuse alveolar hemorrhage and glomerulonephritis

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2
Q

ANCA-associated Vasculitis (aka Pauci-immune vasculitis) is defined as?

A

A necrotizing vasculitis of small vessels with few or no immune deposits present

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3
Q

PR3 (serine protease 3) causes what type of staining pattern?

MPO (myeloperoxidase) causes what type of staining pattern?

A

1) C-ANCA

2) P-ANCA

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4
Q

ANCA-associated Vasculitis is divided into what 3 clinical diseases?

A

1) Granulomatosis with polyangiitis
2) Microscopic polyangiitis
3) Eosinophilic granulomatosis with polyangiitis

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5
Q

Which ANCA-associated Vasculitis is a necrotizing vasculitis without granulomas or asthma symptoms or eosinophilia?

Which antibody is it associated with?

A

1) Microscopic polyangiitis

2) MPO-antibody

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6
Q

Which ANCA-associated Vasculitis is a necrotizing vasculitis with necrotizing granulomas affecting respiratory tract with asthma symptoms and eosinophilia?

Which antibody is it associated with?

A

1) Eosinophilic granulomatosis with polyangiitis

2) MPO-antibody

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7
Q

Which ANCA-associated Vasculitis is a necrotizing vasculitis with necrotizing granulomas affecting respiratory tract with no asthma symptoms
or eosinophilia?

Which antibody is it associated with?

A

1) Granulomatosis with polyangiitis

2) PR3-antibody

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8
Q

Between MPO-ANCA disease and PR3-ANCA disease, which has higher relapse rates?

Which has higher mortality rates?

A

1) PR3-ANCA

2) MPO-ANCA

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9
Q

Which AAV is more common in Northern Europe and Australia?

Which is more common in Southern Europe and Asia?

A

1) GPA

2) MPA

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10
Q

What are some constitutional symptoms associated with ANCA-associated Vasculitis?

A

1) Fever
2) Malaise
3) Weight loss

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11
Q

What are some ENT manifestations associated with ANCA-associated Vasculitis?

A

1) Nasal crusting
2) Sinusitis
3) Saddle nose deformity

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12
Q

ENT symptoms are more common in which AAV?

A

GPA

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13
Q

What are some pulmonary manifestations associated with ANCA-associated Vasculitis?

A

1) Cough
2) Dyspnea
3) Hemoptysis

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14
Q

What are some skin manifestations associated with ANCA-associated Vasculitis?

A

1) Palpable purpura

2) Livedo reticularis

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15
Q

What are some renal manifestations associated with ANCA-associated Vasculitis?

A

1) Hematuria
2) Proteinuria
3) Renal failure

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16
Q

What are some neurologic manifestations associated with ANCA-associated Vasculitis?

A

Mononeuritis multiplex

17
Q

Palpable purpura is raised non-blanching erythematous lesion suggestive of?

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

A

1) Vasculitis

2) Thrombocytopenia

18
Q

Definitive diagnosis of AAV requires?

A

Renal or skin biopsy

19
Q

What are the induction therapy regimens for the treatment of AAV?

A

1) High dose glucocorticoids + Rituximab

2) High dose glucocorticoids + Cyclophosphamide

20
Q

Between Rituximab or Cyclophosphamide, which is considered safer because the other has lots of side effects including sterility, bone marrow suppression, and hemorrhagic cystitis/bladder cancer?

A

Rituximab

21
Q

What is the first line maintenance therapy regimen for AAV?

The second line?

A

1) Azathioprine or Mycophenolate or Rituximab

2) Methotrexate

22
Q

What levels do you want to check prior to starting azathioprine therapy due to genetic variation in the enzyme?

A

Thiopurine methyltransferase (TPMT)

23
Q

Anti-GBM disease is a small vessel vasculitis in which antibodies are directed against?

A

1) Glomerular basement membrane (GBM)

2) Alveolar basement membrane (ABM)

24
Q

Anti-GBM disease is most common in which ethnicity?

A

Caucasians

25
Q

Anti-GBM disease can be associated with which ANCA-associated vasculitis more commonly?

A

MPO

26
Q

Besides laminin, what is the Lamina densa composed of in mature GBM?

A

Type 4 collagen

27
Q

Anti-GBM disease involves antibodies directed against which chain of the type 4 collagen in the lamina densa?

What particular portion of the chain is it directed at?

A

1) The α3 chain

2) NC1 portion

28
Q

If an Anti-GBM patient presents with fever, malaise, weight loss, and arthralgia for longer than a few weeks, this suggests?

A

They have both Anti-GBM disease and ANCA positivity

29
Q

80-90% of Anti-GBM patients present with?

40-60% will have?

A

1) Rapidly progressive glomerulonephritis (RPGN) and Nephritic syndrome
2) Diffuse alveolar hemorrhage

30
Q

A pathologic diagnosis of anti-GBM disease requires the demonstration by immunofluorescence of?

A

Diffuse linear IgG staining along the GBMs in the setting of crescentic glomerulonephritis

31
Q

What is the standard treatment of Anti-GBM Disease?

A

1) Plasmapheresis
2) High dose glucocorticoids
3) Cyclophosphamide