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

Question 1

Pulmonary-renal syndrome is characterized by development of?

Answer 1

Diffuse alveolar hemorrhage and glomerulonephritis

Question 2

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

Answer 2

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

Question 3

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

MPO (myeloperoxidase) causes what type of staining pattern?

Answer 3

1) C-ANCA

2) P-ANCA

Question 4

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

Answer 4

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

Question 5

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

Which antibody is it associated with?

Answer 5

1) Microscopic polyangiitis

2) MPO-antibody

Question 6

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?

Answer 6

1) Eosinophilic granulomatosis with polyangiitis

2) MPO-antibody

Question 7

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?

Answer 7

1) Granulomatosis with polyangiitis

2) PR3-antibody

Question 8

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

Which has higher mortality rates?

Answer 8

1) PR3-ANCA

2) MPO-ANCA

Question 9

Which AAV is more common in Northern Europe and Australia?

Which is more common in Southern Europe and Asia?

Answer 9

1) GPA

2) MPA

Question 10

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

Answer 10

1) Fever
2) Malaise
3) Weight loss

Question 11

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

Answer 11

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

Question 12

ENT symptoms are more common in which AAV?

Answer 12

GPA

Question 13

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

Answer 13

1) Cough
2) Dyspnea
3) Hemoptysis

Question 14

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

Answer 14

1) Palpable purpura

2) Livedo reticularis

Question 15

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

Answer 15

1) Hematuria
2) Proteinuria
3) Renal failure

Question 16

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

Answer 16

Mononeuritis multiplex

Question 17

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?

Answer 17

1) Vasculitis

2) Thrombocytopenia

Question 18

Definitive diagnosis of AAV requires?

Answer 18

Renal or skin biopsy

Question 19

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

Answer 19

1) High dose glucocorticoids + Rituximab

2) High dose glucocorticoids + Cyclophosphamide

Question 20

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?

Answer 20

Rituximab

Question 21

What is the first line maintenance therapy regimen for AAV?

The second line?

Answer 21

1) Azathioprine or Mycophenolate or Rituximab

2) Methotrexate

Question 22

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

Answer 22

Thiopurine methyltransferase (TPMT)

Question 23

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

Answer 23

1) Glomerular basement membrane (GBM)

2) Alveolar basement membrane (ABM)

Question 24

Anti-GBM disease is most common in which ethnicity?

Answer 24

Caucasians

Question 25

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

Answer 25

MPO

Question 26

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

Answer 26

Type 4 collagen

Question 27

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?

Answer 27

1) The α3 chain

2) NC1 portion

Question 28

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

Answer 28

They have both Anti-GBM disease and ANCA positivity

Question 29

80-90% of Anti-GBM patients present with what renal manifestations?

40-60% will have what pulmonary manifestation?

Answer 29

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

Question 30

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

Answer 30

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

Question 31

What is the standard treatment of Anti-GBM Disease?

Answer 31

1) Plasmapheresis
2) High dose glucocorticoids
3) Cyclophosphamide