Pulmonary Renal Vasculitities

Question 1

what are the two primary s/s of pulmonary-renal syndromes?

Answer 1

diffuse alveolar hemorrhage

glomerulonephritis

Question 2

What two umbrella conditions fall under Pulmonary-renal syndrome?

Answer 2

ANCA-associated vasculitis

Goodpasture’s

Question 3

ANCA associated vasculitis includes which condisions and effects which vessels?

Answer 3

small vessels (arteries, arterioles, caps, venules, veins)

Microscopic polyangitis

granulomatosis with polyangitis (Wegners)

Eosinophilic granulomatosis with polyangitis (Churg-straus)

Question 4

PR3 causes which ANCA pattern?

MPO causes which ANCA pattern?

Answer 4

C-ANCA (higher relapse rates)

P-ANCA (higher mortality rates)

Question 5

Granulomatosis with Polyangitis (GPA/Wegners) includes which sx?

Answer 5

necrotizing vasculitis and granulomas affecting upper and lower respiratory track and kidneys

PR3-C-ANCA

Question 6

Microscopic polyangitis causes which sx?

Answer 6

necrotizing vasculitis without granulomas

MPO-P-ANCA

Question 7

Eosinophilic granulomatotsis with polyangitis causes which sx?

Answer 7

necrotizing granulomas and vasculitis affecting upper and lower respiratory and kidney with asthma sx and eosinophils

MPO-P-ANCA

Question 8

What is the epidemiology for ANCA assocaited vasculitis?

Answer 8

uncommon

older men, white and Asian

GPA common in northern europe and asutralia

MPA more common ins southern europe and asia

Question 9

common symptoms to all ANCA vascilities

Answer 9

consititutional sx

ENT sx (GPA)

cough, dyspnea, hemoptysis

palpable purpura, livedo reticularis

hematuria, proteinuria, renal failure

mononeuritis mulitplex

Question 10

palpable purpura is usually due to what?

non-palpable purpra is usually due to what?

Answer 10

vasculitis

thrombocytopenia

Question 11

How is a definitive diagnosis of ANCA vasculitis made?

What are some other commonly ordered tests?

Answer 11

Biopsy required for definitive diagnosis

also order ANCA testing, CXr or CT for all pt’s with pulmonary sx

Question 12

What is induction therapy for ANCA vaculitis?

Answer 12

high dose glucocorticoids + Rituximab

high dose glucocorticoids + Cyclophosphamide

Question 13

What is maintenance therapy for ANCA vasculitis?

What must be checked before starting Azathioprine?

Answer 13

1st line: Azathioprine or mycophenaolate or rituximab

2nd line: methotrexate

TPMT levels

Question 14

what are the main pulmonary and renal complications of ANCA vasculitis?

Answer 14

hemoptysis from DAH and repsiratory failure

pauci-immune glomerulonephritis and renal failure

Question 15

What is Goodpastures disease?

Answer 15

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

Question 16

what is the epidemiology of Goodpasture’s?

Answer 16

rare, more common in caucasions

bimodal distrubition

2nd decade-male and pulmonary/renal involvement

6th decade-female and renal involvment

can be assx with ANCA vasculitis (MPO)

Question 17

Anti-GBM disease involves ab against which part of type 4 collagen in the lamina densa?

alport syndrome typically involves mutation in which chain?

Answer 17

against the a3 chain of type 4 collagen in the lamina densa

the a3 chain is found in the glomerular and alveolar basement membranes

the a5 chain

Question 18

What is the classic presentation of goodpasture’s?

Answer 18

fever, malaise, weight loss, arthralgias for weeks

RPGN and nephritic syndrome

diffuse alveolar hemorrhage

Question 19

A pt presenting with RPGN and nephritic syndrome along with pulmonary hemorrhage, what should be suspected?

Answer 19

anti-GBM disease

test for the ab, ANCA testing, renal biopsy, CXR/CT and or bronchoscopy with BAL

Question 20

how is a pathologic diagnosis of anti-GBM made?

Answer 20

requires demonstration by immunoflurescence of diffuse linear IgG staining along the GBMs in the setting of crescentric glomerulonephritis

Question 21

what is the treatment for Anti-GBM disease?

Answer 21

plasmapheresis + high dose glucocorticoids + cyclophosphamide

Question 22

how does centrifuged blood layer?

Answer 22

less dense are closer to axis of rotation

most dense are farthest from axis of rotation

• plasma
• plt
• buffy coat
• packed RBCs

Question 23

what are the main complications of anti-gbm disease?

Answer 23

DAH

respiratory failure

crescentic RPGN