Pulmonary Renal Vasculitities Flashcards
what are the two primary s/s of pulmonary-renal syndromes?
diffuse alveolar hemorrhage
glomerulonephritis
What two umbrella conditions fall under Pulmonary-renal syndrome?
ANCA-associated vasculitis
Goodpasture’s
ANCA associated vasculitis includes which condisions and effects which vessels?
small vessels (arteries, arterioles, caps, venules, veins)
Microscopic polyangitis
granulomatosis with polyangitis (Wegners)
Eosinophilic granulomatosis with polyangitis (Churg-straus)
PR3 causes which ANCA pattern?
MPO causes which ANCA pattern?
C-ANCA (higher relapse rates)
P-ANCA (higher mortality rates)
Granulomatosis with Polyangitis (GPA/Wegners) includes which sx?
necrotizing vasculitis and granulomas affecting upper and lower respiratory track and kidneys
PR3-C-ANCA
Microscopic polyangitis causes which sx?
necrotizing vasculitis without granulomas
MPO-P-ANCA
Eosinophilic granulomatotsis with polyangitis causes which sx?
necrotizing granulomas and vasculitis affecting upper and lower respiratory and kidney with asthma sx and eosinophils
MPO-P-ANCA
What is the epidemiology for ANCA assocaited vasculitis?
uncommon
older men, white and Asian
GPA common in northern europe and asutralia
MPA more common ins southern europe and asia
common symptoms to all ANCA vascilities
consititutional sx
ENT sx (GPA)
cough, dyspnea, hemoptysis
palpable purpura, livedo reticularis
hematuria, proteinuria, renal failure
mononeuritis mulitplex
palpable purpura is usually due to what?
non-palpable purpra is usually due to what?
vasculitis
thrombocytopenia
How is a definitive diagnosis of ANCA vasculitis made?
What are some other commonly ordered tests?
Biopsy required for definitive diagnosis
also order ANCA testing, CXr or CT for all pt’s with pulmonary sx
What is induction therapy for ANCA vaculitis?
high dose glucocorticoids + Rituximab
high dose glucocorticoids + Cyclophosphamide
What is maintenance therapy for ANCA vasculitis?
What must be checked before starting Azathioprine?
1st line: Azathioprine or mycophenaolate or rituximab
2nd line: methotrexate
TPMT levels
what are the main pulmonary and renal complications of ANCA vasculitis?
hemoptysis from DAH and repsiratory failure
pauci-immune glomerulonephritis and renal failure
What is Goodpastures disease?
Anti-GBM disease is a small vessel vasculitis in which antibodies are directed against the glomerular basement membrane and alveolar basement membrane
what is the epidemiology of Goodpasture’s?
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)
Anti-GBM disease involves ab against which part of type 4 collagen in the lamina densa?
alport syndrome typically involves mutation in which chain?
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
What is the classic presentation of goodpasture’s?
fever, malaise, weight loss, arthralgias for weeks
RPGN and nephritic syndrome
diffuse alveolar hemorrhage
A pt presenting with RPGN and nephritic syndrome along with pulmonary hemorrhage, what should be suspected?
anti-GBM disease
test for the ab, ANCA testing, renal biopsy, CXR/CT and or bronchoscopy with BAL
how is a pathologic diagnosis of anti-GBM made?
requires demonstration by immunoflurescence of diffuse linear IgG staining along the GBMs in the setting of crescentric glomerulonephritis
what is the treatment for Anti-GBM disease?
plasmapheresis + high dose glucocorticoids + cyclophosphamide
how does centrifuged blood layer?
less dense are closer to axis of rotation
most dense are farthest from axis of rotation
- plasma
- plt
- buffy coat
- packed RBCs
what are the main complications of anti-gbm disease?
DAH
respiratory failure
crescentic RPGN
