vasculitis part 2

Question 1

what is wegner’s granulomatosis triad

(granulomatosis with polyangitis) ?

Answer 1

systemic necrotising vasculitis affecting :
upper airway
lungs
kidneys ( pauci immune )

Question 2

what serology would we find in wegners granulomatosis ?

Answer 2

cANCA which is the same as anti PR3

Question 3

what is the criteria for the diagnosis of wegners granulomatosis (granulomatosis with polyangitis) ?

Answer 3

nasal/oral inflammation ( saddle shaped nose )
abnormal chest radiograph
microhematuria
biopsy

Question 4

what does the renal affection in wegners granulomatosis look like ?

Answer 4

asymptomatic hematuria ( FSGN)
RPGN

Question 5

what is the triad of Churg strauss syndrome ( eosinophilic granulomatosis with polyangitis ) ?

Answer 5

asthma
eosinophilia
systemic vasculitis involving more than 2 extra pulmonary organs

Question 6

how is a diagnosis of churg strauss syndrome made ?

Answer 6

asthma
eosinophilia
mononeuropathy - mononeuritis multiplex
non-fixed pulm infiltrates
paranasal sinus abnormality
biopsy containing extravascular eosinophils

Question 7

what type of vessels does microscopic polyangitis affect ?

Answer 7

small vessels

Question 8

what does the serology in microscopic polyangitis show ?

Answer 8

pANCA positive ( anti MPO )

Question 9

what is the most common vasculitis inn adults ?

Answer 9

giant cell arterteritis

Question 10

what is the most common type of vasculitis in children ?

Answer 10

henoch-schonlein purpura

Question 11

what is henoch-schonlein purpra often preceded by ?

Answer 11

upper respiratory tract infection

Question 12

what are the commonm clinical features of henoch-schonlen purpra ?

Answer 12

symmetrical purpra on the buttocks
GI- colics melena haemetemesis
Joints - arthritis/arthralgia
renal- hematuria, proteinuria

Question 13

what is the other name for henoch-schonlen purpra ?

Answer 13

IgA vasculitis

Question 14

what would we find on biopsy of HSP ?

Answer 14

perivascular IgA deposition

Question 15

what is the management of HSP ?

Answer 15

usually self limiting
supportive therapy
corticosteroids , immunosuppressive
plasma exchange in severe cases

Question 16

what is the clinical presentation of essential mixed cryoglobulinemia ?

Answer 16

palpable purpra 
arthritis/arthralgia 
weakness 
peripheral neuropathy 
subclinical alveolitis 
Low C4

Question 17

what is the management for essential mixed cryoglobulinemia ?

Answer 17

treat the underlying condition
immunosuppression
plasma exchange

Question 18

what is the triad of Behcets ?

Answer 18

recurrent oral ulcers
genital ulcers
uveitis
pathergy

Question 19

what can Behcets be difficult to differentiate from ?

Answer 19

IBD

Question 20

what is the genetic association with Behcet?

Answer 20

HLA-B51

Question 21

what is the management of Behcets ?

Answer 21

corticosteroids
colchicine
immunosuppressants
thalidomide

Question 22

which drug could induce vasculitis ?

Answer 22

amphetamine

Question 23

what are the pulmonary manifestations of wegners granulomatosis ?

Answer 23

pulmonary nodules
pulmonary vasculitis
pulmonary haemorrhage

Question 24

what is the clinical presentation of wegners granulomatosis ?

Answer 24

granulomatosis with polyangitis.
chronic sinusitis
saddle shaped nose
epistaxis
hemoptysis
pleuritis
hematuria
proteinuria

Question 25

what are the types of cryoglobullinaemia ?

Answer 25

type I - monoclonal Ig
Type II - monoclonal with polyclonal IgG
Type III - mixed polyclonal

Question 26

what infections could cause vasculitis ?

Answer 26

meningococcal septicemia

Question 27

what is mucocutaneous lymph node syndrome ?

Answer 27

kawasaki vasculitis
( CREAM )

Question 28

what is the Relationship between vessel size and response to treatment ?

Answer 28

large vessels respond very well to steroids
small/medium vessels respond very well to cyclophosphamide and steroids