vasculitis part 2 Flashcards
what is wegner’s granulomatosis triad
(granulomatosis with polyangitis) ?
systemic necrotising vasculitis affecting :
upper airway
lungs
kidneys ( pauci immune )
what serology would we find in wegners granulomatosis ?
cANCA which is the same as anti PR3
what is the criteria for the diagnosis of wegners granulomatosis (granulomatosis with polyangitis) ?
nasal/oral inflammation ( saddle shaped nose )
abnormal chest radiograph
microhematuria
biopsy
what does the renal affection in wegners granulomatosis look like ?
asymptomatic hematuria ( FSGN) RPGN
what is the triad of Churg strauss syndrome ( eosinophilic granulomatosis with polyangitis ) ?
asthma
eosinophilia
systemic vasculitis involving more than 2 extra pulmonary organs
how is a diagnosis of churg strauss syndrome made ?
asthma
eosinophilia
mononeuropathy - mononeuritis multiplex
non-fixed pulm infiltrates
paranasal sinus abnormality
biopsy containing extravascular eosinophils
what type of vessels does microscopic polyangitis affect ?
small vessels
what does the serology in microscopic polyangitis show ?
pANCA positive ( anti MPO )
what is the most common vasculitis inn adults ?
giant cell arterteritis
what is the most common type of vasculitis in children ?
henoch-schonlein purpura
what is henoch-schonlein purpra often preceded by ?
upper respiratory tract infection
what are the commonm clinical features of henoch-schonlen purpra ?
symmetrical purpra on the buttocks
GI- colics melena haemetemesis
Joints - arthritis/arthralgia
renal- hematuria, proteinuria
what is the other name for henoch-schonlen purpra ?
IgA vasculitis
what would we find on biopsy of HSP ?
perivascular IgA deposition
what is the management of HSP ?
usually self limiting
supportive therapy
corticosteroids , immunosuppressive
plasma exchange in severe cases
what is the clinical presentation of essential mixed cryoglobulinemia ?
palpable purpra arthritis/arthralgia weakness peripheral neuropathy subclinical alveolitis Low C4
what is the management for essential mixed cryoglobulinemia ?
treat the underlying condition
immunosuppression
plasma exchange
what is the triad of Behcets ?
recurrent oral ulcers
genital ulcers
uveitis
pathergy
what can Behcets be difficult to differentiate from ?
IBD
what is the genetic association with Behcet?
HLA-B51
what is the management of Behcets ?
corticosteroids
colchicine
immunosuppressants
thalidomide
which drug could induce vasculitis ?
amphetamine
what are the pulmonary manifestations of wegners granulomatosis ?
pulmonary nodules
pulmonary vasculitis
pulmonary haemorrhage
what is the clinical presentation of wegners granulomatosis ?
granulomatosis with polyangitis.
chronic sinusitis
saddle shaped nose
epistaxis
hemoptysis
pleuritis
hematuria
proteinuria
what are the types of cryoglobullinaemia ?
type I - monoclonal Ig
Type II - monoclonal with polyclonal IgG
Type III - mixed polyclonal
what infections could cause vasculitis ?
meningococcal septicemia
what is mucocutaneous lymph node syndrome ?
kawasaki vasculitis
( CREAM )
what is the Relationship between vessel size and response to treatment ?
large vessels respond very well to steroids
small/medium vessels respond very well to cyclophosphamide and steroids
