Vasculitides Flashcards
pathophysiology of mixed cryoglobulinaemia
cryoglobulins are Ig that agglutinate in the cold
vasculitis of small vessels due to mixed IgG and IgM immune cmplex deposition
usually triggered by viral infection i.e. HCV
presentation of mixed cryoglobulinaemia
triad of arthralgia, palpable purpura and weakness
can also have peripheral neuropathy and glomerulonephritis
what can be the cause of stomach pain in a child with IgA vasculitis (HSP)
intususception
presenting features of IgA vasculitis (HSP)
hinge pain (arthralgia)
stomach pain (intususcepion)
palpable purpura
what antibodies are positive in microscopic polyangitis
pANCA
MPO (myeloperoxidase)
microscopic polyangitis presenting features
similar ot granulomatosis with polyangitis but without nasopharyngeal involvement
vasuclitis involving skin, kidneys and lung
skin: pupura
kidneys: haematuria, red cell casts
lung: haemoptysis, cough, dyspnoea
whats the difference between microscopic polyangitis and granulomatosis with polyangitis
they present similar but….
microscopic polyangitis has positive pANCA + MPO and doesnt have nasopharyngeal involvement
granulomatosis with polyangitis has positive cANCA + PR3 and nasopharyngeal involvement
CXR features of granulomatosis with polyangitis
large nodular opacities
(necrotizing granulomas)
what small vessel disease can cause foot/wrist drop
eosinophillic granulomatosis with polyangitis
antibodies positive in granulomatosis with polyangitis
cANCA + PR3
antibodies positive in eosinophillic granulomatosis with polyangitis
pANCA + MPO
pathophysiology of small vessel vasculitis
occurs in adults > 40
develops 7-10 days after exposure to certain medications (e.g. penicillins, cephalosporins, allopurinol etc) or infections (i.e. HIV, HCV)
immune complex mediated leukocytoclastic vasculitis
causes palpable purpura
male > 40 with diagnosis of hcv presents with palpable purpura + no other symptoms
?diagnosis
small vessel vasculitis
male > 40 with diagnosis of HCV presents with palpable purpura, haematuria, arthralgia and abdominal pain.
?diagnosis
polyarteritis nodosa
what HLA antigen is associated with behcets syndrome
HLA B51
features of behcets syndrome
medium sized vessel vasculitis
HLA B51
oral and genital ulcers, uveitis, erythema nodosa
what can be associated with the development of behcets syndrome
HCV
parvovirus
different stages of transmural inflammation with fibrinoid necrosis
polyarteritis nodosa
pearl beads appearance on arteriogram
polyarteritis nodosa
multiple renal microaneurysms and spasms
presenting features of polyarteritis nodosa
PAN affects the SKIN
skin - pupura
kidneys
intestines
neurological
+ fever, headache, malaise, weight loss, arthralgia
presenting features of kawasakis disease
kawasaki’s CRASH and BURN
conjunctivitis
rash
adenopathy
strawberry red tongue
hand-mouth
burn (fever > 5 days)
segmental thrombosing vasculitis with nerve and vein involvement
Buerger’s disease
presenting features of takayatu’s arteritis
weak pulses of upper extremeties
fever, night sweats, arthralgia, myalgia, skin nodules, occular disturbances
granulomatous thickening of aortic arch a great proximal vessels
takayasu’s vasculitis
