Vasculitides and amyloidosis

Question 1

Large vessel

Answer 1

Takayasu’s:
Pulseless disease, Japanese women
Bruits, claudication

Temporal arteritis

Question 2

Medium vessel

Answer 2

PN:
Renal involvement, spares lungs, 30% have hep b
Microaneurysms on angiography

Kawasaki’s

Beurger’s disease:
Heavy smokers, men <35
Corkscrew appearance on angiogram

Question 3

Small vessel

Answer 3

Wegener's:
URT - saddle nose
LRT - pulmonary haemorrhage
Kidneys - crescentic GN
cANCA (anti proteinase-3)

Churg Strauss:
Asthma, eosinophilia
pANCA (anti-MPO)

Microscopic polyangitis:
Pulmonary renal syndrome (pulmonary haemorrhage and GN). pANCA

HSP:
IgA mediated, children, preceding URTI
GN

Question 4

Primary amyloidosis

Answer 4

AL, most common

Ass w/ plasma cell dycrasias and paraproteins e.g. MM

Question 5

Secondary amyloidosis

Answer 5

AA

Acute phase protein therefore secondary to chronic infections/inflammation

e.g. AI diseases: RA, alk stond, IBD
e.g. chronic infections: TB, osteomyelitis, IVDU
Non-immune: renal cell carcinoma, Hodgkin’s

Question 6

Haemodialysis associated

Answer 6

Deposition of beta 2 microglobulin

Question 7

Familial amyloidosis

Answer 7

Most common = familial Mediterranean fever

Lots of IL1
Pyrin

Question 8

Amyloidosis features

Answer 8

Kidney: nephrotic syndrome

Heart: conduction defects

Liver/spleen: hepatosplenomegaly

Tongue: macroglossia

Neuropathies: carpal tunnel