Vasculitides Flashcards
What is Vasculitis?
inflammation + necrosis of blood vessels.
Leads to damaged epithelium, increased clotting, aneurysms + fibrosis
Give 2 vasculitides affecting large vessels
Giant cell arteritis
Takayasu’s arteritis: Aorta + its main branches
Give 2 vasculitides affecting medium vessels
Polyarteritis nodosa: without glomerulonephritis
Kawasaki’s disease: mostly affect coronary arteries
How can vasculitides affecting small vessels be divided?
ANCA +ve
ANCA –ve
How are Primary vasculitides classified?
based on the MAIN VESSEL SIZE affected:
LARGE
MEDIUM
SMALL
List ANCA positive small vessel vasculitides
p-ANCA: Microscopic polyangiitis Glomerulonephritis Churg-Strauss syndrome c-ANCA: Wegener’s granulomatosis (granulomatosis with polyangiitis)
List 3 ANCA negative small vessel vasculitides
Henoch-Schonlein purpura
Goodpasture’s syndrome
Cryoglobulinaemia
What is the classic triad of involvement in Wegners granulomatosis?
Upper resp tract
Lower resp tract
Glomerulonephritis
Describe the nature of vasculitis
Can affect vessels of any organ: presentation depends on which organs are involved
Can be primary or secondary to other diseases e.g. SLE, RA, hep B + C, HIV
What causes the damage to endothelial cell walls in vasculitides?
UNKNOWN
Suggested AI origin
WBCs mistake self-antigens on endothelial cells as foreign antigens due to molecular mimicry + hence cause damage.
Immune complex deposition in the walls of blood vessels leads to inflammation
In other cases, WBCs damage cells near the endothelial cells + endothelial cells get damaged indirectly (mainly in small vessels)
List 5 risk factors for development of vasculitides
Hepatitis B: polyarteritis nodosa
Hepatitis C: mixed essential cryoglobulinaemia
pANCA: microscopic polyangiitis + Churg-Strauss
c-ANCA: Wegner’s granulomatosis
Hx of asthma, allergic rhinitis or sinusitis: Churg-Strauss
What does vessel wall damage lead to in vasculitides?
Exposure of underlying collagen + TF increasing coagulation
Weakening of walls make ANEURYSMs more likely
Healing of the walls causes scarring FIBROSIS which makes the vessels stiffer + narrower
Results in reduced blood flow to organs downstream causing ISCHAEMIA
Describe the classic patient effected by vasculitides (Takayasu’s, GCA, Granulomatosis with polyangiitis, Kawasaki)
RARE Takayasu's arteritis: JAPANESE F < 40 GCA: F > 55 GPA: middle aged M Kawasaki: Children <5, esp. boys
How are large, medium and small vessel vasculitides characterised?
Large: classic clinical patterns based on vessels affected
Medium + small vessel: multiorgan involvement + less specific clinical features
List possible features of all vasculitides based on system involved: Skin Joints GI Kidneys Lungs CVS CNS Eyes
Skin: rash, ulcers, purpura
Joint: arthralgia, arthritis
GI: abdo pain, haemorrhage, diarrhoea
Kidneys: glomerulonephritis, renal failure, HTN
Lungs: dyspnoea, cough, chest pain, haemoptysis, haemorrhage
CVS: pericarditis, coronary arteritis, myocarditis
CNS: mononeuritis multiplex, infarctions, strokes, fits, chorea, confusion, paraesthia
Eyes: retinal haemorrhage, cotton wool spots
List 4 general symptoms that may be experienced in all vasculitides
Fever
Malaise
Night sweats
Weight loss
Give 4 symptoms of GCA
Loss of vision
Jaw claudication
Headache
Scalp tenderness
Give 3 features of Takayasu’s
Affects arteries branching off from the aortic arch.
If affects parts supplying upper extremities: weak or no pulse.
If affects parts supplying the head: visual + neurological symptoms
Give features of Kawasaki’s disease
Affects coronary arteries
Can cause MI
Causes conjunctivitis, rash, fever, lymphadenopathy, strawberry tongue, swollen hands + feet
Give 6 features of Polyarteritis Nodosa
Immune cells directly damage endothelium confusing it with hep B cells
Causes transmural inflammation of entire blood vessel wall, which leads to necrosis + then fibrosis. Weak fibrosis leads to fibrotic aneurysms along vessel wall
Can occur anywhere causing organ ischaemia
Renal arteries: HTN
Mesenteric arteries: mesenteric ischaemia + severe abdo pain
Signs: typical systemic features, skin rash + punched out ulcers, microaneurysms, thrombosis, infarctions, HTN, testicular pain
What occurs in ANCA positive diseases?
B cells mistakenly target their antibodies (ANCA) against granules made by neutrophils. This causes neutrophils to release oxygen free radicals that cause indirect damage to nearby endothelial cells.
ANCAs are usually IgG + there are 2 types: cANCA + pANCA
Give 3 features of Granulomatosis with polyangiitis (Wegener’s)
Affects nasopharynx, lungs + kidneys
Nasopharynx: chronic pain from sinusitis, bloody mucous + nasal discharge, saddle nose shape
Lungs: breathing difficulties, haemoptysis, chest pain, etc
Kidneys: glomerulonephritis, decreased urine production, HTN
Give 2 features of Microscopic polyangitis
Very similar to Wegener’s but does not affect nasopharynx, only kidneys + lungs
pANCA associated rather than cANCA
Give 5 features of Eosinophilic granulomatosis with polyangiitis (Churgg-Straus syndrome)
pANCAs
Affects nasopharynx, kidneys, lungs, GI, skin, nerve + heart
Nasal discharge or stuffiness: purulent or bloody with facial pain
Associated with asthma (so WHEEZE), allergic rhinitis or sinusitis
High eosinophil count
Give 4 features of Henoch-Schonlein Purpura
Not ANCA associated
High IgA antibodies, directly target endothelial cells due to molecular mimicry
Purpura, arthritis, gut Sx (abdo pain), glomerulonephritis (haematuria), IgA deposition
Purpura is palpable: can feel it raised above skin due to fibrosis
What bloods should be investigated in vasculitides?
FBC: normocytic anaemia, high platelets, high neutrophils
High ESR/CRP
Urea + Creatinine (if renal failure)
Autoantibodies e.g. cANCA in Wegner’s
What may be seen on urinalysis in vasculitides?
Haematuria, proteinuria, red cell casts (if glomerulonephritis)
Microscopic haematuria is common for Wegner’s
Why perform pulmonary function tests for vasculitides?
Identify asthma esp for Churg-stauss
What may be seen on CXR in vasculitides patients?
Diffuse, nodular or flitting shadows
Atelectasis
Which sites may be biopsied in vasculitides?
Renal
Lung
Temporal artery: GCA is segmental so biopsy requires a long section
Why perform an echocardiogram in vasculitides?
to identify pericardial involvement
Why perform angiography in vasculitides?
to identify aneurysms
In which form of vasculitis is eosinophil count raised?
Churg-strauss
Give 2 abnormalities in blood tests in polyarteritis nodosa. What serological test would you perform?
Complement may be low
LFTs mild elevation
HBV serology
Which vasculitis is most common in children?
Kawasaki disease
Which vasculitis is associated with hepatitis B?
Polyarteritis nodosa
