Vasculitides Flashcards
What is Vasculitis?
Inflammation of the blood vessels characterised by inflammatory infiltrate and destruction of the vessel wall and categorized based on presence of ANCA (positive or negative) and vessel size (large, medium or small).
How may Vasculitis be classified?
Vessel Size
ANCA positive or ANCA negative
State the large-vessel vasculitides.
GCA
TA
State the medium-vessel vasculitides.
PN
KD
State the small-vessel vasculitides.
GP (WS)
EGP (CSS)
MP
CV
HSP
LA
BD
Outline the pathophysiology of vasculitis
• Idiopathic (unknown cause) with antigen exposure leading to immune response -> immune-complexes mediate damage of the blood vessel wall = fibrinoid necrosis of vessel wall with karyorrhexis (nuclear fragmentation and chromatin break-up) with red blood cell extravasation
What is Takayasu’s Arteritis?
Chronic progressive, inflammatory disease of the aorta causing occlusion. Takayasu’s arteritis commonly affects young females (15-35).
A 34 year old female of SE Asian descent presents with fever, malaise and weakness. She reports pain in the calves when walking about 100m.
She has no CV risk factors, a BMI of 20 and does not smoke or drink. She exercises for 4 hours per week usually.
O/E she has tenderness on palpation of the brachial and radial arteries, a carotid bruit and loss of pulses.
Give your DDx and Tx
Takayasu’s Arteritis
• Corticosteroids: Prednisolone 40-60mg
± (Stenosis)
• Surgery: Angioplasty
What is Polymyalgia Rheymatica
Chronic inflammatory condition of the large vessels of unknown cause characterised by bilateral proximal muscle stiffness (neck and shoulders) and ache affecting > 50 age group
A 53 year old man presents with a headache present at the temples Additionally, he has morning stiffness in his shoulders lasting for 2 hours, improving as the day goes on. Additionally he has pain in the muscles.
O/E he has reduced range of movement and power 3/5 in his deltoids but is neurologically fine in sensation and motor function.
His CRP and ESR are raised and a US of his GH joint shows bursitis.
Give your DDx and Tx
Polymyalgia Rheumatica
- Corticosteroids: Prednisolone 15mg
- Bone protection: Vitamin D; Alendronic acid
What is Giant Cell Arteritis?
Transmural inflammation of all three artery layers (intima, media and adventitia) with patchy infiltration by lymphocytes, macrophages and multinucleated giant cells which results in vessel thickening, stenosis and subsequent ischaemia.
A 57 year old man presents with a continuous headache, severity of 8/10 unilaterally, continuous and in a temporal distribution. Additionally, he reports visual changes.
He is otherwise well, with a PMHx of diabetes mellitus which is controlled on Salbutamol PRN.
O/E he has scalp tenderness, a blue tinge to his vision and prominent temporal artery.
His CRP is raised, ESR is raised and temporal artery biopsy shows inflammatory infiltrates.
Give your DDx and Tx.
Temporal Arteritis
• Corticosteroids: Prednisolone 40mg
-> Taper dose
What is Microscopic Polyangiitis?
Vasculitis which is ANCA-positive, featuring no granulomatosis or eosinophilia and commonly causes glomerulonephritis
A 27 year old male patient presents with haemoptysis and arthralgia. He has no known medical conditions and takes no drugs.
O/E he has a soft, non-tender abdomen. S1, S2 heart sounds are present and no murmur is present. His lung fields are clear with no dullness or hyper-resonance.
His eGFR appears reduced, urinalysis shows microscopic haematuria and autoantibodies are present for pANCA.
Give your DDx and Tx.
Microscopic Polyangiitis
• Corticosteroids: Prednisolone 40mg
-> Taper treatment
What is the eponymous term for a systemic vascular disease featuring necrotising granulomas in an ANCA-positive individual?
Wegners Syndrome
Granulomatosis with Polyangiitis
A 35 year old patient presents with a cough which is productive, producing a teaspoon of blood. He has chest pain and SOB. Additionally, there are systemic symptoms of malaise and tenderness.
O/E his trachea is normal, his CRT is <2 seconds, there is consolidation in the left middle and lower zones and reduced air entry in these zones.
FBC shows leukocytosis and thrombocytosis and no eosinophilia with schistocytes on the film. Autoantibodies are cANCA positive and a CXR shows rounded opacities with cavitation and consolidation in the L lung fields with a meniscus sign.
Give your DDx and Tx.
Granulomatosis with Polyangiiitis
• Immunosuppression: IV methylprednisolone + Cyclophosphamide
What is the eponymous syndrome for ANCA-positive vasculitis featuring asthma and eosinophilia?
Churg-Strauss Symdrome (Eosinophilic granulomatosis with polyangiitis)
What is the triad of Churg-Strauss Syndrome?
Triad: Asthma + Eosinophilia + Systemic vasculitis
A patient presents with SOBE and rhinitis. They have not previously had asthma and they have no fever or headache.
Their PFTs show an obstructive picture with FEV1/FEVC < 0.7 and FBC showing eosinophilia.
Give your DDx and Tx
Churg-Strauss Syndrome
• Corticosteroids: Prednisolone 40mg
-> Taper treatment
A 6 year old child presents with a purpuric rash over the buttocks, abdominal pain and vomiting. He had a URTI 2 weeks ago, with coryzal symptoms.
O/E there is no marked fever, the rash blanches and he has a soft, tender abdomen with diffuse pain.
Give your likely DDx and Tx.
Henoch-Shonlein Purpura
• Conservative: NSAIDs/ Anti-emetics/ Fluids
-> Self-limiting
Give the disease when small proteins precipitate in the small-vessels during the cold.
Small-vessel vasculitis caused by cryoglobulins deposited which precipitate in the cold. This is characterised by skin, kidney and nervous system involvement
A 34 year old man of Turkish descent presents with recurrent oral ulcers. He reports joint pain in his MCPs, PIPs and DIPs.
O/E you identify erythematous plaques on the extensor surfaces of the legs, some eye redness and photophobia.
A skin pathergy test is positive. Autoantibodies for ANCA are negative.
Give your DDx and Tx.
Behçet’s Disease
• Immunosuppressive therapy: Steroids/ Azathioprine/ Ciclosporine
2nd
• Immunosuppressive: Thalidomide
