Vasculitides Flashcards
Define:
- Vasculitis is an inflammatory disorder of blood vessel walls, causing destruction (aneurysm/rupture) or stenosis.
- Can affect vessels of any organ and presentation depends on which organs are involved
- Can be primary or secondary to other diseases e.g. SLE, RA, hep B and C, HIV
Classification:
o Primary vasculitides are classified based on the MAIN VESSEL SIZE affected:
• LARGE
Giant cell arteritis
Takayasu’s arteritis
MEDIUM
Polyarteritis nodosa
Kawasaki’s disease
SMALL
• These can be divided in to ANCA +ve and –ve vasculitis
• ANCA +ve
p-ANCA associated microscopic polyangiitis, glomerulonephritis and Churg-Strauss syndrome
c-ANCA associated Wegener’s granulomatosis (granulomatosis with polyangiitis)
• ANCA –ve
Henoch-Schonlein purpura
Goodpasture’s syndrome
Cryoglobulinaemia
Aetiology:
Unknown
Suggest origin = autoimmune
Risk factors:
o Hepatitis B - polyarteritis nodosa
o Hepatitis C - mixed essential cryoglobulinaemia
o pANCA - microscopic polyangiitis + Churg-Strauss
o c-ANCA - Wegner’s granulomatosis
Epidemiology:
- RARE
- Takayasu’s arteritis is most common in JAPANESE FEMALES and women < 40
- GCA is most common in women over 55
- Granulomatosis with polyangiitis most common in middle aged men
- Kawasaki most common in children <5, especially boys
General signs and symptoms:
o General: fever, malaise, night sweats, weight loss
o Skin: rash, ulcers, purpura
o Joint: arthralgia, arthritis
o GI: abdominal pain, haemorrhage, diarrhoea
o Kidneys: glomerulonephritis, renal failure, hypertension
o Lungs: dyspnoea, cough, chest pain, haemoptysis, haemorrhage
o CVS: pericarditis, coronary arteritis, myocarditis
o CNS: mononeuritis multiplex, infarctions, strokes, fits, chorea, confusion
o Eyes: retinal haemorrhage, cotton wool spots
Characteristics of Takayasu:
affects arteries branching off from the aortic arch.
o If it affects parts supplying upper extremities, you get weak or no pulse.
o If it affects supplying the head, you get visual and neurological symptoms
Characteristics of Granulomatosis with Polyangitis - Wegener’s:
o Affects nasopharynx, lungs and kidneys
o Nasopharynx: chronic pain from sinusitis, bloody mucous and nasal discharge, saddle nose shape
o Lungs: breathing difficulties, haemoptysis
o Kidneys: glomerulonephritis, decreased urine production, hypertension
> Saddle nose
> Associated with cANCA and P53
Microscopic polyangitis
o Very similar to Wegener’s but does not affect nasopharynx, only kidneys and lungs
o Also pANCA associated rather than cANCA
Investigations:
o FBC - normocytic anaemia, high platelets, high neutrophils
o Eosinophil count
o High ESR/CRP
o Creatinine high for renal failure
• Autoantibodies - e.g. cANCA in Wegner’s
• Urine - haematuria, proteinuria, red cell casts (if glomerulonephritis)
• CXR - diffuse, nodular or flitting shadows, atelectasis
• Biopsy - renal, lung, temporal artery – in GCA, but note that GCA is segmental so biopsy requires a long section
• Angiography - to identify aneurysms (in PAN)
Characteristics of polyarteritis nodosa:
> Rosary sign
Associated with Hep B
Abdo pain and rectal bleeding
Skin rash
Characteristics of kwasaki’s:
Usually under 5 years – protracted fever (over 5 days) – acute cervical lymphadenopathy – bilateral non-purulent conjunctivitis – dry, cracked, fissured lips – redness, oedema of palms and soles Platelets, CRP (C-reactive protein) usually ↑↑
Characteristics of Goodpastures:
Affects lungs and kidneys
- Anti GBM antibodies
Characteristics of Churg-strauss:
Eosoniophilic
Rhitinits, asthma
Vasculitic
Asscociated with pANCA
Characteristics of GCA:
Headache
Temporal tenderness
Jaw claudication
Associated with polymalgia rheumatica
Characteristics of Behcet’s:
HLA B51 Oral and genital ulcers Uveitis Erythema nodosum Colitis Pericarditis
