Vasculitides Flashcards
Define Vasculitides
Inflammation and necrosis of the blood vessels
What are the causes of small vessel Vasculitides
Behçet’s
Granulomatosis with polyangiitis (Wegener’s)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Microscopic polyangitis
Henoch-Schönlein purpura
Goodpasture’s
What are the causes of medium vessel Vasculitides
Polyarteritis nodosa
Kawasaki’s disease
What are causes of large bessel Vasculitides
Giant cell arteritis
Polymysalgia rheumatica
Takayasu’s arteritis
Describe Behçet’s disease (RF, epidemiology)
RF: HLA-B51
More common in Mediterranean e.g. Greece, Cyprus
Symptoms and signs of Behçet’s disease
Triad:
Recurrent oral ulcers
Genital ulcers
Uveitis
Rash, arthritis, pericarditis, colitis
Symptoms and signs Granulomatosis with polyangiitis
(Wegener's) Triad: - URT symptoms (rhinitis, nose bleed) - LRT symptoms (haemoptysis) - Glomerulonephritis -> Haematuria
Sore eye, ear, joints, gangrene
Saddle nose
Symptoms and signs of Eosinophilic granulomatosis with polyangiitis
Tri-phasic
- Allergic - asthma/rhinitis
- Eosinophilic - tissue damage in lung and GIT
- vasulitic - widepsread organ damage and death
Haemoptysis, rash, focal neuropathy, kidney damage
Symptoms and signs of Microscopic polyangitis
Rapidly progressing glomerulonephritis
Pulmonary haemorrhage -> haemoptysis
Investigations to distinguish between the different small vessel Vasculitides
Behçet’s disease - Clinical diagnosis. Can do pathergy test (needle prick -> inflamed and sterile pustule within 48h)
GwP - cANCA
EGwP - pANCA
Microscopic polyangitis - pANCA
Describe polyarteritis nodosa (cause, associations, investigations)
Idiopathic
Associated with hepatitis B infection
Can affect any age
Rosary sign seen on angiography
Symptoms and signs of polyarteritis nodosa
Constitutional symptoms Skin rash Abdominal pain Rectal bleeding Peripheral neuropathy
Describe Takayasu’s arteritis
Seen in young-middle-aged Asian/Japanese women
Affects the aorta and its branches
Usually constitutional upset
Absent/weak upper/lower limb pulses and claudication
Tenderness over affected arteries
Describe Kawasaki’s disease
Age <5 years
Fever >5 days
Skin rash
Fissured lips
Red swollen palms and soles
Describe Henoch-Schönlein purpura (HSP)
IgA vasculitis
Purpura on leg and buttocks TRIAD 1. Purpuric rash on buttocks and extensors of lower limbs 2. Abdominal pain 3. Arthralgia (oedema)