Crystal Athropathy Flashcards
(46 cards)
What is vasculitis
Inflammation of the blood vessels
What causes small vessel vasculitis
Henoch-Schonlein purpura
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener’s granulomatosis)
What causes medium vessel vasculitis
Polyarteritis nodosa
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Kawasaki Disease
What causes larger vessel vasculitis
Giant cell arteritis
Takayasu’s arteritis
What generic features may lead you to suspect vasculitis
Purpura Joint and muscle pain Peripheral neuropathy Renal impairment Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding) Anterior uveitis and scleritis Hypertension
What is purpura
These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin
What systemic features may be associated with vasculitis
Fatigue Fever Weight loss Anorexia (loss of appetite) Anaemia
What investigations should you if suspected vasculitus
- ESR/CRP
- Anti neutrophil cytoplasmic antibodies (ANCA): pANCA & cANCA
What is pANCA usually a sign of
Microscopic polyangiitis and Churg-Strauss syndrome
What is cANCA usually a sign of
Wegener’s granulomatosis
What is the management of vasculitis
- Depends on type, refer to rheumatologist
- Steroids
- Immunosupressants
What is Henoch-Schonlein purpura
- IgA vasculitis that commonly presents with a purpuric rash affecting the lower limbs or buttocks in children
- often triggered by an upper airway infection (e.g. tonsillitis) or a gastroenteritis.
- ash is caused by inflammation and leaking of blood from small blood vessels under the skin, forming purpura.
What are the four classic features of Henoch-Schonlein purpura
- purpura (100%)
- joint pain (75%)
- abdominal pain (50%)
- renal involvement (50%) IgA nephritis
What is the management of Henoch-Schonlein purpura
- supportive
- simple analgesia: abdo pain settles in a few days
- rest
- proper hydration
What is the prognosis of Henoch-Schonlein purpura
- without kidney involvement can expect to fully recover within 4-6 weeks
- 1/3 recurrence within 6 months
- 1% of patients develop end stage renal failure.
What is Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
- most associated with lung and skin problems, but can affect other organs such as kidneys.
- presents with severe asthma in late teenage years or adulthood.
- Characteristic finding: elevated eosinophil levels
What is Microscopic polyangiitis
- renal failure
- can also affect the lungs causing shortness of breath and haemoptysis.
What is Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- Affect kidneys and lungs
- Lung symptoms: cough, wheeze and haemoptysis
- Kidney symptoms: Rapidly progressing glomerulonephritis.
What Upper resp tract findings can you see in Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- nose bleeds (epistaxis)
- crusty nasal secretions
- hearing loss
- sinusitis.
- saddle shaped nose due to a perforated nasal septum.
What CXR findings might you see in Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- consolidation- (misdiagnosed as pneumonia)
What is Polyarteritis Nodosa
- affects the medium sized vessels in locations such as the skin, gastrointestinal tract, kidneys and heart
- Can cause renal impairment, strokes and MI
- Associated with a rash called livedo reticularis
What can polyarteritis Nodosa be associated with
- Mainly Hepatitis B
- hepatitis C
- HIV
- No clear cause
What is Kawasaki Disease
- medium vessel vasculitis. It affects young children, typically under 5 years of age. There is no clear cause.
What are the key features of Kawasaki Disease
- Persistent high fever > 5 days
- Erythematous rash
- Bilateral conjunctivitis
- Erythema and desquamation (skin peeling) of palms and soles
- “Strawberry tongue” (red tongue with prominent papillae)